9571/0 Soft tissue perineurioma



Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72

A rare, usually benign tumor typically manifesting in adolescence or early adulthood that may cause pogressive muscle weakness or atrophy. Perineuriomas are composed of neoplastic perineurial cells and can occur intraneurally or as soft tissue lesions 1
 
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds.)
WHO Classification of Tumours of the Central Nervous System.
4th Edition
International Agency for Research on Cancer: Lyon 2007



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Colon and rectum
ICD-O-3 topography code: C18-C20

A rare, usually benign tumor typically manifesting in adolescence or early adulthood that may cause pogressive muscle weakness or atrophy. Perineuriomas are composed of neoplastic perineurial cells and can occur intraneurally or as soft tissue lesions
2
 
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds.)
WHO Classification of Tumours of the Central Nervous System.
4th Edition
International Agency for Research on Cancer: Lyon 2007



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Perineurioma of the colon and rectum
Perineuriomas in the gastrointestinal tract usually occur in the colon and are most often identified by chance as a small sessile polyp in adults. Most measure < 0.5 cm, rarely being larger. Histologically, these are typically intramucosal lesions, which fill and expand the lamina propria, entrapping crypts. They are composed of uniform spindle cells with short tapering nuclei and indistinct eosinophilic cytoplasm, often arranged in a whorled fashion within a delicate collagenous stroma. Perineuriomas are consistently immuno - positive for EMA and less often claudinpositive. They show no evident tendency to recur
3
Click to access Pubmed
Hornick JL, Fletcher CD (2005)
Intestinal perineuriomas: clinicopathologic definition of a new anatomic subset in a series of 10 cases.
Am J Surg Pathol 29: 859-65



. Lesions formerly known as fibroblastic polyps of the colon appear to represent intramucosal perineuriomas 4
Click to access Pubmed
Groisman GM, Polak-Charcon S (2008)
Fibroblastic polyp of the colon and colonic perineurioma: 2 names for a single entity?
Am J Surg Pathol 32: 1088-94



5
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Peripheral nerves and autonomic nervous system
ICD-O-3 topography code: C47

A rare, usually benign tumor typically manifesting in adolescence or early adulthood that may cause pogressive muscle weakness or atrophy. Perineuriomas are composed of neoplastic perineurial cells and can occur intraneurally or as soft tissue lesions
6
 
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds.)
WHO Classification of Tumours of the Central Nervous System.
4th Edition
International Agency for Research on Cancer: Lyon 2007



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Depending on the site of involvement, intraneural and soft tissue perineurioma are distinguished.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49

A rare, usually benign tumor typically manifesting in adolescence or early adulthood that may cause pogressive muscle weakness or atrophy. Perineuriomas are composed of neoplastic perineurial cells and can occur intraneurally or as soft tissue lesions
7
 
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds.)
WHO Classification of Tumours of the Central Nervous System.
4th Edition
International Agency for Research on Cancer: Lyon 2007



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