Abstract
A 38-year-old woman with generalized neurofibromatosis was admitted to the emergency department with complaint of pain and nontender mass in epigastrium. She had been treated in another hospital as an intra-abdominal abscess and tube drain was seen to be coming out of the centre of the mass. Later investigations and surgery revealed a large exophytic tumor arising from the posterior wall of the stomach infiltrating the transverse colon. Resection of the involved stomach and middle third of the transverse colon was done. Histology examination proved to be leiomyosarcoma positive for CD117 and S-100. Gastrointestinal stromal tumors (GISTs) in neurofibromatosis type 1 (NF1) are multiple and commonly involve the small intestine. This is a rare case of GISTs in NF1 involving the stomach and presenting as an intra-abdominal abscess.
References
Kingham P, DeMatteo RP (2009) Multidisciplinary treatment of gastrointestinal stromal tumours. Surg Clin North Am 89:217–233
Miettinen M, Lasda J (2006) Gastrointestinal tumours, review on morphology, molecular pathology, prognosis and differential diagnosis. Arch Pathol Lab Med 130:1466–1478
Kanthalia CV, Prabbu RY et al (2008) Presentation of gastrointestinal stromal tumours. Ind J Surg 70:318–321
Miettinen M, Sobin LH, Lasota J (2005) Gastrointestinal stromal tumours of the stomach: a clinicopathologic, immunohistochemical and molecular genetic study of 1765 cases with long term follow up. Am J Surg Pathol 29(1):52–68
Tkazawaa Y, Sakurai S, Sakuma Y, Ikeda T, Yamaguchi J (2005) Gastro-intestinal stromal tumours of neurofibromatosis type 1. Am J Surg Pathol 29(6):755–763
Ng EH, Pollock RE, Munsell MF et al (1992) Prognostic factors influencing survival in gastrointestinal leiomyosarcomas: implications for surgical management and staging. Ann Surg 215:68–77
Dematteo RP, Lewis JJ, Leung D et al (2000) Two hundred gastrointestinal stromal tumours, recurrence patterns and prognostic factors for survival. Ann Surg 231(1):51–58
Verweij J, Casali PG, Zalcberg J et al (2004) Progression free survival in gastrointestinal stromal tumours with high dose imatinib a randomised trial. Lancet 364(9440):1127–1134
Author information
Authors and Affiliations
Corresponding author
Additional information
Few previous publications by authors
1. Gastric carcinoid – A case report. Ind J Surgery1991;53:322–324.
2. Solitary intrahepatic choledochal cyst. Ind J Surgery 1993; 55: 294.
3. Morbidity and mortality of definitive surgical procedure in Duodenal ulcer perforation. Ind J Surgery 1994;56:102.
4. Malignant granular cell tumour – A case report and review of Literature. Ind J Cancer 1992; 29:31–33.
5. Colostomy closure and wound infection; Evaluation of risk factors. Ind J Surgery 1997;59:211–214.
6. Delay in surgery for acute appendicitis. ANZ J Surgery2004;74:9.
7. Treatment of anal fissure using a combination of minoxidil and Lignocaine a randomized double blind trial. Ind J GE 2005;24:158.
Electronic supplementary material
Below is the link to the electronic supplementary material.
ESM 1
(JPEG 233 kb)
Rights and permissions
About this article
Cite this article
Swain, S.K., Smile, R., Arul, T. et al. Unusual Presentation of Gastrointestinal Stromal Tumor of Stomach in Neurofibromatosis Type 1: A Case Report. Indian J Surg 75 (Suppl 1), 398–400 (2013). https://doi.org/10.1007/s12262-012-0572-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12262-012-0572-y