The possibility of a congenital malignant brain tumor was considered. Although surgery was advised, the parents opted for conservative medical management. The infant was under treatment for seizures with antiepileptic drugs. At 7-month follow-up, there was a noticeable increase in the seizure activity with signs of developmental delay and increased head circumference. Repeat CT and MR imaging showed features of hemimegalencephaly with a calcified mass involving the left hemisphere. The patient was diagnosed to have tuberous sclerosis. This case report illustrates how an enhancing mass (possibly a tuber) over a period of time diminishes in size and becomes calcified in a patient with tuberous sclerosis and associated hemimegalencephaly.
Pediatric testicular germ cell tumors are rare. Fifteen children, all less than 5 years of age, were evaluated and treated during February 1987 to July 1996. The median age was 18 months (range, 4-60 months). All were staged according to the Pediatric Oncology Group/Children's Cancer Study Group staging system. Seven patients had stage III disease. Histologically, 9 patients had pure endodermal sinus tumor, 1 had endodermal sinus tumor with embryonal carcinoma, 1 had embryonal carcinoma alone, 2 had immature teratoma, and 2 had mature teratoma. Six children were kept on surveillance. All others received chemotherapy with cisplatin, bleomycin, and vinblastine. The 10-year actuarial overall survival rate was 86.7%.
The use of intensive chemotherapy and incorporation of prophylactic treatment of the central nervous system have dramatically improved the outcome of children with non-Hodgkin's lymphoma (NHL). The authors analyzed retrospectively the disease characteristics and survival data of 34 children with NHL during a 7-year period. There were 26 boys and 8 girls with a median age of 8 years. The primary sites were the abdomen (41%) and peripheral node (41%). Histopathologically lymphoblastic and undifferentiated lymphoma (small nonclaved cell lymphoma) were equally distributed (41%). Thirteen patients had localized disease (stage I and II) and 21 patients had advanced disease (stage III and IV). Surgical removal of the primary tumor was done in 6 patients with localized gastrointestinal lesions. All 34 patients received chemotherapy, either cyclophosphamide, vincristine, methotrexate, and prednisolone (COMP) or adriamycin, cyclophosphamide, vincristine, and prednisolone (ACOP). Thirty patients achieved complete remission (88.2%). The 5-year event-free survival rate was 64%. The results indicate that most children with localized disease can be cured by COMP chemotherapy, but more aggressive chemotherapy is necessary to improve survival in advanced-stage disease.
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