Undescended Testis and Testicular Tumors Flashcards

Question

What is a varicocele?

Answer 1

The abnormal dilatation or tortuosity of the veins of the pampiniform plexus.

Answer 2

The etiology of a varicocele is usually multifactorial. It is probably related to increased hydrostatic and venous pressure within the left gonadal vein, due to the fact that this vein is longer than the right and drains into the renal vein at a right angle. Additionally, gonadal veins associated with varicoceles have been shown to often have absent or incompetent valves [4]. Isolated right-sided varicoceles are unusual, and this finding should always prompt imaging of the retroperitoneum to exclude a mass causing compression of the right gonadal vein.

Answer 3

Besides discomfort and concerns regarding cosmetic appearance, varicoceles are associated with testicular atrophy and dysfunction. Varicocele may cause loss of testicular volume as well as areas of testicular dysfunction on biopsy, and semen analysis may reveal decreasing sperm density and motility over time.

Answer 4

Through testicular hyperthermia, a varicocele interferes with the mechanisms that usually allow the scrotum to be 1–2° Celsius cooler than body temperature, thereby interfering with spermatogenesis. It is also possible that varicoceles cause hypoxia and oxidative stress in testes, and that renal and adrenal metabolites may reflux into the spermatic vein.

Answer 5

The patient should be made comfortable, and examined in a warm room, in both the recumbent and upright positions, and should be asked to perform a valsalva manoeuvre if the varicocele is not apparent. The testicular cord should be palpated directly above the testis, with the clinician specifically looking for dilated veins above the testicle. The testes should be examined for size discrepancies.

Answer 6

Colour Doppler ultrasound is not indicated routinely, however can be used when the examination is equivocal. Ultrasound is also useful in obtaining an objective assessment of testicular volume. In pre-pubertal boys and in cases of right-sided varicocele, the abdomen should be imaged to exclude a retroperitoneal mass or Wilm’s tumour. Venography is highly sensitive but also invasive, and is generally only used when there is an intention to treat the varicocele with thromboembolism in the same setting.

Answer 7

There are 3 grades: Grade I—palpable with Valsalva manoeuvre only Grade II—palpable without Valsalva manoeuvre Grade III—visible at a distance.

Answer 8

In adolescents, indications for treatment include a discrepancy in testicular vol- ume of more than 20%, pain that cannot be explained by other pathology and that does not respond to conservative therapy, the presence of an additional tes- ticular condition which may predispose to sub-fertility, and bilateral varicoceles. Additionally, males with decreasing sperm counts over serial samples should be offered treatment.

Answer 9

To cause disruption of the internal spermatic drainage of the testicle, whilst pre- serving the spermatic artery, vasal and differential vessels, and lymphatics.

Answer 10

Varicocelectomy can be performed through surgical ligation of the internal sper- matic vein, or by radiographic venous embolization. This may be performed through and inguinal or sub-inguinal approach, or laparoscopically. Magnification should be used, and intra-operative Doppler is useful to differentiate between venous structures and the artery.

Answer 11

AFP (produced by Lacunar Cells) - Half life of 1.5d B-HCG (produces by syncitiotrophoblastic cells) - Half life of 5d

Answer 12

A. Perform the two-stage procedure; Do ligate the vessels and then perform the orchidopexy after 6 months.

Answer 13

E. Decreased incidence of torsion of testes.

Answer 14

B. Beta HCG and LHRH has high success rate.

Answer 15

D AFP normalizes 5-7 weeks post-surgery

Answer 16

Testicular development and descent depend on a coordinated interaction among endocrine, paracrine, growth, and mechanical factors. 6-7 weeks AOG: Bipotential gonadal tissue located on the embryo’s genital ridge begins differentiation into a testis during weeks 6 and 7 under the effects of the testis-determining SRY gene. Sertoli cells begin to produce Müllerian inhibitory factor (MIF) soon thereafter, causing regression of most Müllerian duct structures except for the remnant appendix testis and prostatic utricle. 9 weeks AOG: By week 9, Leydig cells produce testosterone and stimulate development of Wolffian structures, including the epididymis and vas deferens. The testis resides in the abdomen near the internal ring until descent through the inguinal canal at the beginning of the third trimester. Two important hormones in testicular descent are insulin-like factor 3 (INSL3) and testosterone, both secreted by the testis. Two important anatomic players are the gubernaculum testis and the cranial suspensory ligament (CSL). The gubernaculum is thought to help anchor the testis near the internal inguinal ring as the kidney migrates cephalad. Androgens prompt the involution of the CSL, allowing for eventual downward migration of the testicle. In humans, the frequency of UDT is increased in boys with diseases that affect androgen secretion or function. When antiandrogens are given to pregnant rats, the rate of UDT in male offspring is 50%. Estradiol downregulates INSL3 in experimental models, and maternal exposure to estrogens such as diethylstilbestrol (DES) has also been associated with cryptorchidism. Under the influence of INSL3, the gubernaculum undergoes two phases: outgrowth and regression. Outgrowth refers to rapid swelling of the gubernaculum, thereby dilating the inguinal canal and creating a pathway for descent. Mice with homozygous mutant INSL3 have been found to have poorly developed gubernacula and intra-abdominal testes. Next, during regression, the gubernaculum undergoes cellular remodeling and becomes a fibrous structure. It is believed that intra-abdominal pressure then causes protrusion of the processus vaginalis through the internal inguinal ring, transmitting pressure to the gubernaculum and fostering testicular descent. However, the gubernaculum is not directly attached to the scrotum during inguinal passage, and does not appear to act as a pulley. 22-27 weeks AOG: Transit through the inguinal canal is relatively rapid, starting around week 22, and is typically completed after week 27. Other potential mediators of descent include MIF, by causing resorption of Müllerian structures and clearing anatomic roadblocks to descent, and calcitonin generelated peptide (CGRP). While research in rats has implicated CGRP in contraction of cremasteric muscle fibers and subsequent gubernacular and testicular descent, in humans the cremaster is distinct from the gubernaculum. In addition, growth factors such as epidermal growth factor act on the placenta to enhance gonadotropin release, which stimulates secretion of descendin, a growth factor for gubernacular development. Epididymal anomalies are found in up to 50% of men with UDT. Some investigators postulate that the gubernaculum facilitates epididymal descent, indirectly guiding the testis into the scrotum. Others believe that an abnormality of paracrine function is responsible for both epididymal anomalies and UDT, but the epididymal abnormalities are not causative in the failure of testicular descent. H&A —

Answer 17

A retractile testis is a normally descended testis that retracts into the inguinal canal as a result of cremasteric contraction; it is not an UDT. On examination, both retractile testes and low UDTs may be manipulated into the scrotum. Once in the scrotum, the retractile testes remain in place until displaced by a cremasteric reflex, whereas the low UDT retracts back up to its abnormal location once released. The ipsilateral hemiscrotum is fully developed with a retractile testis, whereas it may be underdeveloped with an UDT. Though retractile testes do not require operative repair, in some series as many as one-third become ascending UDTs, suggesting either an initial incorrect diagnosis or suboptimal attachment within the scrotum that changes the position of the testis with growth of the child. H&A — The most common differential diagnosis of an undescended testicle is a retractile testicle. The first hint of this entity on exam is a normally developed scrotum. A testicle that can be found in the inguinal canal, brought down to the scrotum with no tension, and resides in the scrotum for any period of time is a retractile testicle. Previously thought to be an entirely benign condition, it is now recognized that this condition may evolve into what has become known as ascending or acquired undescended testicle in up to one-third of cases. In this case, a testicle that was previously in a normal position can no longer be palpated in the scrotum. The majority are found in the distal inguinal canal or within the superficial inguinal pouch at the pubic tubercle. It is not unusual to see a boy in the 4–8 year age range with this history. This entity may help account for the persistently higher than recommended age of orchidopexy in studies emerging from several countries. Recent evidence suggests that men with acquired undescended testicles more often had significantly abnormal testicular consistency, smaller testes, lower sperm concentration, and less-motile sperm. Therefore, children with retractile testicles should be followed yearly until a scrotal testicular position is well documented after puberty. A scrotal examination should also be completed by pediatricians in all boys during well-child visits. Sherif

Answer 18

A true UDT has halted somewhere along the normal path of descent from the abdomen to distal to the inguinal ring. An ectopic UDT is one that has deviated from the path of normal descent and can be found in the inguinal region, perineum, femoral canal, penopubic area, or even contralateral hemiscrotum.

Answer 19

An ascending or acquired UDT refers to a testis that was previously descended on examination, but cannot be brought down into the scrotum at a later time. While an association between retractile testes and secondary testicular ascent has been identified, a link between the rate of height growth and ascended testes suggests that the ability to reach the scrotum changes with a child’s growth. Thus, a significant growth spurt may be a factor in a retractile testis becoming an undescended testis. An acquired UDT may also be iatrogenic, which can occur when a previously descended testis becomes trapped in scar tissue cephalad to the scrotum after inguinal surgery. A nonpalpable testis may be simply intra-abdominal some of the time, or truly vanished due to intrauterine or perinatal torsion. This condition is known as monorchia, or anorchia if both testes are absent.

Answer 20

UDT occurs in approximately 3% of term male infants and in up to 33–45% of premature and/or low birth weight (<2.5 kg) male infants. The majority of testes descend within the first 6–12 months such that at 1 year, the incidence is down to 1%. Testicular descent after 1 year is unlikely. However, 2–3% of boys in the United States, and up to 5% in some European series, undergo orchiopexy for UDT. This discrepancy between higher orchiopexy rates and the actual incidence of the disease is thought to lie partially in the misdiagnosis of retractile testes, but also likely related to acquired UDT from testicular ascent. The overall rate of secondary testicular ascent has been reported between 2% and 45%.

Answer 21

Series documenting the location of an UDT find that two-thirds to three-quarters of cases are palpable, usually within the inguinal canal or distal to the external ring. Anomalies associated with UDT include a patent processus vaginalis and epididymal abnormalities. Specific syndromes with higher rates of UDT include prune-belly syndrome, gastroschisis, bladder exstrophy, Prader–Willi, Kallman, Noonan, testicular dysgenesis, and androgen insensitivity syndromes.

Answer 22

The patient should be examined in a warm room in both the supine and frog-legged sitting position. The scrotum is observed for hypoplasia and examined for the presence of either testis. In cases of monorchia, the solitary testis may show compensatory hypertrophy. The first maneuver to locate the testis is to walk the fingers from the iliac crest along the inguinal canal towards the scrotum, pushing subcutaneous structures toward the scrotum. The scrotum should not be palpated prior to this maneuver as it may activate the cremasteric reflex, thus retracting the testis. Lubricating gel or soap may help reduce friction. Gentle mid-abdominal pressure may help push the testis into the inguinal canal. A cross-legged sitting or squatting position may also help identify the testis. It can be particularly challenging to obtain an accurate exam on a ticklish or obese boy. Due to challenges during physical exam, it is accepted that up to 20% of nonpalpable testes will be subsequently palpated when examined under anesthesia in the operating room.

Answer 23

If neither testis is palpable, anorchia, androgen insensitivity syndrome, or a chromosomal abnormality must be differentiated from bilateral nonpalpable UDT. Moreover, a rare (but potentially a life-threatening condition) should also be considered. A phenotypically male newborn with bilateral nonpalpable gonads, even in the presence of an otherwise normal-appearing penis, could represent a masculinized 46,XX baby with congenital adrenal hyperplasia (CAH). If the diagnosis is delayed, the salt-wasting form of CAH can lead to severe electrolyte imbalance and cardiovascular compromise. In such cases, a karyotype is warranted. To avoid unnecessary surgical exploration in a 46XY patient with anorchia, studies to determine the presence of viable testicular tissue should include serum MIF, inhibin B, follicle-stimulating hormone (FSH), luteinizing hormone (LH), and testosterone. If the child is <9–12 months of age, in the absence of viable testes, serum MIS and inhibin B should be undetectable. If the baseline FSH level is elevated (three standard deviations above the mean) in a boy younger than 9 years, anorchia is likely and no further evaluation is recommended. If baseline LH and FSH levels are normal and human chorionic gonadotropic (hCG) stimulation results in an appropriate elevation of testosterone, functioning testicular tissue is likely to be present and the patient should undergo exploration. However, if testosterone levels do not increase appropriately, nonfunctional testicular tissue may still be present and exploration should still be considered. The hCG stimulation test does not distinguish between normal nonpalpable testes and functioning testicular remnants. H&A — Bilateral cryptorchidism is far rarer than its unilateral counterpart. It is more common in syndromic patients, those with chromosomal abnormalities, and those with androgen insensitivity. An ultrasound is typically performed to search for testicular tissue but should not be the decisive test. If no testicles can be palpated in the inguinal canals, the level of follicle stimulating hormone (FSH) should be checked. A level three standard deviations above the mean confirms anorchia. If the FSH and luteinizing hormone (LH) levels are normal, baseline testosterone level should be checked and a human chorionic gonadotropin (hCG) stimulation test should be performed. Increase in the level of testosterone after hCG stimulation implies the presence of testicular tissue, and the patient should be explored. The risk of infertility is increased approximately six fold in men with a history of bilateral undescended testicles. An empty scrotum may be rarely associated with an ectopic, rather than undescended, testicle. The testicle may be found anywhere in the perineum or even in the contralateral scrotum. A standard orchidopexy is performed, similar to that for a canalicular testicle. The length of the testicular vessels is usually not an issue in these cases. Sherif

Answer 24

An UDT and, to a lesser degree, its contralateral descended mate (if present) have been demonstrated to be histologically abnormal by investigators who performed bilateral testes biopsies at the time of orchiopexy. Clinically, patients with a history of UDT exhibit subnormal semen analyses. Early studies showed fertility to be related to the position of the UDT. Men with abdominal or canalicular testes had lower fertility than those with inguinal testes (83.3% vs 90%). Despite these findings, the infertility rate of men with a history of unilateral UDT is equivalent to that of the normal population (∼10%). However, men with bilateral UDT have paternity rates of 50–65% even if corrected early, and thus are six times more likely to be infertile relative to their normal male counterparts. Mechanisms of infertility in UDT appear to be associated with effects on Sertoli and Leydig cells, as well as Wolffian duct abnormalities (vasal and epididymal), which may further inhibit transport of already insufficient sperm. Elevated testicular temperature in an UDT results in immaturity of Sertoli cells in monkeys. A blunted normal testosterone surge at 60–90 days postnatally results in a lack of Leydig cell proliferation and delay in transformation of gonocytes to adult dark spermatogonia on histopathology. An experimental rat model has demonstrated preservation of germ cell number and spermatogenesis in rats undergoing early orchiopexy for UDT versus germ cell apoptosis in untreated rats. Furthermore, delayed orchiopexy at 3 years versus 9 months resulted in impaired testicular catch-up growth in boys. A clinical trial of neoadjuvant LH-releasing hormone (LHRH) in young boys undergoing orchiopexy appeared to improve the fertility index (spermatogonia/tubule) in treated versus untreated boys, though these results need confirmation. A similar prospective randomized trial on neoadjuvant gonadotropin-releasing hormone therapy prior to orchiopexy also found an improvement in the mean fertility index compared with the untreated group. Neoadjuvant therapy prior to 24 months achieved the best results. The long-term benefits (or risk) of hormonal stimulation for these purposes remains largely unknown, and it is not commonly practiced in many settings.

Answer 25

UDT appears to be associated with a two- to eightfold increased risk of malignancy. This risk appears to vary with the gonad’s location: 1% with inguinal and 5% with abdominal testes. Cancers arising in testes that remain in the abdomen are most frequently seminomas (74%). In contrast, malignancies arising after successful orchiopexy, regardless of original location, are most frequently nonseminomatous germ cell tumors (63%). Among men with testicular cancer, up to 10% have a history of UDT. There are two competing theories regarding this increased risk. First, the “position theory” implicates the carcinogenic potential of the altered micro- and macroenvironment of the UDT. If true, then the timing of correction could potentially lessen or negate the development of malignancy. A 2007 epidemiologic study examining 16,983 Swedish men who underwent correction of an UDT showed that those having orchiopexy before age 13 had a 2.23 relative risk of developing cancer. Those boys having surgery at 13 years or older had a relative risk of 5.40 (compared with normal men). An additional metaanalysis showed that orchiopexy after 10 years of age compared with before age 10 was associated with six times the risk of malignancy. These associations between age of orchiopexy with a decrease in cancer risk need further verification, yet provide compelling evidence for early surgical intervention. Moreover, by placing the gonad in an accessible location, orchiopexy facilitates subsequent testicular examination and can potentially help with early cancer detection. The alternate “common cause” or “testicular dysgenesis” theory posits that the malignancy risk may be due to an underlying genetic or hormonal etiology that predisposes to both cryptorchidism and testicular cancer. In patients with an UDT, 15–20% of testicular tumors arise in the normally descended contralateral testis. In other words, the normally descended testis still carries an increased relative risk of malignancy of 1.7. The incidence of carcinoma in situ (CIS) is 2–4% in men with cryptorchidism compared with <1% in non-affected men. In the postpubertal male, CIS progresses to invasive germ cell tumors in 50% of cases within 5 years. However, the natural history of CIS diagnosed in a young child at the time of orchiopexy is less clear. Although it has been recommended that patients undergo repeated biopsies after puberty, it is unclear it this intervention leads to benefits in terms of cancer prevention.

Answer 26

Guidelines (AAP 1996 and EAU 2012) recommend that, in otherwise healthy males, orchiopexy should be performed by 12–18 months of age, as the gonad is unlikely to descend after 6–12 months of age and there is not an added benefit from waiting. Similarly, the 2014 AUA update on cryptorchidism recommends referral by 6 months with orchiopexy performed by 18 months. Despite these recommendations, many children are referred after age 2 years. In one review of over 28,000 children with UDT in the Pediatric Health Information System database, only 18% underwent operation by 1 year of age and 43% by 2 years of age. Black and Hispanic boys less commonly underwent orchiopexy by age 2 years, regardless of payer group and socioeconomic status. Repair may be undertaken even earlier if a symptomatic hernia is present. The risk of general anesthesia after 6 months is acceptably low in hospitals with dedicated pediatric anesthesiologists, although the risk of neurotoxicity from anesthetics remains a worrisome and unresolved issue. In addition to the evidence that early scrotal placement may affect the risk of malignancy and infertility, treatment of a UDT also reduces the risk of torsion, facilitates testicular examination, improves the endocrine function of the testis, and creates a normal-appearing scrotum.

Answer 27

The value of hormonal therapy in the treatment of UDT is controversial. Buserelin, an LHRH agonist, is frequently used to treat UDT in some centers (particularly in Europe). The highest success rates have been observed in cases in which the testis is at or distal to the external inguinal ring. Some authors recommend low-dose hCG therapy, regardless of the operative plan, to restore a normal endocrine milieu and enhance germ cell maturation, particularly in bilateral UDT. Trials combining buserelin and hCG have yielded success rates in the range of 60%, but orchiopexy is still required in 40% of patients. Buserelin has not been approved for this use by the U.S. Food and Drug Administration, but as noted earlier, clinical trials of LHRH used in a neoadjuvant fashion in young boys undergoing orchiopexy suggest that it may improve fertility. The 2014 AUA update on cryptorchidism does not recommend the use of hormonal therapy to induce testicular descent. These guidelines have largely been embraced by providers, and thus routine use of hormonal therapy for UDT in North America is now rare.

Answer 28

The operative approach for UDT depends on whether the testis is palpable. It is important to reexamine the patient under anesthesia because, in one study, up to 18% of nonpalpable testes became palpable on examination under anesthesia. Unilateral and bilateral palpable UDT are managed similarly. Routine biopsy of the testis at the time of surgery is not recommended, but may provide prognostic information regarding fertility.

Answer 29

For the unilateral palpable UDT that presents after puberty, orchiopexy or orchiectomy can be offered. Many studies advocate for orchiectomy in the postpubertal male with an UDT given the frequent lack of spermatogenesis in the affected testicle and increased risk of cancer. If orchiopexy is difficult and a normal contralateral testis is present, or if the UDT is abnormally soft and small, then an orchiectomy should be performed. Likewise, orchiectomy is the treatment of choice for the postpubertal, unilateral intra-abdominal UDT because of the increased cancer risk. Laparoscopic orchiectomy is ideal in this setting. In uncommon cases, such as postpubertal males with significant anesthetic risks or males older than age 50, observation is an acceptable alternative to operation.

Answer 30

The mainstay of therapy for the palpable UDT is orchiopexy with creation of a subdartos pouch. This may be performed through a standard two-incision (inguinal and scrotal) approach, or a single-incision high scrotal approach. With the standard inguinal method, the success rate is as high as 95%. Similar success rates have been reported for the high scrotal modification. With both techniques, scrotal fixation is achieved by scarring of the everted tunica vaginalis to the surrounding tissues. Placement of sutures in the tunica albuginea for fixation is debated because of the potential for it to cause testicular inflammation, possibly an increased infertility risk, and may damage intratesticular vessels. Associated findings such as an open processus vaginalis or hernia should be concurrently repaired. A standard inguinal approach to orchiopexy with a subdartos pouch is usually performed as an outpatient procedure under general anesthesia. The patient is positioned supine. Intraoperative administration of an ilioinguinal nerve block with bupivacaine provides excellent postoperative analgesia. An incision is made along one of the Langer lines over the internal ring. The external oblique aponeurosis is incised in the direction of its fibers, avoiding injury to the ilioinguinal nerve. Once located, the testis and spermatic cord are freed from the canal and any cremasteric and ectopic gubernacular attachments. Rarely, a long-looping vas may be found, which can hinder mobilization and can make it difficult to locate the testis in the mid to lower scrotum. The tunica vaginalis is then dissected off the vas deferens and spermatic vessels. The proximal sac is dissected free, suture ligated, and amputated. Retroperitoneal dissection through the internal ring may provide additional cord length for the testis to reach the scrotum. A tunnel is created from the inguinal canal into the scrotum by using a finger or a large clamp. A subdartos pouch is created by making an incision in the scrotum and using a hemostat inserted just under the skin to spread inferiorly. A clamp is carefully passed through this scrotal incision up into the inguinal canal, and the adventitial tissue around the testis is secured, taking care not to grasp the testis or vas deferens. The testis is thereby delivered into the dartos pouch, and a suture is used to narrow the neck of the pouch to prevent testicular retraction. Testis measurements and biopsy can be performed at this time if desired. The scrotal skin incision is closed with absorbable sutures. The external oblique aponeurosis is reapproximated to restore the inguinal canal. The skin and subcuticular tissues are closed with subcuticular stitches. A skin sealant is useful, especially for boys in diapers. The patient is seen in the clinic after a few weeks for a wound check and again several months later for testicular examination, instruction on testicular self-examination, and repeat counseling on fertility and cancer risk (when age appropriate). Final position and condition of the testis should be noted. Although uncommon, complications include atrophy and retraction. A single scrotal incision technique has also been applied to orchiopexy, with similar success rates and shorter operative times, but one group demonstrated an increased risk of postoperative inguinal hernia with this approach. However, this increased risk of a hernia has been disputed, and currently scrotal orchiopexy is considered a reasonable approach for management of a palpable UDT.

Answer 31

For a unilateral UDT that is not palpable under anesthesia, initial management may be either through diagnostic laparoscopy or inguinal exploration. In the last decade, laparoscopy has become the preferred approach. If the surgeon decides to first perform inguinal exploration and no testis or remnant is identified, then diagnostic laparoscopy or laparotomy is mandatory to ensure the testis is not in an intra-abdominal location. In one retrospective review of 215 nonpalpable testes, only 34% were located distal to the internal ring, and an initial inguinal incision would have provided suboptimal exposure for the remaining 66%. The surgeon may begin with diagnostic laparoscopy through an umbilical port. If the vessels appear atretic or “blind ending” as they exit the abdomen, some have recommended no further exploration, though this is controversial. If the testicular vessels are seen exiting the internal ring, a laparoscopic inguinal exploration is performed if the ring is open, or an open inguinal exploration if the ring is closed. Orchiopexy is performed if a viable testis is found. If the vessels end blindly in the inguinal canal, the tip of the vessels can be sent for pathologic examination. Remnants of testicular tissue or hemosiderin and calcifications are indicative of probable perinatal torsion and testicular resorption. If diagnostic laparoscopy reveals a viable intra-abdominal testis, several options are available depending on its location and surgeon preference. A recent review concluded that, while there is no optimal surgical technique for an intra-abdominal testis, preservation of the spermatic vessels is preferable whenever possible. If the gonadal vessels are long enough to allow for tension-free mobilization of the testis into the scrotum, orchiopexy can be performed open or laparoscopically. This is often feasible when the testis lies caudal to the iliac vessels. When the gonadal vessels are too short, there are various options. Whether through an open operation or laparoscopically, the cord structures are mobilized cephalad toward their origin, freed from the posterior peritoneum, and the testicle is brought into the scrotum. A neoinguinal ring may be created medial to the inferior epigastric vessels to shorten the path for scrotalization of the testis (Prentiss maneuver). Most series indicate a 95–100% success rate, defined as lack of atrophy and a normal scrotal position, with single-stage laparoscopic orchiopexy. A staged orchiopexy can also be performed in which the high abdominal testis with its cord structures is first mobilized as low as possible. Six to 12 months later, it is further mobilized into the scrotum. The advantage of this approach lies in preservation of both primary and collateral blood supply. However, during the second stage, injury may occur to the vascular supply and/or vas deferens because of scarring to surrounding tissues, and the total vessel length may not be significantly longer than what resulted from the initial intervention. Based on this limitation, a novel intervention was developed by Shehata et al., based on the old concept of vessel elongation by applying tension. In the Shehata two-stage orchiopexy, the intra-abdominal testicle is laparoscopically mobilized, the gubernaculum is released, and then the gonad is placed on tension within the abdomen to allow the spermatic cord vessels to stretch over a period of several months. The second-stage procedure further mobilizes the testicle into the scrotum while preserving the spermatic vessels, which should now be sufficiently long to allow the testis to reach the scrotum. Early success rates are promising at 80+%, but longer term studies are still needed. In cases where tension on the spermatic cord does not allow placement of the testis within the scrotum, a singlestage Fowler–Stephens orchiopexy can be attempted. In this scenario, the testicle is mobilized along the spermatic cord without dividing the gubernacular attachments. It is important to preserve the peritoneum overlying the vas to reduce the risk of vasal artery spasm or injury. The spermatic cord vessels are then divided, and the testicle is secured within the scrotum. This technique is associated with a higher risk of testicular atrophy and loss, and is not favored when compared with other options. At times, the procedure is performed as a bail out procedure when a singlestage orchiopexy with vessel preservation is attempted, but the length of the vessels is underestimated. Most commonly, in the setting of short spermatic vessels, a two-stage Fowler–Stephens orchiopexy is performed, typically laparoscopically. The first-stage Fowler–Stephens orchiopexy involves clipping and division of the spermatic vessels, which forces the testis to be dependent on the vasal and cremasteric vessels for viability, and the collateral blood supply becomes more robust over time. Due to the dependency on collateral blood flow, anatomic conditions or previous procedures that jeopardize it are contraindications to using this strategy. Thus, the Fowler–Stephens approach is not a good option after prior inguinal exploration because this secondary vascular supply to the testis may have been disrupted. A delay of at least 6 months is commonly recommended before stage 2 to allow full development of the collateral circulation. During the second stage, the testis is maximally mobilized and positioned within the scrotum. The success rate in modern single-center case series with follow-up longer than 3 years is between 80% and 90%. Microvascular orchiopexy is infrequently used as it requires special instrumentation, microsurgical skill, and involvement of a surgical specialist with microvascular skills. It is potentially indicated in the rare case of a solitary gonad that lies high in the abdomen. The procedure is not widely performed, and thus expertise is not available at most referral centers. An 83–96% success rate in experienced hands has been reported. If the testis is atrophic, whether found in the abdomen or inguinal canal, a laparoscopic or open orchiectomy is recommended, respectively. Debate exists regarding the role of contralateral fixation in cases of monorchism because of differing assumptions related to potential torsion. This largely remains a decision made by an informed family and the surgeon’s preference. Boys with bilateral nonpalpable UDT may benefit from genetic, endocrinologic, or imaging evaluation to identify the presence or absence of testicular tissue (i.e., hormonal evaluation confirming testosterone production). It is important to distinguish older children with bilateral nonpalpable testicles from newborns with bilateral nonpalpable gonads as the latter should raise immediate concern for a disorder of sexual differentiation (DSD), specifically CAH. In the modern era, laparoscopy is the preferred approach for bilateral nonpalpable testicles, as it provides diagnostic information as well as an opportunity to provide simultaneous surgical management. If laparoscopy reveals only one viable testicle, the child is managed as in the situation with unilateral, nonpalpable UDT with a laparoscopic one-stage or twostage orchiopexy. When bilateral viable intra-abdominal testes are found, management may depend on the ease of orchiopexy. If difficult, one side may be fixed first, with the contralateral side fixed 6–12 months later. This allows the practitioner to assess the outcome of the first side prior to operating on the contralateral testis. Other options include bilateral one-stage or two-stage orchiopexies. Of these options, the simultaneous bilateral one-stage and/ or first-stage Fowler–Stephens orchiopexies are the most debated. Given the debate surrounding childhood exposure to multiple anesthetics, however, an argument can be made to perform whatever procedure will allow use of the least number of anesthetics.

Answer 32

Secondary UDT is an uncommon complication of inguinal hernia repair, orchiopexy, or hydrocelectomy. Surgical technique differs from that of primary repair because scarring from the previous procedure makes cord dissection difficult with an increased risk of vascular or vasal injury. Cartwright and associates described a technique for reoperative orchiopexy in which the entire cord and scar is mobilized en bloc along with a strip of external oblique aponeurosis. The testis/cord/aponeurosis complex is dissected together superior to the internal ring where the aponeurosis is then cut and dissection continued above the area of previous scar into the retroperitoneum to allow more extended mobilization. If more length is necessary, division of the inferior epigastric vessels allows the cord to be displaced medially. Prevention is key, and most experienced surgeons teach that the ipsilateral testicle should be gently pulled into its normal anatomic position after any inguinal procedure in a boy. H&A — The natural history of this condition is gradual improvement in testicular position in approximately two-thirds of patients. As long as the testicle is within the scrotal confines, it may be observed. A testicle clearly within the inguinal canal should undergo orchidopexy if there is no descent after a period of observation of at least 1 year. In these cases, local mobilization of the testicle by carefully dividing the surrounding scar should allow for successful placement of the testicle into the scrotum. Sherif

Answer 33

Testicular cancer is uncommon in children, accounting for 1–2% of all pediatric solid tumors. The peak incidence of pediatric testicular tumors occurs between ages 12 and 24 months, followed by a second small peak during puberty. Also, prepubertal boys have a much larger percentage of benign-behaving testicular lesions than postpubertal and adult males. Management of testicular tumors in prepubertal boys thus differs rather dramatically from postpubertal males, as testis-sparing procedures (i.e., partial orchiectomy) is favored in the former. Conversely, in postpubertal patients and adults curative treatment tends to favor a radical orchiectomy. Malignant, germ cell cancers tend to respond well to chemotherapy, and the need for retroperitoneal lymph node dissection (RPLND) is relatively rare. In previous reports, germ cell tumors were thought to comprise 65–85% of pediatric testicular tumors. This was largely based on tumor registries that reported that >60% of tumors were yolk sac (YST) and approximately 20% were teratomas. Current literature reports indicate teratomas and epidermoid cysts are the most common neoplasms in prepubertal children. Data also now support the rationale for testis-sparing surgery in prepubertal patients with negative serum markers (particularly α-fetoprotein [AFP], which is almost universally elevated in patients with YSTs). There are several patient populations that have an increased risk for neoplasms. In particular, males with gonadal dysgenesis, hypovirilization, and disorders of sexual development have an increased incidence of gonadal tumors, particularly gonadoblastoma.

Answer 34

See algorithm below.

Answer 35

A testicular tumor typically presents as a painless scrotal mass. A history of trauma is occasionally given and may be the event that brings attention to the enlarged scrotum. Sometimes, a tumor arising in a UDT may cause torsion and present with acute abdominal pain. Malignancy typically is nontender, does not transilluminate, and is associated with a normal urinalysis. An associated hydrocele in 15–20% of patients may impede adequate testicular examination. Hormonally active tumors can cause precocious puberty. As part of the initial evaluation for a testicular mass, color Doppler US and serum tumor markers (AFP, β-hCG, and lactate dehydrogenase [LDH]) should be obtained. US is nearly 100% sensitive for detecting a testicular tumor. Though not pathognomonic, anechoic cystic lesions usually suggest benign disease. Internal calcifications and a mass with “onion-skin” alternating hypo- and hyperechoic lesions suggests an epidermoid cyst or teratoma. These findings may be useful in preoperative planning for a testissparing operation. Serum tumor marker levels are valuable not only in the diagnosis, but also for follow-up of testicular malignancy. AFP is a glycoprotein produced by the fetal yolk sac, liver, and gastrointestinal tract. It is elevated in a variety of benign and malignant diseases, including YSTs of the testis. The half-life of AFP is approximately 5.5 days, and the normal adult levels (<10 ng/mL) are not achieved until around 10 months of age (Fig. 51.10). β-hCG is a glycoprotein produced by some seminomas and mixed germ cell tumors, as well as choriocarcinomas. Its half-life is approximately 24 hours and is normally not detected in significant amounts in boys (<5 IU/L). Once the diagnosis of high-risk testicular cancer is made histologically, CT can be useful evaluation for metastatic disease. CT has largely supplanted RPLND for the purposes of staging. However, it carries a 15–20% false-negative rate. MRI also has been used and may ultimately replace CT in children for diagnosis and follow-up due to the high ionizing radiation of CT and risk of future malignancy. Prepubertal testis tumors are staged using the Children’s Oncology Group staging system.

Answer 36

Prepubertal testis tumors are staged using the Children’s Oncology Group staging system:

Answer 37

Carcinoma in situ (CIS) of the testis is a premalignant lesion. Testicular cancer is reported to develop in at least 50% of testes known to harbor CIS. CIS is seen in patients with UDT and DSD, conditions that are known to carry a higher risk of testicular cancer than that in the general population. Testicular microlithiasis (Fig. 51.11) may be associated with an increased risk of CIS as well as testicular germ cell tumors. The association between microlithiasis and cancer risk remains a matter of debate, although some series hint toward a potential causal association. The presence of microlithiasis in a testis is not conclusively considered premalignant. Rather, it appears to be a marker of an increased risk of cancer, which may be increasingly important in infertile men with atrophic testes or those with known testicular cancer and microlithiasis in the contralateral gonad. CIS is stimulated by endocrinologic changes during puberty. However, the natural history of CIS in prepubertal testes is less clear. When a testicular biopsy at the time of orchiopexy is performed, CIS is seen in 0.36–0.45% of cases. The prevalence of CIS in adult men with a history of UDT is 2–4%. If CIS is identified in a prepubertal testis, it is typically managed with annual testicular examinations and testicular US. In postpubertal men, some clinicians recommend that these patients undergo biopsy of the contralateral testis and unilateral orchiectomy. If biopsy of the remaining testis also reveals CIS, they recommend 18–20 Gy of radiation treatment. The fertility implications of this treatment option can be significant, and the decision has to be taken in the context of cancer risk against need for hormone supplementation and infertility.

Answer 38

YSTs are also known as endodermal sinus tumors, embryonal adenocarcinomas, orchioblastomas, or Teillum tumors. Most occur within the first 2 years of life. Grossly, YSTs are firm and yellow/white. Microscopically, they are characterized by Schiller–Duval bodies and stain positive for AFP. Contrary to the behavior of embryonal carcinoma in adults, YST (which is histologically similar) in children has a more indolent course and spreads hematogenously. Approximately 95% of YSTs are confined to the testis, and metastases to the retroperitoneum are uncommon (5%). The lungs are the most common site of distant metastasis, and retroperitoneal metastases are seen only 5% of the time. The 5-year survival for YST approaches 99%. The standard diagnostic and therapeutic procedure for a YST is radical inguinal orchiectomy, and this alone is usually curative in children. To minimize the risk of metastases during manipulation, the spermatic cord is clamped or ligated immediately on entry into the inguinal canal. Staging of YST requires abdominopelvic CT and chest radiography (CXR), histologic examination of the radical orchiectomy specimen, and determination of serum tumor markers. Stage I Stage I tumors are limited to the testis and thus are usually cured by radical inguinal orchiectomy. Tumor markers are measured monthly and CXRs obtained every 2 months for the first 2 years. Abdominopelvic CT or MRI scans are obtained every 3 months for the first year and every 6 months for the second year, although this practice may be overly aggressive in a child whose tumor markers remain normal. After 2 years without recurrence, followup may be extended to every 6 months or yearly. Traditionally, RPLND was recommended for boys with unknown or normal markers at diagnosis to confirm stage I disease. Although confirmatory RPLND may still be considered, it is used less often because stage I YST has a high likelihood of stage I presentation (85%), has propensity for hematogenous spread to the lungs, and because RPLND has a high complication rate in children. The risk of recurrence is approximately 20% and almost always can be salvaged with chemotherapy. Stage II Stage II disease includes those tumors with residual disease in the scrotum or high proximal inguinal cord, node involvement on imaging, or persistent elevation of tumor markers after orchiectomy. Tumors diagnosed and treated with trans-scrotal biopsy should be considered stage II because trans-scrotal violation alters the normal lymphatic drainage of the tumor. Lymphatic drainage of the testis is to the retroperitoneal nodes, whereas the scrotum drains to the inguinal nodes. All patients with stage II disease should receive combination chemotherapy with cisplatin, etoposide, and bleomycin (PEB). Due to significant ototoxicity and nephrotoxicity with cisplatin, the UK Children’s Cancer Study Group substituted carboplatin for cisplatin and was able to maintain a 100% event-free survival at 5 years. In rare cases with a persistent retroperitoneal mass or elevated AFP after chemotherapy, an RPLND should be considered. Stage III Stage III disease includes enlarged retroperitoneal spread (lymph node >4 cm) seen on imaging studies. Biopsy is used to confirm suspected nodal metastases, such as with lymph nodes >1–2 cm on CT. Stage IV Metastases beyond the retroperitoneum or to any viscera defines stage IV disease. For both stage III and stage IV disease, chemotherapy follows the same protocols as for stage II disease, followed by RPLND. The overall survival approaches 100%.

Answer 39

Teratomas account for over 40% of testicular tumors in prepubertal children. Histologically, teratomas are composed of all three layers of embryonic tissue: ectoderm, endoderm, and mesoderm. Grossly, they can contain differentiated tissue such as cartilage, muscle, bone, and fat. A cystic component also may be present. Before puberty, they follow a benign course and can be cured with testis-sparing surgery. Long-term mean follow-up of 7 years has demonstrated no tumor recurrence in the ipsilateral or contralateral gonad with a testis-sparing approach in one study. Also, no radiographic follow-up is recommended for prepubertal patients who undergo partial orchiectomy. If one accepts the dysplasia theory, however, whether benign or malignant, unilateral testicular tumor could pose an increased risk for the contralateral testis. On the other hand, when a child is seen at or after puberty, radical inguinal orchiectomy is indicated because the teratoma can follow a malignant postpubertal course. The enucleated tumor should always be sent for frozen section examination. If immature elements or pubertal changes are seen (i.e., spermatogenesis on the normal seminiferous tubules surrounding the margin), radical orchiectomy should be performed. Overall disease-free survival after orchiectomy is excellent. An elevated AFP or focus of YST may indicate potential recurrence. These patients can then be salvaged with platinum-based chemotherapy with 5-year survival rates in excess of 90%. Epidermoid cysts comprise about 15% of pediatric testis tumors, and as monodermal teratomas, follow a benign course. As such, a testis-sparing approach can be taken.

Answer 40

Mixed germ cell tumors account for up to 20% of pediatric/adolescent germ cell tumors, almost exclusively in postpubertal patients. Teratocarcinoma can be seen in an operatively-corrected UDT and may contain any mixture of YST, embryonal carcinoma, choriocarcinoma, and seminoma. Up to 80% of these tumors are confined to the testis at presentation. Foci of choriocarcinoma confer a poorer prognosis. The value of RPLND in children has been challenged over the years, and management in children/ adolescents tends to follow management guidelines established for adult patients in which these neoplasms are more common.

Answer 41

Seminoma is rare in children but is the most common tumor in an uncorrected abdominal UDT. Seminoma is treated with radical orchiectomy and retroperitoneal radiation, following guidelines for adult patients.

Answer 42

Leydig cell tumors Leydig cell tumors are one of the most common nongerm cell tumors (NGCTs). The peak incidence in boys occurs from ages 5–9 years. The clinical triad includes a unilateral testicular mass (90–93%), precocious puberty, and elevated 17-ketosteroid levels. As these tumors produce testosterone and occasionally other androgens, roughly 20% of patients may have signs of precocious puberty and gynecomastia. Precocious puberty may also be caused by pituitary lesions, Leydig cell hyperplasia, and CAH so the pituitary/adrenal axis must be evaluated by assaying for 17-ketosteroids, FSH, LH, and performing a dexamethasone suppression test. Reinke crystals on histologic examination are pathognomonic for this tumor and can be found in 35–40% of all patients. When diagnosed preoperatively, testis-sparing enucleation may be considered because these tumors tend to follow a benign course. Granulosa cell tumor The granulosa cell tumor also has a benign course and can be managed with testis-sparing surgery. This tumor should be suspected in neonates with scrotal swelling, normal age-adjusted AFP levels, and a complex, cystic, multiseptated, hypoechoic mass on testicular US. Sertoli cell tumor The Sertoli cell tumor is a rare form of NGCT. A small percentage of patients have gynecomastia, though these tumors are typically not as hormonally active as Leydig cell tumors. The clinical course is usually benign in children under 5, and tumors can be managed with testis-sparing surgery. Older children, however, should have a metastatic evaluation with imaging. Gonadoblastoma Gonadoblastoma is a form of NGCT usually associated with a DSD and typically occurring in dysgenetic gonads. The patients are typically 46XY phenotypic females with complete androgen insensitivity syndrome (CAIS) with intraabdominal testes who undergo virilization at puberty. Up to one-third of patients have bilateral gonadal lesions. The germ cell component of these tumors carries a 10% risk of malignant degeneration. Historically early gonadectomy was recommended, especially if the patient has been raised as a female. Currently, however, there is significant debate about the timing and need for gonadectomy. Some surgeons advocate for the preservation of the gonads until adolescence as puberty can be aided by the peripheral conversion of androgens to estrogens. Others, including many affected patients, are electing to preserve their intra-abdominal gonads indefinitely. Patients with mixed gonadal dysgenesis raised as males should have their streak gonads and UDTs removed, though some suggest that scrotal testes can be preserved because they are less prone to malignancy and can be surveyed more easily.

Answer 43

Lastly, testicular microlithiasis can be seen in conjunction with testicular tumors (seen synchronously in 15–45% of patients), but is also seen incidentally in 5% of healthy young men. While the risk of testicular microlithiasis in children for the development of cancer is not well studied and reported numbers are small, there appear to be low-risk and high-risk individuals. Boys who have (1) atrophic or dystrophic testes, (2) known chromosomal abnormalities, (3) contralateral testis cancer, and possibly (4) a history of UDT need closer follow-up with routine serial examination and scrotal US. Education about testicular self-examination is important for these patients. In patients without known risks factors, however, routine testicular self-examination is generally all that is recommended.

Answer 44

AFP is a glycoprotein produced by the fetal yolk sac, liver, and gastrointestinal tract. It is elevated in a variety of benign and malignant diseases, including YSTs of the testis. The half-life of AFP is approximately 5.5 days, and the normal adult levels (<10 ng/mL) are not achieved until around 10 months of age. β-hCG is a glycoprotein produced by some seminomas and mixed germ cell tumors, as well as choriocarcinomas. Its half-life is approximately 24 hours and is normally not detected in significant amounts in boys (<5 IU/L). H&A — AFP is synthesized mainly in the yolk sac and the liver of the fetus. The synthesis of AFP begins at 6 to 7 weeks of fetal life and serum AFP levels reach their maximum level at 13 weeks (about 300mg/dL) and decrease gradually until birth. Almost all of the amniotic AFP in the early fetal life originates from the yolk sac and in later fetal life from the liver. The synthesis of AFP does not cease entirely at term, but a small amount of AFP is continuously synthesized until 8 months of age, when the serum AFP levels approach adult levels. Ohama, et al AFP Levels in Normal Children

Answer 45

Orchidopexy is performed in order to accomplish five goals, namely to: 1) decrease the risk of torsion, 2) to decrease the risk of trauma, 3) to restore normal anatomy and scrotal appearance, 4) to address the increased risk of testicular cancer, and 5) to improve the reproductive function of the involved testicle. The decreasing age of recommended orchidopexy was based mostly on animal studies showing improved hormonal and reproductive function of the testicle with earlier orchidopexy. However, in the last decade, randomized trials comparing orchidopexy at 9 months versus 3 years of age have shown better testicular growth as well as histologic evidence of increased numbers of germ and Sertoli cells with earlier orchidopexy. These have led to the recommendation to perform orchidopexy between 6 and 12 months of age. It has been difficult however to prove that earlier orchidopexy has led to lower infertility rates because adults with a history of unilateral orchidopexy have infertility rates that are very close to the overall male population, about 10%. In addition, the median age for orchidopexy continues to be significantly higher than the recommended age because of delays in detection and referral. Sherif

Answer 46

Cryptorchidism is the most prominent risk factor for testicular cancer and accounts for 5%–10% of all testicular cancer cases in males. Orchidopexy early in life has been traditionally viewed as improving testicular cancer detection, rather than testicular cancer incidence. However, more recent population studies have revealed a steadily increasing risk of cancer with increasing age at orchidopexy. There is also strong evidence that the location and duration of an undescended testicle strongly influence cancer risk. The risk is higher in abdominal testicles and in patients who enter puberty with an undescended testicle. This has resulted in the recommendation to perform an orchiectomy for an abdominal testicle discovered at puberty, since the rate of dysplasia in these testicles is very high. Some recommend the same for canalicular testicles, but another option in that situation is to perform a testicular biopsy at the time of orchidopexy. Sherif

Answer 47

An undescended testicle may also be identified in a baby boy with gastroschisis and may be eviscerated along with the bowel. Gentle lysis of any adhesions between the testicle and the bowel and placement of the testicle as close as possible to the deep ring, at the time of silo placement or abdominal wall closure, is the recommended intervention. Remarkably, many of these testicles will descend spontaneously into the scrotum. If they don’t descend by 1 year of age, an abdominal exploration may be necessary to divide adhesions and mobilize the testicle at the time of orchidopexy. Sherif

Answer 48

When confirming testicular torsion at operation, the surgeon must decide whether to perform an orchidopexy or orchiectomy. The first step is to detorse the testicle by rotating it away from the midline (rotating a right testicle counterclockwise and a left testicle clockwise), and wrap it in wet warm gauze. A testicle that improves should be salvaged, while a frankly necrotic one that shows no signs of recovery should be removed. There is some controversy regarding what to do with a testicle that shows only minimal improvement but no signs of parenchymal bleeding or definite viability. In prepubertal boys, an orchidopexy should be performed. In postpubertal boys, a concern exists regarding the potential development of antisperm antibodies that may then harm the healthy contralateral testicle and further diminish fertility. These concerns are based mostly on animal studies, and the physiologic evidence for this potential autoimmunity in humans is uncertain. A more recent approach to the questionably viable testicle mimics the fasciotomy approach to an ischemic limb. The tunica albuginea (testicular fascia) is divided longitudinally along the entire length of the testicle and a tunica vaginalis flap is used to cover the defect. Early experience with this approach seems promising. Sherif

Answer 49

C Gubernaculum is attached at lower pole of testes. Processus vaginalis grows caudally into the gubernaculum. Process of descends accomplished by 35 weeks. Androgen control regression of cranial suspensory ligaments. Mullerian inhibiting substance causes enlargement of gubernaculum. Syed/MCQ

Answer 50

D Microcephaly leads to undescended testes because of low gonadotropin production. Syed/MCQ

Answer 51

E Intra-abdominal testes is an arrest in descent, so it is an undescended testes. Syed/MCQ

Answer 52

C Cremesteric muscle contraction causes retraction. Stimulation of cutaneous branch of genitofemoral nerve is responsible. Retractile testes are descended testes, but careful follow-up is required because it does not always remains descended. Sometimes fixation of testes is required. Syed/MCQ

Answer 53

E Incidence of torsion in undescended testes is 20 percent because of mobility of testes in superficial inguinal pouch. Syed/MCQ

Answer 54

B Beta HCG has 10 percent and LHRH has 10–50 percent success rate. Syed/MCQ

Answer 55

D Most testicular tumours in childhood occur in normal testes. Syed/MCQ

Answer 56

Testicular descent is believed to occur in two stages. The first occurs between 8 and 15 weeks of gestational age. During this phase, the testicle travels from a perirenal position to the internal inguinal ring. The second stage occurs in the late third trimester, when the testicle migrates through the inguinal canal into the scrotal sac. This final migration may not occur until several months after birth, providing the rationale for a period of observation in boys diagnosed with cryptorchidism at birth. Androgenic stimulation is considered essential in facilitating distal migration of the testicle. Therefore anything that interferes with androgen secretion or function may increase the risk of cryptorchidism. For example, maternal exposure to polybrominated diphenyl ethers, which are flame retardants with antiandrogenic properties found in a number of household products, have been associated with cryptorchidism. Failure of testicular descent may in fact reflect diffuse abnormalities in the affected testicle, as epididymal anomalies have been identified in more than half of undescended testicles. Sherif

Answer 57

Undescended testicles are broadly classified as palpable or nonpalpable, with an approximate frequency of twothirds and one-third of all cases of cryptorchidism, respectively. A testicle palpable in the inguinal canal is classified as a canalicular testicle. Standard treatment for this condition is an inguinal orchidopexy, as shown in Figure 48.3 in an 8-month-old boy. The challenge that may be encountered in some cases is gaining adequate length despite an inguinal position. The testicle is almost always tethered by the testicular vessels, not the vas. In fact, in many cases, the vas may be quite redundant, known as the long looping vas. This has to be kept in mind during division of the gubernaculum, which is the first step in mobilizing the testicle. A number of maneuvers may be used to gain adequate length. Although the patent processus vaginalis in cases of cryptorchidism is usually trivial, its separation from the vas and vessels and high ligation will aid in testicular mobilization. Skeletonization of the vas deferens and testicular vessels by dividing the intervening adventitial tissue and carrying the mobilization into the retroperitoneum proximal to the deep ring, using the tip of a wet sponge, is also useful. Finally, a Prentiss maneuver can medialize the vas and vessels and provide a direct route to the scrotum. To perform this maneuver, the epigastric vessels are ligated, the deep ring is opened medially, the cord structures are medialized, and the deep ring is closed lateral to the cord structures. Success rates of inguinal orchidopexy should be above 90%. Sherif

Answer 58

In postpubertal testicular germ cell tumors, the actual values of the serum markers have prognostic significance. AFP <1,000 µg/L, hCG <5,000 mIU/mL, and LDH <1.5 times upper limit of normal are associated with good-prognosis lesions. Sherif

Answer 59

Similar tumors in descended testicles have prompted a significant practice change from radical orchiectomy for testicular masses in all boys to a potential testicle-sparing approach in infants and toddlers. If preoperative markers are normal, an inguinal approach is still used with control of the cord structures at the deep ring. However, after eviscerating the testicle through the inguinal incision, a biopsy of the tumor is completed. If frozen section confirms the absence of malignant elements, which is almost always the case with preoperative normal tumor markers, the tumor may be enucleated from the testicle and the testicle placed back in the scrotum. This approach is similar to resection of benign ovarian teratomas in girls. Other benign testicular lesions in this age group include epidermoid cysts, dermoid cysts, and Leydig cell hyperplasia. Sherif

Answer 60

The COG staging system is used for prepubertal tumors. Stage I tumors are those that are completed resected following by normalization of tumor markers. The tumor is classified as stage II if resection is not associated with normalization of markers or if there is lymph node involvement less than 2 cm in size, stage III if retroperitoneal lymph node involvement larger than 2 cm is present, and stage IV if there is distant metastasis. Patients who present with stage II or higher receive chemotherapy. Retroperitoneal lymph node dissection is very rarely indicated for prepubertal tumors. Survival of patients with prepubertal tumors approaches 100%. Almost all malignant prepubertal tumors demonstrate elevated AFP and yolk sac tumor histology. Stage I tumors are sufficiently treated by radical orchiectomy. AFP levels are followed after orchiectomy. Recurrences are rare, but quite easily salvaged by platinum-based chemotherapy. Figure 59.8 shows a right testicular yolk sac tumor in a 14-month-old boy seen for a presumptive hydrocele. The patient was adequately treated by radical orchiectomy with normalization of AFP levels and absence of recurrence after several years of follow-up. Sherif

Answer 61

Unlike prepubertal testicular tumors, pubertal or adolescent tumors are highly likely to be malignant. In distinction to adult testicular tumors, non-seminomatous germ cell tumors predominate. Most are mixed germ cell tumors, with a teratoma component. Embryonal carcinoma is the most common malignant element in mixed germ cell tumors, and may be associated with high AFP and hCG levels. Yolk sac tumor and choriocarcinoma may also be seen. The latter is the most aggressive, with metastasis to the lungs and mediastinum quite common on presentation. Brain metastasis can also occur and result in cerebrovascular accidents due to tumor hemorrhage. Imaging and pathology of a mixed germ cell testicular tumor with predominant embryonal carcinoma in a 15-year-old boy is shown in Figure 59.9. The patient did not report the testicular mass and presented instead with supraclavicular lymphadenopathy. Imaging at presentation showed a large mediastinal mass and multiple lung lesions. One year after orchiectomy and chemotherapy, he suffered a cerebrovascular accident due to a large metastatic lesion in the right frontal lobe, and underwent a craniotomy and resection of the brain lesion. Despite maximal treatment, he succumbed to metastatic disease throughout the central nervous system 2.5 years after diagnosis, at age 17. Sherif

Answer 62

Cryptorchidism is a significant risk factor for adolescent germ cell tumors, increasing the risk by 4–8 times. The timing of orchidopexy has been found to influence the risk, with postpubertal orchidopexy conferring the highest risk. Other risk factors include family history, disorders of sexual differentiation, and Down syndrome. The predisposing histology of these tumors is referred to as ITGCN, a lesion not seen in prepubertal tumors. The approach to pubertal testicular tumors has been presented in the index case. Staging follows the AJCC system and relies on the characteristics of the primary tumor, the status of the lymph nodes, the presence of metastasis, and the serum tumor markers (TNMS). The presence of lymphovascular invasion is specifically examined as a tumor characteristic and is associated with worse prognosis. The size and number of lymph nodes involved is used to characterize the nodes. The level of elevation of tumor markers is used to determine the serum marker status. These four factors are used to calculate the stage. In addition, the International Germ Cell Cancer Consensus Group (IGCCCG) has developed a risk stratification system that takes into account the location of the tumor, the metastatic status, and the level of tumor marker elevation. Testicular tumors that present without non-pulmonary visceral metastasis and with marker elevations <1,000 µg/L for AFP, <5,000 IU/L for hCG, and <1.5 × upper limit of normal for LDH are associated with good prognosis and a 5-year overall survival rate above 90%. Those that present with non-pulmonary visceral metastasis or with very high levels of tumor markers (AFP> 10,000 µg/L, hCG >50,000 IU/L, LDH >10 × upper limit of normal) are associated with poor prognosis and a 5-year overall survival of approximately 50%. Intermediate prognosis lesions lie between these two risk categories and have an overall 5-year survival of 80%. Stage I pubertal tumors are treated by radical orchiectomy and observation with serial tumor marker levels. In this group, lymphovascular invasion is associated with a higher risk of relapse. However, relapses are readily salvaged by chemotherapy. Survival of stage I patients approaches 100%. The role of retroperitoneal lymph node dissection for higher stage tumors has diminished somewhat in recent years. Patients who present with retroperitoneal lymphadenopathy that resolves after BEP chemotherapy (Chemotherapy for germ cell tumors is platinum-based. The classic regimen is a combination of bleomycin, etoposide, and cisplatin, or BEP) may be observed. Persistence of adenopathy is an indication for a retroperitoneal lymph node dissection. If tumor markers are normal in this scenario, histology often reveals mature teratoma not amenable to chemotherapy. A testicular-sparing approach is not recommended in pubertal testicular cancer. Situational exceptions may include bilateral cancer, metachronous cancer in the contralateral testicle after radical orchiectomy, and tumor in a solitary testicle. Even in these situations, the risk of residual disease has to be weighed carefully against the benefits of potential fertility preservation. Given the toxicity of the treatment, any residual testicle may still not render the patient fertile. Sherif

Answer 63

Testicular microlithiasis is an interesting entity. Incidence in boys may be as high as 3%–4%. The condition seems more common in boys with a history of cryptorchidism or hydrocele. It is typically identified incidentally on an ultrasound performed for another indication and represents calcium deposition in the seminiferous tubules. The condition may be focal or diffuse, unilateral or bilateral. It is unclear if this at all predisposes to malignancy. Follow-up is best done by self-examination, but annual ultrasound evaluation of the testicles is also a reasonable option, particularly if there are risk factors for testicular cancer. Sherif

Answer 64

D The blastocyst forms primordial cells in week 1 of gestation. At week 3, cells form on the wall of the yolk sac. Actual migration of totipotential primordial germ cells does not occur until weeks 5–6, when cells migrate from the yolk sac along the mesentery to the gonads and then become gonadal or extragonadal. Abnormal migration may give rise to midline tumours, which mainly occur in the cervical, retroperitoneal, sacrococcygeal, mediastinum and pineal gland. SPSE 1

Answer 65

B AFP is produced by yolk sac (endodermal sinus) tumours and has a half-life of 4 days. Embryonal carcinoma, seminoma, dysgerminoma and choriocarcinoma can secrete β-hCG. In addition, lactate dehydrogenase (LDH) and ALP can also be elevated in patients with germinoma tumours. SPSE 1

Answer 66

A mediastinal teratomas are the most common site of extragonadal germ cell tumours in older children. They are found in the anterior mediastinum and present with airway symptoms. Germ cells make up 20% of all mediastinal tumours. About 80% of mediastinal germ cell tumours are benign; these occur with equal frequency in males and females. However, there is a male predominance (9 : 1) for malignant mediastinal germ cell tumours. Germ cell tumours are associated with Klinefelter’s syndrome (47,XXY). In patients with Klinefelter’s syndrome, the relative risk of having a malignant mediastinal germ cell tumour is 66.7. At least 8% of male patients with primary mediastinal tumours have Klinefelter’s syndrome (50 times the expected frequency). SPSE 1

Answer 67

A In adults retroperitoneal lymph node dissection (RPLND) may be used as a primary treatment modality for low-volume non-seminomatous germ cell tumours localised to the retroperitoneum as well as a salvage therapy for residual masses following chemotherapy. In children a routine selective lymph node dissection is indicated for patients with enlarged nodes. RPLND may also be used in boys with persistently elevated levels of tumour markers after chemotherapy. In children diagnosed with paratesticular rhabdomyosarcoma over age 10, routine retroperitoneal lymph node dissection is indicated. SPSE 1

Answer 68

C Cryptorchidism is a risk factor for the development of testicular germ cell tumours. Ten per cent of testis tumours are associated with cryptorchidism. A quarter of the tumours associated with cryptorchidism are actually within the contralateral normally descended testis. The risk of testicular cancer in a boy with cryptorchidism is directly related to the degree of maldescent. The risk is 1 in 20 if the testis is high within the abdomen and decreases to 1 in 80 if the testis is within the inguinal canal. After radical orchidectomy, patients with stage I seminoma are treated only with external beam radiotherapy to retroperitoneal and ipsilateral pelvic lymph nodes, typically 2500 cGy, which gives nearly 100% survival rate, chemotherapy is not required. Although in adults the incidence of testicular cancer is much higher in white people, among paediatric patients the incidence is equal among both black and white people. SPSE 1

Answer 69

D Trans-scrotal biopsy should not be performed; an inguinal approach to testicular lesions should be done. markers including AFP, β-hCG, ALP and LDH are often helpful in testicular cancer. Chest CT has now replaced chest X-ray as a means of evaluating the chest for spread. Abdominal CT scan is necessary to look at retroperitoneal nodes for staging purposes. Testicular ultrasound also helps differentiate a solid testicular mass from other masses – which may include hydrocele, hernia and varicoceles. SPSE 1

Answer 70

E Testicular tumours can be divided into those arising from germ cells (yolksac tumours and teratomas in children) and those arising from gonadal stroma (leydig cell, Sertoli cell or granulosa cell tumours). Yolk-sac tumours are the commonest tumour in childhood and are usually associated with raised levels of alpha-fetoprotein. The β-hCG levels are rarely raised in paediatric testicular tumours. The incidence of tumours is increased with intra-abdominal testes but in the order of 5–10 times normal. Gonadoblastoma is a rare testicular tumour (1% of all tumours), which occurs almost exclusively in dysgenetic gonads usually associated with intersex disorders. It frequently develops into seminoma. While there is a lower incidence of malignancy in childhood, all tumours should be treated as such and explored via an inguinal incision. SPSE 1

Answer 71

B Testicular tumours in prepubertal boys tend to be more benign than those occurring in adults. Certain benign tumours such as teratoma and leydig cell tumours can be treated with testicle-sparing surgery, using an inguinal approach and frozen section. The inguinal approach involves opening the canal and mobilising the vessels. These are then occluded with a soft clamp. The testis is mobilised and biopsied if required before ligation and division of the vessels. The lymphatic drainage of the testis is to the retroperitoneal nodes along the iliac vessels. It is important therefore not to approach the testicle via the scrotum as lymphatic spread may also occur to the superficial inguinal nodes. most testicular tumours present with increase in size of the testicle and sometimes discomfort. A hydrocele is present in 10%–20%. The testes can also be a site of secondary tumours particularly T-cell leukaemia. SPSE 1

Answer 72

D Descent of the testes occurs in two phases: intra-abdominal descent, controlled by müllerian inhibiting substance, and inguinoscrotal descent which depends on the gubernaculum. Testes in the line of descent but which are not fully descended have been called ‘arrested’ testes and those which are outside the line of descent, ‘obstructed’ or ‘ectopic’. The commonest position of these is the superficial inguinal pouch (a space between tissue planes superficial to the external oblique). other positions include perineal, femoral or pubopenile. The testes may not be fully descended at birth, but in the term baby they should have fully descended by 4 months (later in pre-term babies). At orchidopexy for undescended testes, abnormalities of fusion between the testis and epididymis are not uncommon and may be related to later fertility. SPSE 1

Answer 73

A Peutz–Jeghers’s syndrome is an autosomal dominant condition characterised by mucocutaneous pigmentation and hamartomatous intestinal polyps. It is associated with some types of testicular tumour. The others are just some of the associations of cryptorchidism, and also include posterior urethral valves and cerebral palsy. SPSE 1

Answer 74

E Examination normally begins by looking at and palpating the scrotum. If the scrotum is empty palpate the inguinal area. If a testicle is palpable ‘milk’ the testicle to the external ring by firmly running fingers from the anterior superior iliac spine to the pubic tubercle. The testis can be gently grasped between the forefinger and thumb of the other hand and put under traction to find the lowest point of descent. If a testicle is not palpable in the inguinal area, other positions of an ectopic testis are palpated. In younger children, gentle abdominal pressure can sometimes force a testis lying just inside the internal ring, into the canal. If the testis is still impalpable, measuring the contralateral testis may indicate the likely presence of the impalpable testis (if absent there is often compensatory hypertrophy). The cremasteric reflex may be helpful to indicate to parents the mechanism of a retractile testis, but is not useful clinically. SPSE 1

Answer 75

C The retractile testicle is probably the commonest diagnosis in patients referred with undescended testes. It is characteristically a normal-sized testis that can be manipulated to a normal position in the scrotum and will remain there without immediately springing back into the groin (suggesting tension of the cord). These patients usually have a very noticeable cremasteric reflex whereby stimulation of the medial aspect of the thigh results in contraction of the dartos and cremasteric muscles pulling the testis from the scrotum. This reflex is mediated by the genitofemoral nerve. There is an incidence (~15%) of later ascent of the testes. SPSE 1

Answer 76

E laparoscopy is generally considered the most effective investigation for intra-abdominal testes. In children who are difficult to examine, ultrasound can be helpful if the testis is within the canal. MRI can detect intra-abdominal testes but is unreliable (relatively high false-negative rate). At laparoscopy the testis can be assessed in terms of position (whether a single-stage or two-stage procedure is preferable) and size (some small testes may be best removed). It is essential to see both blind-ending vas and vessels before concluding the testis in not in the abdomen. SPSE 1

Answer 77

B Orchidopexy is usually planned for between 12 and 18 months, although some will perform as early as 6 months. There is an incidence of late (after the age of 2 years) orchidopexy because of the phenomenon of the ascending testis (an initially descended testes that gradually retracts from the scrotum as the child grows). orchidopexy can be performed by the inguinal or scrotal route. For the latter the testis should be able to be manipulated into the scrotum under anaesthetic. The operation then consists of dissecting the coverings from the cord, dissecting and dividing any associated patent processus vaginalis, dividing the gubernaculum and fixing the testis in a subdartos pouch. The tunica is everted and the testicle delivered (this allows inspection of the testis, the removal of any hydatids/appendices, and promotes fixation). The specific complications of the procedure are damage to the vas, re-ascent and testicular atrophy. With a standard orchidopexy the risks of atrophy are around 2%–5%, depending on the position of the testis. Atrophy rates are higher than this for two-stage procedures (10%–20%). It appears that prepubertal orchidopexy reduces the rate of subsequent testicular tumour compared with later orchidopexy. In addition, orchidopexy reduces the risk of seminoma (which accounts for two-thirds of malignant tumours that develop in undescended testes). SPSE 1

Answer 78

E Testicular trauma is not common in children and is usually due to blunt trauma. It is important to distinguish between a scrotal haematoma (best managed conservatively) and haematocele secondary to rupture of the tunica albuginea (best managed by operative drainage, debridement of the seminiferous tubules and closure of the tunica). The distinction may be difficult clinically, in which case ultrasound is useful to detect damage to the tunica (but not to indicate blood flow). In the very rare instance where a testicle is avulsed, it needs to be reimplanted within 4–6 hours to ensure viability. SPSE 1

Answer 79

D A varicocele is an abnormal dilatation of the pampiniform plexus of veins in the scrotum. Ninety per cent occur on the left side and they become increasingly prevalent around puberty, although they have been reported in the under-10 age group. many small varicoceles are asymptomatic; they may, however, cause discomfort. There is evidence that larger varicoceles can impair testicular growth and may lead to reduced fertility. Surgery is usually reserved for symptomatic larger varicoceles particularly if there is evidence of poor testicular growth. There are several possible treatment options, including retrograde or antegrade embolisation or sclerotherapy, microsurgical inguinal ligation, open or laparoscopic ligation. There are advantages and disadvantages to each technique and none has been proven superior to the others. SPSE 1

Answer 80

A These are all described complications following varicocele surgery. The commonest complications are recurrence (around 5%–10%) and hydrocele formation (5%–20%). The incidence and type of complications vary according to technique. Focal testicular necrosis is specific to antegrade scrotal sclerotherapy for example. SPSE 1