1/2. Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity.We describe a family in which lentigines were present in the index patient, in three of her seven siblings, in their mother, and in a niece (the daughter of an affected sister). Cutaneous myxomas were present in the index patient, in two of her brothers, and probably in their mother. In addition, the index patient had two cardiac myxomas. multiple myxoid mammary fibroadenomas, and the cushing syndrome, and an affected brother had acromegaly caused by a growth hormone-secreting tumor of the pituitary gland. Thus, at least one manifestation of the complex of myxomas, spotty pigmentation, and endocrine overactivity has occurred in three successive generations of this family. Both male and female family members were affected, and 5 of the 11 children of affected persons had the disorder. The karyotypes of two affected persons were normal. These observations are consistent with mendelian dominant inheritance of the syndrome.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
2/2. Intrasellar gangliocytoma and syndromes of pituitary hypersecretion. Case report.Syndromes of hypersecretion of pituitary hormone and sellar enlargement may on occasion be caused by a gangliocytoma instead of a pituitary adenoma. At least some of these rare tumors are apparently independent of and separable from the pituitary gland, its stalk, and the hypothalamus, and are therefore surgically removable without incurring further endocrine deficit. The authors report such a case, with successful removal of the tumor via a frontal craniotomy. The associated hypersecretion of pituitary hormone was corrected without disturbing normal pituitary function.- - - - - - - - - - ranking = 9keywords = pituitary (Clic here for more details about this article) |