Pleura
Other tumors
Fibromatosis


Topic Completed: 1 March 2014

Revised: 1 March 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Fibromatosis pleura full text[sb]

See also: Soft tissue chapter

Vaidehi Avadhani, M.D.
Page views in 2018: 970
Page views in 2019 to date: 1,694
Cite this page: Avadhani V. Fibromatosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pleurafibromatosis.html. Accessed November 13th, 2019.
Definition / general
  • Borderline neoplasm of deep soft tissue
Terminology
  • Also called "desmoid tumor," aggressive fibromatoses
Epidemiology
Sites
  • Deep soft tissue, abdomen, pelvis, peritoneum
Etiology
  • In FAP (2% of all desmoids), tumors are due to germline mutations
  • In sporadic desmoids, somatic mutations in β catenin or APC occur, leading to stabilization of the β catenin protein
Clinical features
  • Intrathoracic fibromatoses does not cause symptoms until mass is large enough to compress pulmonary parenchyma or locally invade surrounding structures
Radiology description
  • Well circumscribed on imaging with homogenous radiodensity
Radiology images

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Typical localization of desmoid tumors

Desmoid tumor of right musculus deltoideus

Desmoid of thoracic / abdominal wall

Desmoid tumor (arrows)

Heterogeneous uptake (arrows)

Case reports
Treatment
  • Complete surgical excision with wide surgical margin is curative
  • Local recurrences can occur with positive margins (Ann Thorac Surg 2004;78:1219)
Clinical images

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Desmoid tumor originates from transverse mesocolon

Gross description
  • Fleshy, uniform tan cut surface with fibrous bands
  • May deceptively appear to have a well circumscribed interface with surrounding tissue but microscopically the tumor infiltrates surrounding tissue
Gross images

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Pleural tumors

Colonic tumor

Microscopic (histologic) description
  • Bland spindle cells arranged in sweeping bundles in a highly collagenized background, with scattered thick walled small blood vessels with open lumina and a small number of chronic inflammatory cells
Microscopic (histologic) images

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H&E

H&E (colon)

H&E (thigh)

H&E, β catenin, SMA, CD34

Cytology images

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FNA smears

Positive stains
Negative stains
Differential diagnosis
Additional references
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