PubMed2000
PURPOSE: To describe neoplasms diagnosed in children </= 28 days of age along with their treatment, associated congenital anomalies, and the long-term consequences of the diagnoses and treatments. METHODS AND MATERIALS: Utilizing autopsy records, a computerized tumor registry, and medical records, we identified patients and stillborns at Duke University Medical Center (DUMC) diagnosed with neoplasms at </= 28 days of age between 1930 and 1998. RESULTS: Twenty-three neonates with neoplasms were identified. There were 7 males (30%) and 16 females (70%). Follow-up of survivors ranged from 4 months to 27 years (mean 9 years). The 20 patients identified via the computerized registry system for 1980-1998 constitute 2% (20/925) of all neoplasms seen in patients </= 16 years of age over this same time period at DUMC. The histologic diagnoses were teratoma/germ cell tumor (n = 8, 35%), neuroblastoma (n = 5, 22%), retinoblastoma (n = 4, 17%), primary central nervous system (CNS) tumor (n = 3, 13%), and one case each of rhabdomyosarcoma, glossal glial choristoma, and hemangioma in the setting of Kasabach-Merritt Syndrome. Of the eight teratoma/germ cell tumor patients, 6 were female (75%) and 2 male (25%). There was one malignant germ cell tumor, 2 immature teratomas, and 5 teratomas. Two of the seven patients with immature teratomas or teratoma were long-term survivors following surgery. The one patient with malignant germ cell tumor, treated with surgery and chemotherapy, died. Associated anomalies were imperforate anus, congenital absence of a limb, left ventricular hypertrophy, fusion or absence of toes, coarctation of the aorta, and pulmonary valve dysplasia. Of the five children with neuroblastoma, 4 were female. INSS Stages were 1 (n = 1), 2A (n = 1), 3 (n = 1), and 4S (n = 2). Two were treated with surgery + chemotherapy + radiotherapy; two with surgery + chemotherapy; and one with surgery alone. Four children are long-term survivors. Associated congenital anomalies and medical problems were ventricular septal defect, seizure disorder, and Fanconi's anemia. A child with a dumbbell neuroblastoma, treated with surgery and chemotherapy, is paraplegic. Of the four children with retinoblastoma, two were female. Two had trilateral disease and two bilateral. Three of the four had a family history of retinoblastoma. The two children with trilateral retinoblastoma died after therapy with surgery, craniospinal and orbital irradiation, and chemotherapy. Two children with bilateral disease are long-term survivors: one treated with radiotherapy + chemotherapy and one with radiotherapy alone. They have marked orbital bone growth abnormalities. The three patients with CNS tumors were female. The histologies were glioblastoma multiforme, anaplastic astrocytoma, and malignant mixed oligodendroglioma. Two of the patients are long-term survivors after surgery + chemotherapy. Six children received eight courses of radiation therapy: 2 for Stage 4S neuroblastoma with respiratory compromise from an enlarging liver and 4 for retinoblastoma. The two infants with trilateral retinoblastoma received two courses of irradiation each: one of the treatment of intraocular tumor and a second, at an older age, for the pineal tumor. The most serious complication of anesthesia was a case of enterobacter cloacae sepsis in the central venous access line used for repetitively administering the anesthetic. CONCLUSION: The most common neonatal neoplasm histologic diagnoses are teratoma/germ cell tumor, neuroblastoma, and retinoblastoma. Neonatal neoplasms may be associated with congenital anomalies. Radiation therapy is administered infrequently in a population highly susceptible to late ill effects. When radiotherapy is required, anesthesia may be repetitively administered to aid in reproducible treatment.