Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Genital / Urinary Tumors
Genital/urinary tumors include carcinoma of the bladder, non-germ cell testicular cancer, non-germ cell ovarian cancer, and carcinoma of the cervix and vagina. The prognosis, diagnosis, classification, and treatment of these genital/urinary tumors are discussed below. It must be emphasized that these tumors are seen very infrequently in patients younger than 15 years, and most of the evidence is derived from case series.
Standard Treatment Options for Extramedullary Plasmacytoma
Standard treatment options for extramedullary plasmacytoma include the following:
Radiation therapy to the isolated lesion with fields that cover the regional lymph nodes, if possible.[1,2]
In some cases, surgical resection may be considered, but it is usually followed by radiation therapy.
If the monoclonal (or myeloma) protein (M protein) persists or reappears, the patient may need further radiation therapy. In...
Incidence, risk factors, and clinical presentation
Carcinoma of the bladder is extremely rare in children. The most common carcinoma to involve the bladder is papillary urothelial neoplasm of low malignant potential, which generally presents with hematuria.[1,2]
Bladder cancer in adolescents may develop as a consequence of alkylating-agent chemotherapy given for other childhood tumors or leukemia.[3,4] The association between cyclophosphamide and bladder cancer is the only established relationship between a specific anticancer drug and a solid tumor.
Prognosis and treatment
In contrast to adults, most pediatric bladder carcinomas are low grade, superficial, and have a good prognosis following transurethral resection.[2,5,6,7,8] Squamous cell carcinoma and more aggressive carcinomas, however, have been reported and may require a more aggressive surgical approach.[9,10]
(Refer to the PDQ summary on adult Bladder Cancer Treatment for more information.)
Testicular Cancer (Non-Germ Cell)
Testicular tumors are very rare in young boys and account for an incidence of 1% to 2% of all childhood tumors.[11,12] The most common testicular tumors are benign teratomas followed by malignant nonseminomatous germ cell tumors. (Refer to the PDQ summary on Childhood Extracranial Germ Cell Tumors for more information.) Non–germ cell tumors such as sex cord–stromal tumors are exceedingly rare in prepubertal boys. In a small series, gonadal stromal tumors accounted for 8% to 13% of pediatric testicular tumors.[13,14] In newborns and infants, juvenile granulosa cell tumors are the most common stromal cell tumor. In older males, Leydig cell tumors are more common. Stromal cell tumors have been described as benign in young boys.[16,17,18]
There are conflicting data about malignant potential in older males. Most case reports suggest that in the pediatric patients, these tumors can be treated with surgery alone.[Level of evidence: 3iii]; [Level of evidence: 3iiiA]; [Level of evidence: 3iiiDii] However, given the rarity of this tumor, the surgical approach in pediatrics has not been well studied.
Ovarian Cancer (Non–Germ Cell)
The majority of ovarian masses in children are not malignant.
The most common neoplasms are germ cell tumors, followed by epithelial tumors, stromal tumors, and then miscellaneous tumors such as Burkitt lymphoma.[20,21,22,23] The majority of malignant ovarian tumors occur in girls aged 15 to 19 years.