Biomaterial Database

Suprasellar tumors of maldevelopmental origin in Klinefelter's syndrome. A report of two cases. 1992

L M Hamed, and B L Maria, and R Quisling, and M M Fanous, and P Mickle

Department of Ophthalmology, University of Florida College of Medicine, Gainesville 32610-0284.

Patients with Klinefelter's syndrome may have a predisposition for the development of neoplasia, particularly extragonadal germ-cell tumors, but a suprasellar location is rarely reported. The clinical and neuroradiologic features in two patients with Klinefelter's syndrome and dysmorphic suprasellar masses of maldevelopmental origin (presumably lipomas or lipodermoids) are described. One patient had bilateral optic atrophy and decreased vision. To our knowledge, only one similar case (a suprasellar hamartoma) has been previously reported in association with Klinefelter's syndrome.

UI	MeSH Term	Description	Entries
D007713	Klinefelter Syndrome	A form of male HYPOGONADISM, characterized by the presence of an extra X CHROMOSOME, small TESTES, seminiferous tubule dysgenesis, elevated levels of GONADOTROPINS, low serum TESTOSTERONE, underdeveloped secondary sex characteristics, and male infertility (INFERTILITY, MALE). Patients tend to have long legs and a slim, tall stature. GYNECOMASTIA is present in many of the patients. The classic form has the karyotype 47,XXY. Several karyotype variants include 48,XXYY; 48,XXXY; 49,XXXXY, and mosaic patterns ( 46,XY/47,XXY; 47,XXY/48,XXXY, etc.).	48,XXYY Syndrome,49,XXXXY Syndrome,Klinefelter Syndrome, Variants,Klinefelter's Syndrome,XXXY Males,XXY Syndrome,XXY Trisomy,Xxyy Syndrome,Klinefelter Syndromes,Klinefelter Syndromes, Variants,Klinefelters Syndrome,Syndrome, Klinefelter,Syndrome, Klinefelter's,Syndrome, Variants Klinefelter,Syndrome, XXY,Syndrome, Xxyy,Syndromes, Klinefelter,Syndromes, Variants Klinefelter,Syndromes, XXY,Syndromes, Xxyy,Trisomies, XXY,Trisomy, XXY,XXXY Male,XXY Syndromes,XXY Trisomies,Xxyy Syndromes
D008067	Lipoma	A benign tumor composed of fat cells (ADIPOCYTES). It can be surrounded by a thin layer of connective tissue (encapsulated), or diffuse without the capsule.	Fatty Tumor,Hibernoma,Lipoma, Pleomorphic,Atypical Lipoma,Lipomata,Lipomatosis, Multiple,Atypical Lipomas,Fatty Tumors,Hibernomas,Lipoma, Atypical,Lipomas,Lipomas, Atypical,Lipomas, Pleomorphic,Lipomatas,Lipomatoses, Multiple,Multiple Lipomatoses,Multiple Lipomatosis,Pleomorphic Lipoma,Pleomorphic Lipomas,Tumor, Fatty,Tumors, Fatty
D008297	Male		Males
D001932	Brain Neoplasms	Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.	Brain Cancer,Brain Metastases,Brain Tumors,Cancer of Brain,Malignant Primary Brain Tumors,Neoplasms, Intracranial,Benign Neoplasms, Brain,Brain Neoplasm, Primary,Brain Neoplasms, Benign,Brain Neoplasms, Malignant,Brain Neoplasms, Malignant, Primary,Brain Neoplasms, Primary Malignant,Brain Tumor, Primary,Brain Tumor, Recurrent,Cancer of the Brain,Intracranial Neoplasms,Malignant Neoplasms, Brain,Malignant Primary Brain Neoplasms,Neoplasms, Brain,Neoplasms, Brain, Benign,Neoplasms, Brain, Malignant,Neoplasms, Brain, Primary,Primary Brain Neoplasms,Primary Malignant Brain Neoplasms,Primary Malignant Brain Tumors,Benign Brain Neoplasm,Benign Brain Neoplasms,Benign Neoplasm, Brain,Brain Benign Neoplasm,Brain Benign Neoplasms,Brain Cancers,Brain Malignant Neoplasm,Brain Malignant Neoplasms,Brain Metastase,Brain Neoplasm,Brain Neoplasm, Benign,Brain Neoplasm, Malignant,Brain Neoplasms, Primary,Brain Tumor,Brain Tumors, Recurrent,Cancer, Brain,Intracranial Neoplasm,Malignant Brain Neoplasm,Malignant Brain Neoplasms,Malignant Neoplasm, Brain,Neoplasm, Brain,Neoplasm, Intracranial,Primary Brain Neoplasm,Primary Brain Tumor,Primary Brain Tumors,Recurrent Brain Tumor,Recurrent Brain Tumors,Tumor, Brain
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003397	Craniopharyngioma	A benign pituitary-region neoplasm that originates from Rathke's pouch. The two major histologic and clinical subtypes are adamantinous (or classical) craniopharyngioma and papillary craniopharyngioma. The adamantinous form presents in children and adolescents as an expanding cystic lesion in the pituitary region. The cystic cavity is filled with a black viscous substance and histologically the tumor is composed of adamantinomatous epithelium and areas of calcification and necrosis. Papillary craniopharyngiomas occur in adults, and histologically feature a squamous epithelium with papillations. (From Joynt, Clinical Neurology, 1998, Ch14, p50)	Craniopharyngioma, Adamantinous,Craniopharyngioma, Papillary,Rathke Pouch Tumor,Craniopharyngioma, Adult,Craniopharyngioma, Child,Neoplasm, Rathke Cleft,Neoplasm, Rathke's Cleft,Rathke Cleft Neoplasm,Rathke's Cleft Neoplasm,Rathke's Pouch Tumor,Adamantinous Craniopharyngioma,Adamantinous Craniopharyngiomas,Adult Craniopharyngioma,Adult Craniopharyngiomas,Child Craniopharyngioma,Child Craniopharyngiomas,Craniopharyngiomas,Craniopharyngiomas, Adamantinous,Craniopharyngiomas, Adult,Craniopharyngiomas, Child,Craniopharyngiomas, Papillary,Neoplasm, Rathkes Cleft,Papillary Craniopharyngioma,Papillary Craniopharyngiomas,Rathkes Cleft Neoplasm,Rathkes Pouch Tumor,Tumor, Rathke Pouch,Tumor, Rathke's Pouch
D004814	Epidermal Cyst	Intradermal or subcutaneous saclike structure, the wall of which is stratified epithelium containing keratohyalin granules.	Epidermoid Cyst,Pilar Cyst,Sebaceous Cyst,Cyst, Epidermal,Cyst, Epidermoid,Cyst, Pilar,Cyst, Sebaceous,Cysts, Epidermal,Cysts, Epidermoid,Cysts, Pilar,Cysts, Sebaceous,Epidermal Cysts,Epidermoid Cysts,Pilar Cysts,Sebaceous Cysts
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012658	Sella Turcica	A bony prominence situated on the upper surface of the body of the sphenoid bone. It houses the PITUITARY GLAND.	Sella Turcicas,Turcica, Sella,Turcicas, Sella