Desmoid tumor pathophysiology: Difference between revisions
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Frequent locations in the abdomen are the abdominal wall, the root of the mesentery and the retroperitoneum. | Frequent locations in the abdomen are the abdominal wall, the root of the mesentery and the retroperitoneum. | ||
Histologically, desmoid tumors consist of elongated fibroblasts and 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. | Histologically, desmoid tumors consist of elongated fibroblasts and 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.<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | ||
==Reference== | ==Reference== | ||
Revision as of 16:38, 21 January 2016
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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: Faizan Sheraz, M.D. [2]
Overview
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
- Gardner syndrome
- In the case of mesenteric desmoid they are seen either sporadically or in association with familial polyposis coli syndrome (FAP): 9-18 of FAP cases may have a desmoid tumor.
Location
Frequent locations in the abdomen are the abdominal wall, the root of the mesentery and the retroperitoneum.
Histologically, desmoid tumors consist of elongated fibroblasts and 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.[2]
Reference
- ↑ 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.