RESUMO
@#The mother of a 9-month-old female infant complained that her child was unable to pass urine at the same time noticing a mass protruding from the vaginal orifice.The infant had a single episode of vaginal bleeding.The primary concern of the mother was the inability of the daughter to micturate. Malignant germ cell tumour arising from an infant vagina is rare and accounts for about 3% of all paedriatic malignancies. These are also referred to as endodermal sinus tumours or yolk sac tumours, and are mostly the commonest form of infant vaginal malignancies encountered. A diagnosis of endodermal sinus tumour was established based on the histology and raised α-fetoprotein levels.These tumours had Schiller-Duval bodies which are primarily blood vessels surrounded by primordial germ cells and were periodic acid shift (PAS) positive diastase resistant hyaline globules which also stain positive with αfetoprotein which is an important diagnostic feature. Tumours with high α-fetoprotein levels have a poorer prognosis. However, they respond satisfactorily to chemotherapy
RESUMO
Erdheim-Chester disease (ECD) is a rare multiple system histiocytosis that is characterized pathologically by xanthogranulomatous infiltrates and radiologically by symmetrical sclerosis of long bones. The diagnosis is often confirmed by biopsy of bone or of orbital or retroperitoneal soft tissue. Intracranial involvement is rare. The authors report a case of ECD in which the diagnosis was made after biopsy of a hypothalamic mass. The mass had been discovered during a workup for panhypopituitarism in a 55-year-old man with urological and bone disease. Aside from diabetes insipidus, other features of pituitary insufficiency have seldom been reported and no patients have presented with a hypothalamic tumor. The endocrinological and neurological aspects of ECD are discussed, as is its differential diagnosis. Reported cases of the disorder associated with hypopituitarism or found during biopsy of central nervous system structures are also reviewed.
