should be queried as to:
FIGURE 156-1 From Bostwick DG, Cheng L. Urologic surgical pathology. 3rd ed.
Edinburgh: Mosby; 2014.
a.the depth of invasion.
b.the histologic type.
c.the degree of differentiation.
d.the dominant pattern.
e.the percent of rhabdomyoblasts in the tumor.
Answers
1.b. Li-Fraumeni syndrome. Subgroups of children with a genetic predisposition to the development of rhabdomyosarcoma have been identified. The Li-Fraumeni syndrome associates childhood sarcomas with mothers who have an excess of premenopausal breast cancer and with siblings who have an increased risk of cancer. A mutation of the TP53 tumor suppressor gene was found in the tumors in all patients with this syndrome.
2.a. Alveolar histologic type. The second most common form is alveolar,
which occurs more commonly in the trunk and extremities than in genitourinary sites and has a worse prognosis. Alveolar rhabdomyosarcoma also has a higher rate of local recurrence and spread to regional lymph nodes, bone marrow, and distant sites.
3.d. Observation. If tumor is shrinking during chemotherapy, and another biopsy after completing radiotherapy shows maturing rhabdomyoblasts without frank tumor cells, total cystectomy can be postponed or avoided altogether.
4.a. Vincristine, dactinomycin, and cyclophosphamide. Before effective chemotherapy, surgery alone produced a 50% 2-year relapse-free survival rate. With current multimodal treatment, survival rates of 90% are expected. Currently, the Intergroup Rhabdomyosarcoma Study Group recommends that children 10 years and older undergo ipsilateral retroperitoneal lymph node dissection before chemotherapy.
5.b. Observation. It is important to note that an elevated α-fetoprotein level after orchiectomy for yolk sac tumor in an infant does not always represent persistent disease. Normal adult reference laboratory values for α- fetoprotein cannot be used in young children, because α-fetoprotein synthesis continues after birth. Normal adult levels (less than 10 mg/mL) are not reached until 8 months of age.
6.c. Left orchiectomy. Early gonadectomy is advocated, because tumors have been reported in children younger than 5 years. In patients with mixed gonadal dysgenesis who are reared as males, all streak gonads and undescended testes should be removed. Scrotal testes can be preserved, because they are less prone to tumor development.
7.b. Observation. Prepubertal mature teratomas have a benign clinical course, which contrasts with the clinical behavior of teratomas in adults, which have the propensity to metastasize. This benign behavior has led to the consideration of testicular-sparing procedures rather than radical orchiectomy.
8.c. Observation. The initial treatment for yolk sac tumor is radical inguinal orchiectomy. This treatment is curative in most children. Routine retroperitoneal lymph node dissection and adjuvant chemotherapy are not indicated.
9.c. No further treatment and surveillance consisting of bladder sonography. TCC in children is uncommon and the lesions are unifocal, typically low grade and not prone to recurrence; therefore treatment
1.Of rhabdomyosarcomas, 15% to 20% occur in the genitourinary system. RMS is the most common soft tissue sarcoma in children.
2.Rhabdomyosarcoma has three histologic types: embryonal, alveolar, and undifferentiated.
3.In the genitourinary tract, embryonal histology is by far the most common.
4.Alveolar rhabdomyosarcoma has a higher rate of local recurrence and spread to regional lymph nodes, bone marrow, and distant sites when compared to embryonal.
5.There are two variants of embryonal histology: botryoid (bunch of grapes), which occur in the bladder, and spindle cell, which are common in the paratesticular region.
6.For patients with rhabdomyosarcoma, generally, chemotherapy and radiation precede surgical resection unless the tumor is amenable to a partial cystectomy. Organ preservation is the goal of treatment.
7.Mature rhabdomyoblasts found after treatment do not require further treatment.
8.Radiation therapy for patients with bladder or prostate rhabdomyosarcoma results in a markedly reduced functional bladder capacity and abnormal voiding patterns. Only 40% of patients treated will have normal bladder function—usually these are patients who have not received radiation therapy.
9.Patients with rhabdomyosarcoma of the bladder or prostate typically present with symptoms of outlet obstruction and hematuria. A urethral catheter for the lower tract and an internal stent for the upper tracts are the preferred methods of decompression.
10.In paratesticular rhabdomyosarcoma, children who are 10 years and older should have an ipsilateral retroperitoneal lymph node dissection. Those less than 10 years of age do not require an RPLND.
11.Favorable sites of origin for rhabdomyosarcoma include paratesticular, vulvar-vaginal, and uterine. In the female, the vagina is the most common site. Bladder and prostate are unfavorable sites.
12.Only one third of patients are fertile following treatment for rhabdomyosarcoma.
13.Treated patients are at risk for secondary malignancies, which include osteosarcoma, Ewing sarcoma, lymphoma, cancer of the colon, and cervical cancer.
14.The Li-Fraumeni syndrome associates childhood sarcomas with mothers who have an excess of premenopausal breast cancer and with siblings who have an increased risk of cancer. A mutation of the TP53 tumor suppressor gene is found in the tumors of these patients.
15.TCC of the bladder in children is uncommon and the lesions are unifocal, often located on the trigone, typically low grade, and not prone to recurrence; therefore, treatment consisting of resection only is adequate. Ultrasound is remarkably accurate and is an adequate surveillance strategy. Cyclophosphamide and dantrolene exposure in selected cases may be etiologic.
16.Patients with a history of smoking, those with end-stage renal disease, and those with transplants who are immunosuppressed and have bladder intestinal augments are at greatest risk for cancer in the augment.
17.Patients with bladder exstrophy are at increased risk for adenocarcinoma of the bladder. Squamous cell carcinoma has also been reported in these patients.
18.Nephrogenic adenoma is a benign lesion usually associated with bladder injury.
19.Ovarian germ cell tumors in children include teratoma, gonadoblastoma, and yolk sac tumor. Sex cord tumors include thecofibroma, Sertoli cell tumors, Leydig cell tumors, and granulosa cell tumors.
20.Teratoma and yolk sac tumors are the most common childhood testicular tumors.
21.Schiller-Duval bodies are a characteristic finding in yolk sac tumors of the testis.
22.Teratomas are classified as mature, immature, and malignant.
23.Mature teratomas have a benign course in childhood and are successfully treated with radical orchiectomy alone. This is not true in the adult.
24.Immature teratomas generally behave in a benign fashion in childhood unless they have foci of yolk sac tumor. If the latter is the case, they should be treated as one would treat a yolk sac tumor.
25.All streak gonads in patients with gonadal dysgenesis should be removed. In patients with mixed gonadal dysgenesis who are reared as males, all streak gonads and undescended testes should be removed. Scrotal testes can be preserved, because they are less prone to tumor development.
26.α-Fetoprotein is secreted by yolk sac tumors.
27.Normal adult reference laboratory values for α-fetoprotein cannot be used in young children, because α-fetoprotein synthesis continues after birth. Normal adult levels (less than 10 mg/mL) are not reached until 8 months.
28.Reinke crystals are diagnostic of Leydig cell tumors.
29.The most common metastatic tumors to the testis in children are leukemia and lymphoma.
30.Testicular microlithiasis in children does not require periodic imaging follow-up unless it is associated with a risk factor such as a previous testicular tumor.
31.Testis organ-sparing surgery is preferred for most testicular tumors in children as they are often benign. Yolk sac tumor is not benign, and testis-sparing surgery is not advised.