Malignant peripheral nerve sheath tumor of breast in patient without Von Recklinghausen's neurofibromatosis: a case report.

Malignant peripheral nerve sheath tumor (MPNST) of the breast without Von Recklinghausen's neurofibromatosis (VRN) is extremely rare. The authors report a 19 year-old woman who presented with a 12 month history of a painless mass of the left breast. Tissue biopsy was performed. The histologic diagnosis was made with immunohistochemical study in which the tumor showed positivity of vimentin, S-100 protein, neuron-specific protein(NSE), neurofilament protein(NF) and glial fibrillary acidic protein(GFAP). The patient was referred for radiation therapy after simple mastectomy.

Adrenocortical tumors in children.

Adrenocortical tumors are very rare in children. The records of seven patients (four boys and three girls) who attended Srinagarind Hospital between January 1986 and September 2000 were retrospectively reviewed. Virilization and hypertension were found in four patients, two of whom had untreated congenital adrenal hyperplasia. Hypertension or virilization was the single manifestation found in two other patients. Only one patient showed clinical symptoms of Cushing's syndrome and another nonfunctioning tumor in Down's syndrome. Abdominal ultrasonography was helpful in locating the tumors. Unilateral tumors were found in all of the patients and surgical exploration was done in six of them. Pathological examination revealed four adrenocortical carcinomas, one adrenal gland hyperplasia and one lipoma. The two patients in which the cancer metastasized to the liver and lungs died 1 and 1 1/2 months after diagnosis, respectively. The authors concluded that virilization and hypertension remain the primary diagnostic symptoms of adrenocortical tumors in children. Early detection and adrenalectomies prolonged the survival time in these patients.