Biomaterial Database

Multiple smooth muscle tumours in neurofibromatosis presenting with chronic gastrointestinal bleeding. 1988

J G Cox, and C M Royston, and D R Sutton

Department of Neurology, Hull Royal Infirmary, UK.

Gastrointestinal involvement in neurofibromatosis is well recognised. We present an unusual manifestation of gastro-intestinal neurofibromatosis--chronic gastrointestinal bleeding from extensive smooth muscle tumours.

UI	MeSH Term	Description	Entries
D007579	Jejunal Diseases	Pathological development in the JEJUNUM region of the SMALL INTESTINE.	Disease, Jejunal,Diseases, Jejunal,Jejunal Disease
D007580	Jejunal Neoplasms	Tumors or cancer in the JEJUNUM region of the small intestine (INTESTINE, SMALL).	Cancer of Jejunum,Cancer of the Jejunum,Cancer, Jejunal,Jejunal Cancer,Cancers, Jejunal,Jejunal Cancers,Jejunal Neoplasm,Jejunum Cancer,Jejunum Cancers,Neoplasm, Jejunal,Neoplasms, Jejunal
D007583	Jejunum	The middle portion of the SMALL INTESTINE, between DUODENUM and ILEUM. It represents about 2/5 of the remaining portion of the small intestine below duodenum.	Jejunums
D007889	Leiomyoma	A benign tumor derived from smooth muscle tissue, also known as a fibroid tumor. They rarely occur outside of the UTERUS and the GASTROINTESTINAL TRACT but can occur in the SKIN and SUBCUTANEOUS TISSUE, probably arising from the smooth muscle of small blood vessels in these tissues.	Fibroid,Fibroid Tumor,Fibroid Uterus,Fibroids, Uterine,Fibroma, Uterine,Fibromyoma,Leiomyoma, Uterine,Fibroid Tumors,Fibroid, Uterine,Fibroids,Fibromas, Uterine,Fibromyomas,Leiomyomas,Tumor, Fibroid,Tumors, Fibroid,Uterine Fibroid,Uterine Fibroids,Uterine Fibroma,Uterine Fibromas,Uterus, Fibroid
D008297	Male		Males
D009456	Neurofibromatosis 1	An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).	Peripheral Neurofibromatosis,Recklinghausen Disease of Nerve,von Recklinghausen Disease,Cafe-au-Lait Spots with Pulmonic Stenosis,Molluscum Fibrosum,NF1 (Neurofibromatosis 1),Neurofibromatosis I,Neurofibromatosis Type 1,Neurofibromatosis Type I,Neurofibromatosis, Peripheral Type,Neurofibromatosis, Peripheral, NF 1,Neurofibromatosis, Peripheral, NF1,Neurofibromatosis, Type 1,Neurofibromatosis, Type I,Pulmonic Stenosis with Cafe-au-Lait Spots,Recklinghausen Disease, Nerve,Recklinghausen's Disease of Nerve,Recklinghausens Disease of Nerve,Watson Syndrome,von Recklinghausen's Disease,Cafe au Lait Spots with Pulmonic Stenosis,Neurofibromatoses, Peripheral,Neurofibromatoses, Type I,Neurofibromatosis, Peripheral,Peripheral Neurofibromatoses,Pulmonic Stenosis with Cafe au Lait Spots,Syndrome, Watson,Type 1 Neurofibromatosis,Type 1, Neurofibromatosis,Type I Neurofibromatoses,Type I, Neurofibromatosis,von Recklinghausens Disease
D006471	Gastrointestinal Hemorrhage	Bleeding in any segment of the GASTROINTESTINAL TRACT from ESOPHAGUS to RECTUM.	Hematochezia,Hemorrhage, Gastrointestinal,Gastrointestinal Hemorrhages,Hematochezias
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults