Bladder and Testis Oncology

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Rhabdomyosarcoma (RMS)[edit | edit source]

  • Epidemiology
    • Most common soft-tissue sarcoma in children
  • Genetics
    • Most cases are considered to be sporadic
    • Genetic syndromes associated with RMS:
      • Li-Fraumeni syndrome
      • Neurofibromatosis
      • Basal cell nevus syndrome
      • Costello syndrome
      • Noonan syndrome
      • Multiple endocrine neoplasia type 2A
  • Pathology and molecular biology
    • Histologic categories (3):
      1. Embryonal
        • Most common form of bladder or prostate RMS.
      2. Alveolar
        • Uncommon in the bladder or prostate
        • Two distinct types, PAX-FOXO1 fusion positive and negative, and fusion-positive tumors carry a worse prognosis
      3. Undifferentiated
  • Management
    • Radical surgery may be required if there is lack of response to non-operative treatment.
  • Prognosis
    • Prognostic factors
      • Site of origin
        • Prostate has the worst prognosis
        • Vaginal has best prognosis
      • Histology
        • Embryonal histology associated with improved prognosis compared to alveolar

Urothelial carcinoma of the Bladder[edit | edit source]

  • Uncommon
  • Lesions are unifocal, typically low grade and not prone to recurrence
  • Management
    • Treatment consisting of resection only is adequate. Adjuvant treatment is not given.
    • Surveillance cystoscopy is not needed; ultrasound is remarkably accurate and is an adequate surveillance strategy

Testicular Tumors[edit | edit source]

  • More likely benign in pre-pubertal males (in contrast to post-pubertal males where most testicular tumours are malignant)
  • Epidemiology
    • The most common pathologic diagnosis in children with a testicular mass is a YST, followed by teratoma
  • Germ Cell Tumors
    • Pathology
      • Tertatoma
        • According to some series, teratomas are the most common testicular tumor in children and consist of variable combinations of the three primitive embryologic germ cell layers [contradicts above]
        • Consist of variable combinations of the three primitive embryologic germ cell layers
        • Do not express AFP
        • In contrast to teratoms in post-pubertal males, most teratomoas in pre-pubertal male are benign, consisting of mature elements only, and do not metastasize
        • Preferred treatment for suspected teratoma is partial orchiectomy
      • Epidermoid cysts
        • Benign lesion; represent a monolayer teratoma
        • May account for >15% of prepubertal testis lesions
        • Do not express AFP
        • Preferred treatment for suspected epidermoid cysts is partial orchiectomy
        • No surveillance is required after treatment
      • Yolk-sac tumour (YST)
        • Pathology
          • Schiller-Duval bodies consist of a central blood vessel surrounded by two layers of tumor cells
            • Pathognomonic for YSTs.
        • AFP levels are elevated  
        • When metastases occur, YSTs often exhibit hematogenous spread to the lungs, without retroperitoneal disease. For these reasons retroperitoneal lymph node dissection (RPLND) plays very little role in pre-pubertal YST.
          • RPLND is used exclusively for patients with a residual retroperitoneal mass or persistently elevated AFP after chemotherapy and orchiectomy
    • Presentation, Evaluation, and Staging
      • See CW11 Figure 156-7
      • Ultrasonography
        • Teratomas
          • Heterogeneous complex lesions and may contain cystic and solid components.
          • Bony elements appear as calcifications with shadowing.
          • Adipose tissue appears as echogenic areas without shadowing.
        • Epidermoid cysts
          • Characteristic appearance described as “onion skin” comprised of concentric rings of alternating hypoechoic and hyperechoic lesions.
        • YSTs
          • Typically well circumscribed and heterogeneous in appearance.
          • May appear hypervascular and may have areas of hemorrhage and necrosis.
        • Sertoli and Leydig cell tumors
          • Similar in appearance
          • Commonly manifest as a well-circumscribed hypoechoic mass.
      • Labs
        • Tumour markers
          • Interpretation of AFP levels in infants undergoing evaluation for a testis mass must be performed with caution as a result of the physiologically persistent elevation of AFP in infants age < 1
  • Tumors Associated with Disorders of Sexual Differentiation
    • See Disorders of Sexual Differentiation Chapter Notes
    • Patients with dysgenetic testis or hypovirulization have an increased incidence of testicular tumors
  • Gonadal Stromal Tumors
    • Juvenile granulosa cell tumors
      • Most common testicular tumors affecting neonates
    • Leydig cell tumors
      • Hormonally active; associated with precocious puberty.
    • Sertoli cell
      • Usual presentation is a painless mass
      • Associated with endocrinologic and genetic syndromes such as Peutz-Jeghers and Carney syndromes
      • 10% are hormonally active
  • Leukemia and Lymphoma
    • The most common cancers with metastases to the testis are leukemia and lymphoma

Paratesticular Rhabdomyosarcoma[edit | edit source]

  • Arises from the testicular tunicae, epididymis, or spermatic cord.
  • Up to 40% of paratesticular lesions are due to RMS
  • Management
    • Multimodal involving surgery and chemotherapy with vincristine, dactinomycin, and cyclophosphamide
    • Children age > 10 should undergo a staging ipsilateral retroperitoneal lymph node dissection, and not observation, because of a high occurrence of retroperitoneal failure

References[edit | edit source]

  • Wein AJ, Kavoussi LR, Partin AW, Peters CA (eds): CAMPBELL-WALSH UROLOGY, ed 11. Philadelphia, Elsevier, 2015, vol 4, chap 156