Copyright (c) 2020 Dr. Hrushikesh S Kolhe
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Mesenteric Fibromatosis with Involvement of Colon, Mimicking as Gastrointestinal Stromal Tumor- a Case Report
Corresponding Author(s) : Dr. Hrushikesh S Kolhe
International Journal of Allied Medical Sciences and Clinical Research,
Vol. 3 No. 2 (2015): 2015 Volume 3- Issue -2
Abstract
Mesenteric Fibromatosis is a locally aggressive myofibroblastic proliferation of the mesentery and adjacent tissues that lacks the capacity to metastasize.Mesenteric Fibromatosis (MF) and gastrointestinal stromal tumors (GISTs) are distinct lesions, but they are often confused with each other. Although rare, it is the most common primary tumor of the mesentery and can develop at any age.Here, we describe a rare case of primary Intra AbdominalFibromatosis involving the mesentery and transverse colon in a 50 year old female which clinically, grossly and histologically mimicked gastrointestinal stromal tumor (GIST). Immunohistochemistry was done which revealed that the tumor mass showed focal coarse granular positivity for c-kit (CD117), while negative for CD34, DOG, desmin, S-100 and SMA. Furthermore, tumor mass showed over expression of ?-catenin. This confirmed the diagnosis of localized mesenteric fibromatosis. Correct identification of both the entities (GIST &Fibromatosis) is of utmost importance as they differ vastly in therapeutic and prognostic considerations.
Keywords
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[1] Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig EB. Intra-abdominal fibromatosis. A pathologic analysis of130 tumors with comparison of clinical subgroups. Am J SurgPathol1990; 14: 335-341
[2] Yannopoulos K, Stout AP. Primary solid tumors of the mesentery. Cancer. 1963;16:914-927.
[3] Al Nafussi A, Wong NA. Intra-abdominal spindle cell lesions: a review and practical aids to diagnosis. Histopathology 2001;38: 387-402
[4] Kindblom LG, Remotti HE, Aldenborg F, MeisKindblom JM. Gastrointestinal stromal tumors show phenotypic characteristicsof the interstitial cells of Cajal. Am J Pathol1998; 152: 1259-1269
[5] Robinson TL, Sircar K, Hewlett BR, Chorneyko K, Riddell RH, Huizinga JD. Gastrointestinal stromal tumors may originatefrom a subset of CD34-positive interstitial cells of Cajal. Am J Pathol2000; 156: 1157-1163
[6] Weiss S, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. St Louis, MO: Mosby; 2001:309-346.
[7] Stout AP. Fibrosarcoma, the malignant tumor of fibroblasts. Cancer. 1948;1:30-63.
[8] Rosai J. GIST: an update. Int J SurgPathol. 2003;11:177-186.
[9] Kempson RL, Fletcher CDM, Evans HL, et al. Tumors of the Soft Tissues. Washington, DC: Armed Forces Institute ofPathology; 2001:78-81.
[10] Bridge JA, Sreekantiah C, Mouron B, et al. Clonal chromosomal abnormalities in desmoid tumors: implications
a. forhistopathogenesis. Cancer.1992;69:430-436.
[11] Li M, Cordon-Cardo C, Gerald W, et al. DesmoidFibromatosis is a clonal process. Hum Pathol. 1996;27:939-943.
[12] Middleton SB, Frayling IM, Phillips RK. Desmoids in familial adenomatous polyposis are monoclonal proliferations. Br J Cancer. 2000;82:827-832.
[13] Alman BA, Li C, Pajerski ME, et al. Increased beta-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors). Am J Pathol. 1997; 151: 329-334.
[14] Yantiss R.K., Sipro I J., Compton C., Rosenberg A.: Gastrointestinal stromal tumor versus intra-abdominal fibromatosis of the bowel wall: a clinically important differential diagnosis. Am. J. Surg. Pathol.2000; 24:947-957.
[15] Monihan J.M., Carr N J., Sobin L.H.: CD34 immunoexpression in stromal tumors of the gastrointestinal tract and in mesenteric fibromatosis. Histopathology 25:469-473, 1994.
[16] Rodriguez J. A., Guarda L.A., Rosa J.: Mesenteric fibromatosis with involvement of the gastrointestinal tract. A GIST simulator: a study of 25 cases. Am. J. Clin. Pathol. 121: 93-98, 2004.
References
[2] Yannopoulos K, Stout AP. Primary solid tumors of the mesentery. Cancer. 1963;16:914-927.
[3] Al Nafussi A, Wong NA. Intra-abdominal spindle cell lesions: a review and practical aids to diagnosis. Histopathology 2001;38: 387-402
[4] Kindblom LG, Remotti HE, Aldenborg F, MeisKindblom JM. Gastrointestinal stromal tumors show phenotypic characteristicsof the interstitial cells of Cajal. Am J Pathol1998; 152: 1259-1269
[5] Robinson TL, Sircar K, Hewlett BR, Chorneyko K, Riddell RH, Huizinga JD. Gastrointestinal stromal tumors may originatefrom a subset of CD34-positive interstitial cells of Cajal. Am J Pathol2000; 156: 1157-1163
[6] Weiss S, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. St Louis, MO: Mosby; 2001:309-346.
[7] Stout AP. Fibrosarcoma, the malignant tumor of fibroblasts. Cancer. 1948;1:30-63.
[8] Rosai J. GIST: an update. Int J SurgPathol. 2003;11:177-186.
[9] Kempson RL, Fletcher CDM, Evans HL, et al. Tumors of the Soft Tissues. Washington, DC: Armed Forces Institute ofPathology; 2001:78-81.
[10] Bridge JA, Sreekantiah C, Mouron B, et al. Clonal chromosomal abnormalities in desmoid tumors: implications
a. forhistopathogenesis. Cancer.1992;69:430-436.
[11] Li M, Cordon-Cardo C, Gerald W, et al. DesmoidFibromatosis is a clonal process. Hum Pathol. 1996;27:939-943.
[12] Middleton SB, Frayling IM, Phillips RK. Desmoids in familial adenomatous polyposis are monoclonal proliferations. Br J Cancer. 2000;82:827-832.
[13] Alman BA, Li C, Pajerski ME, et al. Increased beta-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors). Am J Pathol. 1997; 151: 329-334.
[14] Yantiss R.K., Sipro I J., Compton C., Rosenberg A.: Gastrointestinal stromal tumor versus intra-abdominal fibromatosis of the bowel wall: a clinically important differential diagnosis. Am. J. Surg. Pathol.2000; 24:947-957.
[15] Monihan J.M., Carr N J., Sobin L.H.: CD34 immunoexpression in stromal tumors of the gastrointestinal tract and in mesenteric fibromatosis. Histopathology 25:469-473, 1994.
[16] Rodriguez J. A., Guarda L.A., Rosa J.: Mesenteric fibromatosis with involvement of the gastrointestinal tract. A GIST simulator: a study of 25 cases. Am. J. Clin. Pathol. 121: 93-98, 2004.