Editing Bladder and Testis Oncology

== Rhabdomyosarcoma (RMS) ==

* '''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):'''
**# '''Embryonal'''
**#* '''Most common form of bladder or prostate RMS.'''
**# '''Alveolar'''
**#* Uncommon in the bladder or prostate
**#* Two distinct types, PAX-FOXO1 fusion positive and negative, and fusion-positive tumors carry a worse prognosis
**# '''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 ==

* 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 ==

* '''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 ==

* '''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 ==

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