Primary Extra-Gastrointestinal Stromal Tumor (GIST) arising from mesentery of small bowel and presenting as abdominal mass: A rare entity

Alok Kumar Tiwari, Anil Kumar Choudhary, Hemant Khowal, Poras Chaudhary, Mohinder P. Arora

Department of Surgery, Dr R.M.L Hospital, New Delhi, India.
Department of Surgery, Lady Hardinge Medical College, New Delhi, India.

DOI: 10.4236/ojgas.2013.35045                

Abstract

Introduction: Majority of mesenchymal tumors of gastrointestinal tract are Gastrointestinal Stromal Tumor (GIST). It is, however, a rare tumor, accounting for less than 1% of primary gastrointestinal (GI) neoplasms. Though, these tumors are refractory to conventional chemotherapy or radiotherapy but show a good response to targeted adjuvant chemotherapy with tyrosine kinase inhibitors following surgical resection. Case Report: we report here a case of primary Extra-GIST tumor arising from mesentry of small bowel near duodeno-jejunal junction in a 69 years old male patient. The patient presented with a palpable mass in upper abdomen for past 15 days. On examination, a non-tender mobile lump of size around 17 × 10 cm, with bosselated surface and firm in consistency was palpable involving epigastric, left hypochondrium and umbilical region. Contrast enhanced computed tomography of abdomen revealed a heterogenous mesentric mass. On surgical intervention a mass was found involving mesentery near dudenojejunal junction without involvement of gastrointestinal tract. Complete surgical resection of the tumor was done and adjuvant chemotherapy with Imatinib mesylate was started as HPE revealing GIST with mitotic index of >10/50 HPF and 17 × 10 cm size placed the patient in high risk category. Patient was discharged on 12th of post-operative day with advice of regular follow-up. Conclusion: GIST occurrence is not restricted to bowel but can involve unusual sites also. The mainstay of treatment remains surgical resection with adequate margin. In cases where tumour has malignant potential (high mitotic figures on histopathology) adjuvent treatment with tyrosine kinase may prevent or delay relapse.

Keywords

Extra-Gastrointestinal Stromal Tumors; GIST; Mesentric Tumors; Imatinib Mesylate; Abdominal Mass

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	Mazur, M.T. and Clark, H.B. (1983) Gastric stromal tumours. Reappraisal of their histogenesis. American Journal of Surgical Pathology, 7, 507-519. doi:10.1097/00000478-198309000-00001
[2]	Miettinen, M. and Lasota, J. (2001) Gastrointestinal stromal tumors: Definition, clinical, histological, immune-histochemical, and molecular genetic features and differential diagnosis. Virchows Arch, 438, 1-12. doi:10.1007/s004280000338
[3]	Filippou, D.K., Pashalidis, N., Skandalakis, P. and Rizos, S. (2006) Malignant gastrointestinal stromal tumor of the ampulla of Vater presenting with obstructive jaundice. Journal of Postgraduate Medicine, 52, 204-206.
[4]	Towu, E. and Stanton, M. (2006) Gastrointestinal stromal tumour presenting with severe bleeding: A review of the molecular biology. Pediatric Surgery International, 22, 462-464. doi:10.1007/s00383-006-1636-5
[5]	Torihashi, S., Nishi, K., Tokutomi, Y., Nishi, T., Ward, S. and Sanders, K.M. (1999) Blockade of kit signaling induces transdifferentiation of interstitial cells of Cajal to a smooth muscle phenotype. Gastroenterology, 117, 140-148. doi:10.1016/S0016-5085(99)70560-3
[6]	Young, H.M., Ciampoli, D. and Southwell Newgreen, D.F. (1996) Origin of interstitial cells of Cajal in the mouse intestine. Developmental Biology, 180, 97-107. doi:10.1006/dbio.1996.0287
[7]	Sinha, R., Verma, R. and Kong, A. (2004) Mesenteric gastrointestinal stromal tumor in a patient with neurofibromatosis. American Journal of Roentgenology, 183, 1844-1846. doi:10.2214/ajr.183.6.01831844
[8]	Basile, A., Kettenbach, J., Mundo, E., et al. (2006) Erratum: Primitive mesenteric gastrointestinal stromal tumor with autonomic nerve/ganglionic differentiation presenting as a huge mass with small synchronous nodules. European Radiology, 16, 519. doi:10.1007/s00330-005-2845-3
[9]	Gupta, N., Mittal, S., Lal, N., Misra, R., Kumar, L. and Bhalla, S. (2007) A rare case of primary mesenteric gastrointestinal stromal tumor with metastasis to the cervix uteri. World Journal of Surgical Oncology, 5, 137. doi:10.1186/1477-7819-5-137
[10]	Patil, S., Jain, S., Kaza, R.C.M. and Chamberlain, R.S. (2011) Giant gastrointestinal stromal tumor presenting as a palpable abdominal mass: An unusual presentation. ISRN Surgery, 2011, Article ID: 894829.
[11]	Miettinen, M., Monihan, J.M., Sarlomo-Rikala, M., Kovatich, A.J., Carr, N.J., Emory, T.S. and Sobin, L.H. (1999) Gastrointestinal stromal tumors/smooth muscle tumors/GISTs in the omentum and mesentery—Clinicopathologic and immunohistochemical study of 26 cases. American Journal of Surgical Pathology, 23, 1109-1118. doi:10.1097/00000478-199909000-00015
[12]	Reith, J.D., Goldblum, J.R., Lyles, R.H. and Weiss, S.W. (2000) Extragastrointestinal (soft tissue) stromal tumors. An analysis of 48 cases with GIST 23 emphasis on histological predictors of outcome. Modern Pathology, 13, 577-585. doi:10.1038/modpathol.3880099
[13]	Chourmouzi, D., Sinakos, E., Papalavrentios, L., Akriviadis, E. and Drevelegas A. (2009) Gastrointestinal stromal tumors: A pictorial review. Journal of Gastrointestinal and Liver Diseases, 18, 379-383.
[14]	Blay, J.Y., Bonvalot, S., Casali, P., et al. (2005) Consensus meeting for the management of gastrointestinal stromal tumors. Annals of Oncology, 16, 993.
[15]	Cunningham, R.E., Federspiel, B.H., McCarthy, W.F., Sobin, L.H. and O’Leary, T.J. (1993) Predicting prognosis of gastrointestinal smooth muscle tumors. Role of clinical and histologic evaluation, flow cytometry, and image cytometry. American Journal of Surgical Pathology, 17, 588-594. doi:10.1097/00000478-199306000-00006
[16]	Emory, T.S., Sobin, L.H., Lukes, L., Lee, D.H. and O’Leary, T.J. (1999) Prognosis of gastrointestinal smooth-muscle (stromal) tumors. Dependence on anatomic site. American Journal of Surgical Pathology, 23, 82-97. doi:10.1097/00000478-199901000-00009
[17]	Miettinen, M., Sarlomo-Rikala, M., Sobin, L.H. and Lasota, J. (2000) Esophageal stromal tumors: A clinicopathologic, immunohistochemical, and molecular genetic study of 17 cases and comparison with esophageal leiomyomas and leiomyosarcomas. American Journal of Surgical Pathology, 24, 211-222. doi:10.1097/00000478-200002000-00007
[18]	DeMatteo, R.P., Lewis, J.J., Leung, D., et al. (2000) Two hundred gastrointestinal stromal tumors: Recurrence patterns and prognostic factors for survival. Annals of Surgery, 231, 51-58. doi:10.1097/00000658-200001000-00008
[19]	Miettinen, M., Makhlouf, H., Sobin, L.H., et al. (2006) Gastrointestinal stromal tumors of the jejunum and ileum: A clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with longterm follow-up. American Journal of Surgical Pathology, 30, 477-489. doi:10.1097/00000478-200604000-00008
[20]	Allander, S.V., Nupponen, N.N., Rigner, M., Hostetter, G., Maher, G.W., Goldberger, N., Chen, Y., Carpten, J., Elkahloun, A.G. and Meltzer, P.S. (2001) Gastrointestinal stromal tumors with KIT mutation exhibit a remarkably homogenous gene expression profile. Cancer Research, 61, 8264-8268.