Biomaterial Database

Solitary plexiform neurofibroma. 1989

F G Aloi, and R Massobrio

Institute of Dermatology, University of Turin, Italy.

A 35-year-old man with a solitary subcutaneous cord running on the abdomen wall is reported. Histologic examination of the lesion permitted a diagnosis of plexiform neurofibroma. In this patient and in his family no other signs of von Recklinghausen's disease were found. The plexiform neurofibroma considered as pathognomonic of neurofibromatosis may occur in a solitary form.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009455	Neurofibroma	A moderately firm, benign, encapsulated tumor resulting from proliferation of SCHWANN CELLS and FIBROBLASTS that includes portions of nerve fibers. The tumors usually develop along peripheral or cranial nerves and are a central feature of NEUROFIBROMATOSIS 1, where they may occur intracranially or involve spinal roots. Pathologic features include fusiform enlargement of the involved nerve. Microscopic examination reveals a disorganized and loose cellular pattern with elongated nuclei intermixed with fibrous strands. (From Adams et al., Principles of Neurology, 6th ed, p1016)	Neurofibromas
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
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000009	Abdominal Muscles	Muscles forming the ABDOMINAL WALL including RECTUS ABDOMINIS; ABDOMINAL OBLIQUE MUSCLES, transversus abdominis, pyramidalis muscles and quadratus abdominis.	Cremaster Muscle,Pyramidalis Muscle,Quadratus Abdominis,Transverse Abdominal,Transversus Abdominis,Abdominal Muscle,Abdominal, Transverse,Abdominals, Transverse,Abdomini, Quadratus,Abdominis, Quadratus,Cremaster Muscles,Muscle, Abdominal,Muscle, Cremaster,Muscle, Pyramidalis,Muscles, Abdominal,Muscles, Cremaster,Muscles, Pyramidalis,Pyramidalis Muscles,Quadratus Abdomini,Transverse Abdominals
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
D012983	Soft Tissue Neoplasms	Neoplasms of whatever cell type or origin, occurring in the extraskeletal connective tissue framework of the body including the organs of locomotion and their various component structures, such as nerves, blood vessels, lymphatics, etc.	Neoplasm, Soft Tissue,Neoplasms, Soft Tissue,Soft Tissue Neoplasm