Desmoid tumor pathophysiology: Difference between revisions
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{{Desmoid tumor}} | {{Desmoid tumor}} | ||
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==Overview== | ==Overview== | ||
Desmoid tumor may occur as part of [[Gardner syndrome]]. A minority of desmoid tumors are associated with [[Turcot syndrome]], [[Familial adenomatous polyposis]], and estrogen therapy.<ref name="radio"> Desmoid tumor. Dr Tim Luijkx and Radswiki et al. Radiopedia 2015 http://radiopaedia.org/articles/aggressive-fibromatosis. Accessed on January 20, 2015</ref> | Desmoid tumor may occur as part of [[Gardner syndrome]]. A minority of desmoid tumors are associated with [[Turcot syndrome]], [[Familial adenomatous polyposis]], and estrogen therapy.<ref name="radio"> Desmoid tumor. Dr Tim Luijkx and Radswiki et al. Radiopedia 2015 http://radiopaedia.org/articles/aggressive-fibromatosis. Accessed on January 20, 2015</ref> | ||
Revision as of 20:27, 27 February 2019
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Desmoid tumor Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]Faizan Sheraz, M.D. [3]
Overview
Desmoid tumor may occur as part of Gardner syndrome. A minority of desmoid tumors are associated with Turcot syndrome, Familial adenomatous polyposis, and estrogen therapy.[1]
Pathophysiology
Their exact aetiology remains uncertain, although they are frequently associated with previous trauma or surgical incision. On the molecular level, desmoids are characterised by mutations in the β-catenin gene, CTNNB1, or the adenomatous polyposis coli gene, APC.[1]
Associated Diseases
- Some cases have been associated with estrogen therapy
- Turcot syndrome
- Gardner syndrome
- Familial adenomatous polyposis
- In the case of mesenteric desmoid they are seen either sporadically or in association with familial polyposis coli syndrome(FAP)
Gross Pathology
- Abdominal wall desmoid tumors arise from:
- Musculoaponeurotic structures of the abdominal wall (especially the rectus and internal oblique muscles and their fascial coverings)
- External oblique muscle and the transversalis muscle or fascia
- These masses have a firm, gritty texture. On the cut surface, they are glistening white and coarsely trabeculated, resembling scar tissue
Location
Frequent locations in the abdomen are:
- Abdominal wall
- Root of the mesentery
- Retroperitoneum
Microscopic Pathology
Histologically, desmoid tumors consist of:[2]
- Elongated fibroblasts.
- Myofibroblasts characterized by elongated, tapered cytoplasm; elongated, vesicular, typical-appearing nuclei; and multiple small nucleoli. The cells are linearly arranged and are surrounded and separated from each other by collagen.
- These tumors show a tendency to evolve over time.
- Vandevenne et al described three stages of evolution of desmoid tumors:
- In the first stage, lesions are more cellular and have fewer areas of hyalinized collagen.
- In the second stage, there is an increasing amount of collagen deposition in the central and peripheral areas of the tumor.
- In the third stage, there is an increase in the fibrous composition with a decrease in cellularity and water content.
Reference
- ↑ 1.0 1.1 Desmoid tumor. Dr Tim Luijkx and Radswiki et al. Radiopedia 2015 http://radiopaedia.org/articles/aggressive-fibromatosis. Accessed on January 20, 2015
- ↑ Economou, Athanasios; Pitta, Xanthi; Andreadis, Efstathios; Papapavlou, Leonidas; Chrissidis, Thomas (2011). "Desmoid tumor of the abdominal wall: a case report". Journal of Medical Case Reports. 5 (1): 326. doi:10.1186/1752-1947-5-326. ISSN 1752-1947.