A case of ganglioneuroblastoma associated with malignant mesenchymoma is reported. The tumor originated from the retroperitoneum and was comprised not only of neuroblastoma and ganglioneuroma, but also of typical rhabdomyosarcoma, liposarcoma, undifferentiated mesenchymoma, as well as cartilaginous tissue. No distinct border existed between thses different components. The histogenesis of the tumor is discussed. We suggest that the tumor derived from a remnant of neural crest (ectomesenchyme).

A case of fatal familial intrahepatic cholestasis (Byler disease) developed a neuromuscular syndrome similar to that in experimental vitamin E deficiency and abetalipoproteinemia, and died of hepatic and cardiac failure. Serum vitamin E level was extremely low. Autopsy revealed intrahepatic cholestatic cirrhosis without obliterative lesions in the bile duct system and marked splenomegaly with splenoma-like nodules. The other pathological lesions were considered to be due to chronic vitamin E deficiency as follows:1. Mitochondrial changes especially of the hepatocyte and cardiac muscle. 2. Cardiomyopathy. 3. Myopathy. 4. Vasculopathy. 5. Systemic lipofuscinosis. 6. Lesions of the reproductive and endocrine organs. 7. Kyphoscoliosis and pes cavus. 8. Systemic neuroaxonal dystrophy with peripheral neuropathy.

A 34-year-old woman had episodes of hypoglycemic attack 8 years after a surgical resection of a retroperitoneal hemangiopericytoma. In spite of normal levels of serum IRI, insulin radioreceptor assay demonstrated high level of plasma ILA (insulin like activity). The patient underwent resection of a recurrent retroperitoneal tumor with metastatic lesions of the liver. Postoperatively, ILA level in plasma by insulin radioreceptor assay decreased, and hypoglycemic attacks disappeared. Therefore, this associated hypoglycemia was presumed to be not caused by excess glucose consumption by the tumor, not by excess secretion of IRI by the tumor, but caused by the presence of high level of ILA related to the tumor.

No abstract