An unusual presentation of a retroperitoneal gastrointestinal stromal tumors


  • Samir Paruthy Consultant Surgeon, East Delhi Medical Centre, Shahdara, Delhi, India Holy Child Nursing Home, Krishna Nagar, Delhi, India
  • Shivani B. Paruthy Department of Surgery, Safdarjung Hospital and VMMC, New Delhi, India



Retroperitoneum, GIST, Platelet-derived growth factor receptor α polypeptide


Gastrointestinal stromal tumors (GIST) are soft tissue mesenchymal tumor originating from intestinal cells of Cajal which are pace makers of the intestine. GIST commonly arises from stomach and intestine although they seem to appear occasionally in mesentery, omentum and esophagus, but origin from retro-peritoneum is very infrequent.  We herein report case of a huge retroperitoneal GIST measuring 16x13x9 cm size, in a 52-year-old lady occupying whole abdomen arising as pelvic mass. GIST was initially mistaken with associated large intramural fibroid uterus and a large pedunculated sub-serosa broad ligament fibroid. Patient was taken for abdominal open hysterectomy with complete tumor excision after all adhesions from adjoining intestine was separated. Histopathological examination of the mass confirmed it was retroperitoneal GIST with positive immune-histochemistry. Patient responded well to Imatinib therapy, and for last 4 years there has been no residual disease or recurrence as evidenced with regular follow up. 

Author Biography

Shivani B. Paruthy, Department of Surgery, Safdarjung Hospital and VMMC, New Delhi, India

professor and consultant


Matthews BD, Joels CS, Kercher KW, Heniford BT. Gastrointestinal stromal tumors of the stomach. Minerva chirurgica. 2004;59:219-31.

Szanto P, Barbus A, Al Hajjar N, Zaharia T, Manciula D. Gastric stromal tumor: a rare cause of upper gastrointestinal bleeding. J gastrointestinal liver dis. 2007;16:441-3.

Sanders KM, Koh SD, Ward SM. Interstitial cells of cajal as pacemakers in the gastrointestinal tract. Ann review physiol. 2006;68:307-43.

Miettinen M, Monihan JM, Sarlomo-Rikala M, Kovatich AJ, Carr NJ, Emory TS, Sobin LH. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunohistochemical study of 26 cases. Am J surgical pathol. 1999;23:1109-18.

Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ et al. Diagnosis of gastrointestinal stromal tumors: A consensus approach. Human pathol. 2002;33:459-65.

Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch pathol lab med. 2006;130:1466-78.

Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Seminars diagnostic pathol. 2006;23:70-83.

Miettinen M, El-Rifai W, Lasota J. Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a review. Human pathol. 2002;33:478-83.

Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998;279:577-80.

Heinrich MC, Corless CL, Duensing A, McGreevey L, Chen CJ, Joseph N et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science. 2003;299:708-10.

Heinrich MC, Owzar K, Corless CL, Hollis D, Borden EC, Fletcher CD et al. Correlation of kinase genotype and clinical outcome in the North American Intergroup Phase III Trial of imatinib mesylate for treatment of advanced gastrointestinal stromal tumor: CALGB 150105 Study by Cancer and Leukemia Group B and Southwest Oncology Group. J clin oncol. 2008;26:5360-7.

Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. J clin oncol. 2004;22:3813-25.

Blanke CD, Demetri GD, Von Mehren M, Heinrich MC, Eisenberg B, Fletcher JA et al. Long-term results from a randomized phase II trial of standard- versus higher-dose imatinib mesylate for patients with unresectable or metastatic gastrointestinal stromal tumors expressing KIT. J clin oncol. 2008;26:620-5.

Cirocchi R, Farinella E, La Mura F, Cavaliere D, Avenia N, Verdecchia GM et al. Efficacy of surgery and imatinib mesylate in the treatment of advanced gastrointestinal stromal tumor: a systematic review. Tumori. 2010;96:392-9.

Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY et al. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet. 2004;364:1127-34.

Demetri GD, Van Oosterom AT, Garrett CR, Blackstein ME, Shah MH, Verweij J et al. Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet. 2006;368:1329-38.






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