Wilms tumor (Nephroblastoma) Flashcards
Overview
Embryonic, three-phase neoplasm (blastemal, stromal,
epithelial), characteristic of childhood
• The second most common embryonic tumor after
neuroblastoma
• 2-year survival: 81%
• It affects 1/10000 children under 15 years of age
• Associated with congenital malformations:
1.WAGR
2.Beckwith-Wiedemann syndrome
• Associated with urological abnormalities
WAGR
Wilms tumor,
aniridia,
genitourinary malaria,
mental retardation
Beckwith-Wiedemann syndrome
visceromegaly,
macroglossia,
hyperinsulinism
Clinical exam
• Asymptomatic abdominal tumor
• Weight loss
• Varicocele (left spermatic vein obstruction)
• Cardiac dysfunction (progression of tumor thrombus in VCI)
• Minor injuries can cause the tumor to rupture
• Examination of the abdomen: gentle palpation,
without pressure; round, smooth tumor mass in the
abdominal flank
• High blood pressure secondary to renin secretion
or compression of renal vessels
Laboratory data and paraclinical investigations
- Inconclusive blood and urine tests
- Abdominal Computed Tomography
- Abdominal Ultrasound
- Abdominal Magnetic Resonance
- Pulmonary x-ray
Staging
I - tumor limited to the kidney, completely resectable; intact renal capsule • II - tumor that extends beyond the kidneys, but completely resectable; • III - damage to the abdominal lymph nodes, incompletely resectable tumor • IV - hematogenous metastases or invasion of lymph nodes outside the abdominal region • V - bilateral renal impairment
Differential diagnosis
Other renal tumors (mesoblastic nephroma,
clear cell sarcoma, renal rhabdoid tumors,
renal adenocarcinoma, renal teratoma, diffuse
hyperplastic nephroblastomatosis, etc.)
• Retroperitoneal tumors (neuroblastoma,
teratomas, neuroectodermal tumors,
lymphomas, rhabdomyosarcomas, etc.)
Positive diagnosis
Based on the result of the
histopathological examination
Surgical treatment
Purpose: surgical staging and tumor resection
• Lymph node biopsy, liver examination (possible
metastases) and contralateral kidney (tumor
invasion)
• Wide abdominal, transperitoneal incision
• Nephrectomy with or without adrenalectomy
(depending on gland invasion),
nephroureterectomy, excision of perirenal adipose
tissue and lymph node dissection
• Bilateral nephroblastoma: conservative surgery
(total tumorectomy and partial nephrectomy)
• Sometimes the tumor may be inoperable due to
extension in the VCI
Undescended testis
Definition
permanent absence of the testis
from the scrotal bursa
Sexual differentiation - 7-8 weeks of gestation
• At the beginning of the third trimester of
pregnancy, the testicle migrates to the scrotal
region.
True undescended testicle
is located on the normal
descent path and has a normal insertion of the
gubernaculum testis
Ectopic testis
not on the normal path and has abnormal
gubernaculum insertion
Floating testicle
is lowered, but ascends into the
inguinal canal by triggering the cremaster reflex
Retractable testicle
can be brought into the scrotum by
manipulation, but resumes its high position
The ectopic position of the testicle
Incidence 4.3% of infants, 1% of 1-year-olds Significantly increased in premature infants More often on the right side • Complications Infertility Malignancy Testicular torsion Psychological effects
The ectopic position of the testicle
clinical examination and diagnostic
Clinical examination:
Purpose - identification of the presence / absence
of the palpable gonad (80-90% of the testicles are
palpable in the groin region); absence of the testis from
the scrotum, hypoplasic hemiscrotum
• Diagnostic
Clinical examination
Ultrasonography (scrotal, inguinal and abdominal)
Computed tomography / Magnetic resonance
Exploratory laparoscopy
The ectopic position of the testicle
Treatment
Treatment
Hormone therapy
hCG
Success rate 10-50%
Surgical treatment (orhidopexy)
Early (6-12 months) to prevent degeneration secondary to high temperature (3-4 degrees Celsius)
Classic / laparoscopic
General anesthesia (I.O.T. or laryngeal mask)
Classical orchidopexy (“open”)
1) Incision in the lower abdominal skin fold
2) Inguinal canal opening
3) Testicular identification and dissection
4) Elongation of spermatic vessels
5) Orchiopexy
6) Closing the deep groin
7) Parietoraphy
Laparoscopically assisted orchidopexy (for the
intra-abdominal testis)
1) “Mini” instruments - laparoscopy
2) Umbilical optic switch
3) 2 working exchanges in the abdominal flanks
4) Creation of capnoperitoneum (pneumoperitoneum)
5) Exploration of the peritoneal cavity
6) Identification of the testis and spermatic vessels
7) Dissection of spermatic vessels and vas deferens
8) Orhiopexy
9) Closing the deep groin
10) CO2 exhalation
11) Closure of the trocar sites
Fowler-Stephens procedure
Principle of intervention: spermatic vessel
sectioning (first step)
Testicular descent (second step)
Testicular vascularization is supported by
collateral sources
Orchidectomy
- for the dysfunctional testicular rudiment
- in case of the undescended testicle
discovered postpubertal
Orchidectomy
Intra / postoperative complications
- Impossibility of lowering the testicle into the scrotum
- Secondary testicular atrophy
- Testicular retraction outside the scrotum
- Compression of the vas deferens
- Hemorrhage
- Wound infection
Varicocele
Dilation of the testicular veins at the level of the
pampiniform plexus
• 90% on the left side, 9% bilaterally, 1% on the right side
• Increases the temperature in the testicle and hemyscroum (thermography), testicular atrophy, infertility
- Effects on the testicle: testicular hypotrophy, histological abnormalities, decreased intratesticular testosterone and infertility.
- Testicular arterial vascularization is triple: testicular artery, deferential artery, cremasteric artery
Primary and secondary causes of varicocele
• Primary causes: insufficiency of the valves of the left
testicular vein or compression of the left renal vein
(aorto-mesenteric forceps = a syndrome in which the aorta and mesenteric artery compress the renal vein)
• Secondary causes: abdominal tumors, hydronephrosis etc
Varicocele
Diagnosis
• Symptomatic varicocele: chronic pain or discomfort • Clinical examination: "worm lump" at the upper pole of the testicle (Robert Gross) • Scrotal Doppler ultrasound
Classification of varicocele
Grade 0 (subclinical) - impalpable
(non-palpable) varicocele, identifiable only by
ultrasound
Grade 1 - palpable varicocele only at the
Valsalva maneuver, with the patient standing
(orthostatism)
Grade 2 - palpable varicocele with the patient
standing (orthostatism)
Grade 3 - visible varicocele
Varicocele
Treatment
Ivanissevich: ligation of spermatic veins only
• Palomo procedure: ligation of testicular vascular pedicle (artery, veins, lymphatics)
• Both procedures can be performed in
an open manner and also in a laparoscopic version
• Embolization techniques
Varicocele
Postoperative complications
- Recurrence
- Reactive hydrocele
- Testicular atrophy
- Injury to the ilio-inguinal nerve
- Damage to the vas deferens
Hypospadias
• Common congenital anomalies (1/250 nn)
(birth defect)
• Urethra opens on the underside of the penis or below the penis
• Chordee (curvature of the penis on the ventral face)
• Foreskin / scrotal abnormalities
• Frequently associated with the undescended testicle and inguinal hernia
Classification
Depending on the location of the urethral meatus: Anterior (distal) Glandular Coronal (in the balanic groove) Distal (1/3 distal to penis) Middle (1/3 average of the penis) Posterior (proximal) Posterior penis (1/3 proximal to the penis) Peno-scrotal Scrotal Perineal
Urethral meatus abnormalities - abnormal location and configuration
• Penile curvature (cordea)
deficiency of the structures on the ventral face of the
penis; connective tissue cord
Urethral meatus abnormalities - abnormal location and configuration
• Abnormalities of the skin and scrotum
distal to the meatus, insufficiency of the
skin (contributes to the cords), absence of the brake; the proximal skin of extremely thin meat; excess skin agglomeration on the dorsal face
Hypospadias
treatment
- Meatoplasty
- Urethroplasty
- Chordee correction
- Preputioplasty (Foreskin plastic surgery)
• Anterior hypospadias MAGPI, GAP, Mathieu, Snodgrass • Posterior hypospadias Bracka • Penile curvature • Optimal age: 6-18 months
Hypospadias
Postoperative complications
- Meatus stenosis
- Urethro-cutaneous fistula
- Stricture of the urethra
- Diverticulum
Phimosis
Definition
the foreskin of the penis cannot stretch to
allow it to be pulled (the tight foreskin)
Phimosis
classification
- Physiological phimosis (at birth, adhesions between the foreskin and the glans)
• - Pathological phimosis (secondary to a scarring
process)
Phimosis
Conservative treatment + Intermediate variant
Conservative treatment (local hygiene, ointments with corticosteroids) or surgical (Duhamel dorsal debridement)
• Intermediate variant: balano-preputial adhesions; to
be unfolded gently, without surgery
Circumcision
- Circular sectioning of the foreskin ring, optionally sectioning of the brake
- For therapeutic or ritual or cosmetic purposes (on request)
- Circular of separate wires or various devices
Labial fusion
• The labia minora become fused together
• Partial / total attachment through a very thin bridge
(mucosa)
• Typically presents in girls at least 3 months old
• Could be associated with urinary tract infections
• Treatment: rigorous local hygiene, ointments with
estrogen or corticosteroids, deck sectioning
Imperforate hymen
• Congenital vaginal obstructions
• Hymen without an opening obstructs the vagina
• Types hydrocolpos (distension of the vagina),
hydrometrocolpos (distension of the vagina and uterus)
• Transverse vaginal septum
Vaginal obstruction can lead to vaginal and / or
uterine distension by the accumulation of
secretions:
- Hydro (metro) colpos - clear liquid
- Mucus (metro) colpos - mucus
- Pio (metro) colpos - pus
- Hemato (metro) colpos - blood
Imperforate hymen
Prenatal diagnosis
ultrasonography
Imperforate hymen
Postnatal diagnosis
tumor in the lower abdomen (newborn), amenorrhea (puberty), cyclic abdominal pain
Imperforate hymen
Treatment
Incision of the imperforate hymen / septum
