Undescended Testis and Testicular Tumors Flashcards
Patients with an undescended testicle:
A. Should have an orchiopexy done within the first 3 months of life
B. Need orchiopexy to decrease the incidence of testicular cancer in the future
C. Are not at increased risk for testicular torsion if unrepaired
D. Have no difference in fertility compared with the normal population if the other testicle is in normal position
E. Can be successfully treated with hormone therapy alone.
ANSWER: D
COMMENTS: Undescended testes are found in up to 4.5% of infants, but drop to less than 1% by the age of 1 year. For this reason, surgical correction of undescended testes is deferred until 6 to 9 months of age.
The rate of undescended testes in premature infants is significantly higher.
Undescended testes are at an increased risk for developing cancer in the future, whether or not they are surgically corrected. However, orchiopexy makes examining the testicle for abnormalities easier.
The risk for torsion of a nonrepaired undescended testicle is as high as 20%.
Fertility rates are decreased compared with the general population if bilateral undescended testes are present; however, a unilateral undescended testicle with a normally positioned opposite testicle is normal.
Hormonal therapy with agents such as testosterone, beta-human chorionic gonadotropin (β-hCG), and luteinizing hormone-releasing hormone has been attempted, but the success rates remain low. Therefore if an undescended testis is present after 6 to 9 months of age, surgical correction should be offered.
What is the differential diagnosis for groin and scrotal swellings in children?
It is broad. Includes inguinal hernia, hydrocele, infections (such as epididymitis), testicular tumors, inguinal lymphadenopathy or abscess, and medical causes of scrotal swelling such as Henoch-Schönlein Purpura.
What should be established on history in children with groin or scrotal swelling?
In babies, gestational age at birth should be determined as inguinal hernias are more common in premature infants. Acuity of the swelling should be established, as well as a history of change in swelling size. It should be determined if there is any associated pain.
What should be established during examination?
Clinicians should confirm that the child is clinically well, with no systemic evi- dence of infection, abdominal distension or bowel obstruction. The swelling should be assessed for its relationship to the inguinal crease, the external ingui- nal ring and the testicle. Lymphadenopathy tends to occur lateral to the inguinal crease, and inguinal hernias will be apparent at the inguinal ring, occasionally descending into the scrotum. It should be established whether or not the swelling is reducible into the abdomen, which is diagnostic of hernia. Hydroceles transil- luminate. A painless mass within the scrotum should raise suspicion of a possible neoplasm.
What is the cause of a hydrocele?
In children, a partially patent processus vaginalis, with fluid trapped between the
layers of the tunica vaginalis is the most common cause.
How is the diagnosis of hydrocele made?
Hydrocele is a clinical diagnosis. On history there may be swelling of the scrotum that may be static in size, or increase with ambulation. Findings on examination consist of a simple cystic structure in the scrotum or related to the spermatic cord, which typically cannot be reduced, and transilluminates.
Is imaging necessary in cases of suspected hydrocele?
Imaging is not generally indicated, although ultrasound should be utilized if there
is a suspicion of an intra-scrotal mass
When is surgery indicated in hydrocele?
Most hydroceles will close spontaneously by two years of age. If a hydrocele per- sists beyond this age, surgery may be offered. There is no evidence that hydroceles cause testicular damage or other morbidity and the natural history of hydroce- les beyond 2 years of age is poorly documented. As such, it is safe to observe hydroceles if surgical correction is not desired. An absolute indication for repair is ipsilateral inguinal hernia.
How are hydroceles repaired?
By ligation of the processus vaginalis (usually through an inguinal incision) and wide opening of the distal sac. Cord hydroceles should be deroofed or excised. Secondary or non-communicating hydroceles may be addressed through a scrotal approach with opening and eversion of the sac.
What is the definition of an acute scrotum?
Acute scrotal pain with or without swelling or erythema.
What is the differential diagnosis for the acute scrotum?
Testicular torsion, torted appendix testis, epididymitis or viral orchitis, pain fol- lowing scrotal or testicular trauma, incarcerated inguinal hernia and some medical conditions such as idiopathic scrotal oedema and rheumatoid purpura.
What should be established during history taking in these patients?
Acuity of pain, history of previous symptoms, history of recent groin trauma, the presence of a current or recent systemic illness and a history of urinary tract infec- tions or conditions that may predispose to these.
How does clinical examination help in determining the cause of acute scrotum?
It can be difficult to differentiate between causes of acute scrotum on clinical examination. Clinicians should assess patients for evidence of systemic illness or fever. A horizontal-lying testicle and absence of cremasteric reflex favours tes- ticular torsion. Relief of pain with elevation of the scrotum favours epididymi- tis. In the early stages, patients with epididymitis may have pain localized to the epididymis.
What additional testing can be employed in the investigation of acute scrotum?
Urinalysis may be useful in identifying patients with epididymitis, although abnor- mal analysis does not exclude torsion, and bland urine does not exclude orchitis.
Doppler ultrasound may exclude some patients with epididymitis, but is opera- tor dependent and the presence of arterial flow may be falsely reassuring in cases of early or intermittent torsion. High-resolution ultrasound may be useful in visu- alizing a twist in the spermatic cord.
Importantly, rapid detorsion improves testicular salvage, and so transfer to the operating room should not be delayed to seek imaging in cases where the index of suspicion for torsion is high.
What is the treatment of testicular torsion?
Early scrotal exploration, detorsion and pexy of the affected testicle, and contralat- eral fixation. Fasciotomy and tunica vaginalis flap may be considered in borderline cases. In cases of frank testicular necrosis, orchiectomy should be considered.
What is the fate of the torted testicle?
Torsion results in impaired blood flow to and ischaemia of the testicle. Overall, the rate of testicular loss following torsion may be as high as 60%, once accounting for early orchiectomy and late atrophy. It is reasonable to counsel families that there is concern for testicular loss if exploration occurs more than 6 hours after onset of pain, and significant concern for loss at 10 hours after onset of pain [2].
What is the management of epididymitis?
In cases of positive urine bacterial culture, children should be treated and inves- tigated as for a UTI. Most cases, however, are self-limiting and no organisms are identified on urine culture.
What is the definition of an undescended testicle?
A testicle that is not present in the scrotum and cannot be brought into the scrotum with manipulation, or does not remain in the scrotum for any length of time after exhaustion of the cremaster.
How can undescended testicles be classified?
They may be palpable or impalpable. Palpable testes may be in the groin in the expected path of descent between the inguinal canal and the scrotum, they may be ectopic, or they may be retractile. Impalpable testes may be intra-abdominal, or absent entirely.
What is a retractile testis?
A testicle that can be manipulated into the scrotum and remains there for a period of time, but that returns to the groin due to an overactive cremasteric reflex. Orchidopexy is not indicated in retractile testes but their position should be moni- tored as they may become undescended.
What investigations should be performed in children with undescended testes?
Imaging is not indicated to confirm testicular position.
Children with features to suggest a difference in sexual differentiation (such as
bilateral impalpable testes, proximal hypospadias, bifid scrotum) should be inves- tigated accordingly.
What is the treatment for undescended testicles?
Orchidopexy is surgical placement of the testicle within the scrotum. This may be performed through a scrotal or inguinal approach in the case of palpable testes, or as a single- or two-stage laparoscopic procedure in the case of abdominal testes.
When should orchidopexy be performed?
Ideally, orchidopexy should be performed between the ages of 6 and 18 months [3].
What are the reasons for performing orchidopexy?
Undescended testicles may result in sub-fertility. Early orchidopexy, prior to 12 months of age, is recommended in order to improve preservation of fertility.
Boys with undescended testes also have increased risk for testicular malig- nancy. There is evidence that pre-pubertal orchidopexy may decrease the risk of malignancy, while also facilitates self-examination.
What is a varicocele?
The abnormal dilatation or tortuosity of the veins of the pampiniform plexus.
What is the cause of a varicocele?
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.
What problems do varicoceles cause?
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.
By what mechanism do varicoceles cause testicular dysfunction and sub- fertility? [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.
How should a clinician examine a patient with suspected varicocele?
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.
What imaging modalities can be used in the investigation of varicocele?
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.
How are varicoceles classified? [5]
There are 3 grades:
Grade I—palpable with Valsalva manoeuvre only
Grade II—palpable without Valsalva manoeuvre
Grade III—visible at a distance.
What are the indications for treatment of varicocele in adolescents? [6]
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.
What are the goals of treatment of varicocele?
To cause disruption of the internal spermatic drainage of the testicle, whilst pre-
serving the spermatic artery, vasal and differential vessels, and lymphatics.
What are the options for treatment of varicocele?
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.
What are the half-lives of BCHG and AFP?
AFP (produced by Lacunar Cells)
- Half life of 1.5d
B-HCG (produces by syncitiotrophoblastic cells)
- Half life of 5d
A 23-month old male is found to have an empty left hemiscrotum by his mother. On PE, his right testicle is in the scrotum, whereas the left is nowhere to be palpated. Ultrasound of the abdomen and pelvis was unable to locate the left testicle along the path of descent of the testis. He otherwise appears healthy. The clinician/surgeon advised a laparoscopic exploration surgery. At laparoscopy, the testis is found to be in the left paracolic gutter. What is the next step in management?
A. Perform the two-stage procedure; Do ligate the vessels and then perform the orchidopexy after 6 months.
B. Do the one-stage procedure; ligate the testicular vessels and do the orchidopexy
C. Orchiectomy for biopsy and repair the internal ring defect
A. Perform the two-stage procedure; Do ligate the vessels and then perform the orchidopexy after 6 months.
Complications of undescended testis include all below, except:
A. Affected endocrine function of Sertoli and Leydig cell.
B. Fall in number of germ cells.
C. Decrease in fertility.
D. Increased incidence of testicular trauma.
E. Decreased incidence of torsion of testes.
E. Decreased incidence of torsion of testes.
In the management of undescended testes, which of the following is false?
A. Diagnosis is usually clinical.
B. Beta HCG and LHRH has high success rate.
C. Orchidopexy is done in 6-12 months.
D. Orchiectomy, if testes is small and dysgenetic.
E. Intraabdominal testés has poor prognosis.
B. Beta HCG and LHRH has high success rate.
Regarding malignancy in undescended testes, all are true, except:
A. Relative risk in undescended testes is 5-10 times.
B. There is no conclusion that orchiectomy reduces the risk of malignancy.
C. Placement of testes in scrotum facilitates early diagnosis of malignancy, if it occurs.
D. Most testicular tumors in childhood occur in undescended testes.
E. The most common age range for cancer to occur in an undescended testes is 20-40 years.
?
Regarding testicular tumour marker, which of the following statements is not true?
A. Alpha-fetoprotein and beta-HCG both are glycoprotein.
B. Alpha-fetoprotein half-life is 5 days.
C. Beta HCG half-life is 24 hours.
D. Alpha-fetoprotein level comes to normal after 2 days of surgery.
E. B-HCG level comes to normal after 5–7 days of surgery.
.
What is the embryology of undescended testis?
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
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What is a retractile testis?
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
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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
How is non-palpable UDT managed?
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.
Generally, how should you manage a prepubertal patient with a testicular mass?
See algorithm below.
How are testicular tumors staged in prepubertal patients?
Prepubertal testis tumors are staged using the Children’s Oncology Group staging system:
What maneuvers can be done to gain adequate length for inguinal orchidopexy?
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
What is the COG staging system for prepubertal tumors?
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
What are the most common testicular tumors in pubertal or adolescent patients?
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
What is the approach to management of pubertal testicular tumors?
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
