Melanotic neuroectodermal tumor of infancy and fetal hydantoin syndrome.

Fetal hydantoin syndrome (FHS), a characteristic pattern of altered growth and development, has been well described in recent years in offsprings of epileptic mothers taking phenytoin or other hydantoin anticonvulsants during the gestational period. Recent reports of neuroblastoma in three patients with the FHS further raise the questions of the "oncogenic effect " of hydantoin compounds. A case of melanotic neuroectodermal tumor of infancy (MNTI) has been studied clinically and pathologically including light microscopy, histochemistry, and electron microscopy. This case strengthens the evidence for the teratogenic and oncogenic effects of hydantoin compounds and we believe that it represents the first reported case of FHS associated with MNTI. It would be most important from a clinical standpoint to carefully scrutinize individuals with the FHS for neoplasias. Furthermore, detailed gestational drug history in children with neuroblastoma and other neoplasias should be carefully searched for, with the hope of clarifying the definitive oncogenic effect of hydantoin compounds.

[Coincidence of a thalidomide-induced malformation and a lymphoma of high malignancy (author's transl)]. / Zusammentreffen einer Thalidomid-induzierten Fehlbildung mit einem malignen Lymphom hohen Malignitätsgrades.

The case history of a fifteen year old patient with Thalidomide induced malformation who developed a lymphoma of high malignancy is presented.

[On pathology of the diffuse pachygyria (author's transl)]. / Zur Pathologie der diffusen Pachygyrie

A case report on a 4 year old boy with physical and mental retardation noted since the age of 7 months is presented. In the 10th month a hydrocephalus internus was diagnosed. The autopsy showed a cerebral dystrophy with diffuse pachygyria presumably caused by a disturbance in the neuroblast migration during the 2nd and the 5th month of gravidity. The 33 cases of diffuse pachygyria hitherto described in the literature are compared with our own one. In more than 80% of the cases pachygyria is associated with oligophrenia or idiotism, resp., and 75% with epilepsy. Paralysis is rarely observed. The average life expectancy is about 5 years. Etiologic factors are intrauterine hypoxemia and infectious diseases during the early pregnancy period as well as hereditary afflictions. In the case described here the teratogenic action of a drug probably was the etiologic factor.