Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Tumors are rare, but well-documented causes of precocious puberty in both sexes. The therapeutic and prognostic implications of a diagnosis of cancer require that the presence of a neoplastic process be ruled out in any case of precocious puberty. Granulosa-cell tumor of the ovary and Leydig-cell tumor of the testis are the most frequent gonadal tumors inducing precocious pseudopuberty in the two sexes. Adrenal tumors sustain a variety of endocrine syndromes, the most frequent one being virilization with or without hypercortisolism. Pure feminizing adrenal neoplasms have been described. For reasons not yet well understood, hypothalamochiasmatic glioma (beta-HCG) secreting tumors have almost never been described in association with female precocious puberty. Among these neoplasia, pineal germ-cell tumor inducing sexual maturation must be included. Hypothalamochiasmatic glioma and craniopharyngioma are the two cerebral tumors capable of inducing true precocious puberty. Even if equally distributed between both sexes, these tumors interfere with sexual maturation less frequently in girls than in boys. Hypothalamic hamartoma is considered a benign tumor, since it does represent a space-occupying mass. It more correctly could be called a malformation if its histologic characteristics are recalled. This cerebral lesion is now frequently described in children with true precocious puberty, probably because of improved diagnostic imaging methods.
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PMID:Oncologic causes of precocious puberty. 270 2

Pituicytoma is an exceptionally rare low-grade glioma (WHO grade I) of the neurohypophysis and infundibulum. We are reporting the case of a 48-year-old man who presented with severe Cushing's syndrome. Endocrinological evaluation unequivocally confirmed pituitary-dependent Cushing's syndrome (=Cushing's disease). Cranial MR-imaging displayed a conspicuous area in the dorsal and basal pituitary gland and a minimal bulging of the pituitary gland paramedian of the pituitary stalk on the right side. Transsphenoidal inspection revealed a small tumor in the basal and dorsal pituitary gland. Surprisingly, the definite postoperative histopathological diagnosis of the removed tumor was pituicytoma and not pituitary adenoma. Hence, the microadenoma responsible for Cushing's disease was not yet removed and persistent hypercortisolism necessitated transsphenoidal re-operation. During re-operation, hemihypophysectomy was performed on the right side. The non-tumorous specimen of the adeno-hypophysis showed signs of Crooke's hyalinization consistent with Cushing's disease. Undetectable postoperative ACTH- and cortisol levels provided clear evidence that the underlying ACTH-source was successfully removed during re-operation. Coincidence of pituicytoma and pituitary-dependent Cushing's disease has not previously been reported.
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PMID:Pituicytoma in a patient with Cushing's disease: case report and review of the literature. 2094 2

Pituicytoma is a rare low-grade tumor (WHO Grade I) of pituicytes involving the sellar-suprasellar region, and originating from the specialized glial cells of the neurohypophysis. Clinically and radiologically, they closely mimic pituitary adenoma or meningioma. Diagnosis requires histopathological examination of the resected tissue. This uncommon glial neoplasm is a rarity, with only 57 cases reported in the literature so far. We report three cases of pituicytoma (aged between 7 and 24 years) presenting with reduced vision, headache and features of hypercortisolism in one case. Radiologically, these lesions mimicked meningioma, hypothalamic chiasmatic glioma and pituitary microadenoma, respectively. The second case is a 7-year-old girl, the youngest case on record. Since this tumor is uncommon, it is frequently misdiagnosed. Awareness of this entity is essential for planning management and treatment. We present a brief review of all cases reported in the medical literature, and describe the clinical symptomatology, associated endocrinological abnormalities, imaging characteristics, behavior and outcome.
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PMID:Pituicytoma: report of three cases with review of literature. 2323 62