Nuchal-type fibroma is a rare benign proliferation involving the dermis and subcutaneous tissues, that is a collection of dense, hypocellular bundles of collagen with entrapped adipocytes and increased numbers of small nerves. It is no longer called a nuchal fibroma, but instead a "nuchal-type fibroma" since it develops in other anatomic sites. There is no known etiology.[1][2] The World Health Organization in 2020 classified nuchal fibromas as a specific tumor form in the category of benign fibroblastic and myofibroblastic tumors.[3]
These lesions are generally asymptomatic, although patients give a long history of a solitary, superficial mass. The mass is usually in the neck (hence the name "nuchal-type"), but it can be seen in the extremities, lumbosacral area, buttocks, and face.[2][4] There is a strong association with diabetes mellitus and Gardner syndrome; in fact, it may be the initial manifestation of Gardner syndrome.[5]
Pathologyedit
A low power of a nuchal-type fibroma showing entrapped fat.
The tumors are unencapsulated and poorly circumscribed, showing a firm, white cut surface. Most tumors are about 3.5 cm, but can be up to 8 cm.[1] By microscopic examination, there are haphazardly arranged thick collagen fibers, with a low cellularity and no pleomorphism. There are usually entrapped fat cells, skeletal muscle, and peripheral nerves. The may be perineural fibrosis. The elastic fibers may be altered, which is why an elastofibroma is considered in the differential diagnosis.[1]
Nuchal fibroma
Immunohistochemistryedit
The tumor cells are strongly positive for vimentin, CD34, and sometimes with CD99. There is often (up to 2/3rds) a nuclear reaction with β-catenin.[1]
Simple excision is curative. However, in patients with Gardner syndrome, up to 45% will develop desmoid-type fibromatosis at other sites, and so this should be searched for and excluded. Patients can develop a recurrence, so follow-up is required.[2]
Epidemiologyedit
This is a rare tumor, presenting over a wide age range, but usually in the third to fifth decades of life. There is a slight male predilection, although this is not seen in syndrome-associated patients. The most common site is the posterior neck, but may also be seen in other sites (extremities, lumbosacral area, buttocks, face).[1]
^ abcdefMichal, M.; Fetsch, J. F.; Hes, O.; Miettinen, M. (1999). "Nuchal-type fibroma". Cancer. 85 (1): 156–163. doi:10.1002/(SICI)1097-0142(19990101)85:1<156::AID-CNCR22>3.0.CO;2-O. PMID 9921988.
^ abcSamadi, D. S.; McLaughlin, R. B.; Loevner, L. A.; Livolsi, V. A.; Goldberg, A. N. (2000). "Nuchal fibroma: A clinicopathological review". The Annals of Otology, Rhinology, and Laryngology. 109 (1): 52–55. doi:10.1177/000348940010900110. PMID 10651413.
^Sbaraglia M, Bellan E, Dei Tos AP (April 2021). "The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives". Pathologica. 113 (2): 70–84. doi:10.32074/1591-951X-213. PMC8167394. PMID 33179614.
^Balachandran, K.; Allen, P. W.; MacCormac, L. B. (1995). "Nuchal fibroma. A clinicopathological study of nine cases". The American Journal of Surgical Pathology. 19 (3): 313–317. doi:10.1097/00000478-199503000-00009. PMID 7872429.
^Dawes, L. C.; La Hei, E. R.; Tobias, V.; Kern, I.; Stening, W. (2000). "Nuchal fibroma should be recognized as a new extracolonic manifestation of Gardner-variant familial adenomatous polyposis". The Australian and New Zealand Journal of Surgery. 70 (11): 824–826. doi:10.1046/j.1440-1622.2000.01958.x. PMID 11147449.
Further readingedit
Lester D. R. Thompson; Bruce M. Wenig (2011). Diagnostic Pathology: Head and Neck: Published by Amirsys. Hagerstown, MD: Lippincott Williams & Wilkins. pp. 8:44–45. ISBN978-1-931884-61-7.
April 12, 2024
nuchal, fibroma, nuchal, type, fibroma, rare, benign, proliferation, involving, dermis, subcutaneous, tissues, that, collection, dense, hypocellular, bundles, collagen, with, entrapped, adipocytes, increased, numbers, small, nerves, longer, called, nuchal, fib. Nuchal type fibroma is a rare benign proliferation involving the dermis and subcutaneous tissues that is a collection of dense hypocellular bundles of collagen with entrapped adipocytes and increased numbers of small nerves It is no longer called a nuchal fibroma but instead a nuchal type fibroma since it develops in other anatomic sites There is no known etiology 1 2 The World Health Organization in 2020 classified nuchal fibromas as a specific tumor form in the category of benign fibroblastic and myofibroblastic tumors 3 Nuchal fibromaSpecialtyOncology Contents 1 Signs and symptoms 2 Pathology 2 1 Immunohistochemistry 3 Diagnosis 3 1 Differential diagnoses 4 Management 5 Epidemiology 6 See also 7 References 8 Further readingSigns and symptoms editThese lesions are generally asymptomatic although patients give a long history of a solitary superficial mass The mass is usually in the neck hence the name nuchal type but it can be seen in the extremities lumbosacral area buttocks and face 2 4 There is a strong association with diabetes mellitus and Gardner syndrome in fact it may be the initial manifestation of Gardner syndrome 5 Pathology edit nbsp A low power of a nuchal type fibroma showing entrapped fat The tumors are unencapsulated and poorly circumscribed showing a firm white cut surface Most tumors are about 3 5 cm but can be up to 8 cm 1 By microscopic examination there are haphazardly arranged thick collagen fibers with a low cellularity and no pleomorphism There are usually entrapped fat cells skeletal muscle and peripheral nerves The may be perineural fibrosis The elastic fibers may be altered which is why an elastofibroma is considered in the differential diagnosis 1 nbsp Nuchal fibromaImmunohistochemistry edit The tumor cells are strongly positive for vimentin CD34 and sometimes with CD99 There is often up to 2 3rds a nuclear reaction with b catenin 1 Diagnosis editDifferential diagnoses edit The differential diagnosis includes elastofibroma fibrolipoma desmoid type fibromatoses and nuchal fibrocartilaginous pseudotumor 1 Management editSimple excision is curative However in patients with Gardner syndrome up to 45 will develop desmoid type fibromatosis at other sites and so this should be searched for and excluded Patients can develop a recurrence so follow up is required 2 Epidemiology editThis is a rare tumor presenting over a wide age range but usually in the third to fifth decades of life There is a slight male predilection although this is not seen in syndrome associated patients The most common site is the posterior neck but may also be seen in other sites extremities lumbosacral area buttocks face 1 See also editFibrous lesionsReferences edit a b c d e f Michal M Fetsch J F Hes O Miettinen M 1999 Nuchal type fibroma Cancer 85 1 156 163 doi 10 1002 SICI 1097 0142 19990101 85 1 lt 156 AID CNCR22 gt 3 0 CO 2 O PMID 9921988 a b c Samadi D S McLaughlin R B Loevner L A Livolsi V A Goldberg A N 2000 Nuchal fibroma A clinicopathological review The Annals of Otology Rhinology and Laryngology 109 1 52 55 doi 10 1177 000348940010900110 PMID 10651413 Sbaraglia M Bellan E Dei Tos AP April 2021 The 2020 WHO Classification of Soft Tissue Tumours news and perspectives Pathologica 113 2 70 84 doi 10 32074 1591 951X 213 PMC 8167394 PMID 33179614 Balachandran K Allen P W MacCormac L B 1995 Nuchal fibroma A clinicopathological study of nine cases The American Journal of Surgical Pathology 19 3 313 317 doi 10 1097 00000478 199503000 00009 PMID 7872429 Dawes L C La Hei E R Tobias V Kern I Stening W 2000 Nuchal fibroma should be recognized as a new extracolonic manifestation of Gardner variant familial adenomatous polyposis The Australian and New Zealand Journal of Surgery 70 11 824 826 doi 10 1046 j 1440 1622 2000 01958 x PMID 11147449 Further reading editLester D R Thompson Bruce M Wenig 2011 Diagnostic Pathology Head and Neck Published by Amirsys Hagerstown MD Lippincott Williams amp Wilkins pp 8 44 45 ISBN 978 1 931884 61 7 Retrieved from https en wikipedia org w index php title Nuchal fibroma amp oldid 1170699167, wikipedia, wiki, book, books, library,
