The online version of this article (doi:10.1186/1752-1947-8-321) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
All of the authors were involved in the conception of the case report, the data collection and the literature review as well as in writing the manuscript. SMK performed gross and histological examination of resected specimen, including the immunohistochemistry interpretation and was a major contributor in writing the manuscript. IK and LK reviewed the literature. MK contributed to data collection and to histological and immunohistochemistry interpretation. VSM and AJS analyzed and interpreted the clinical data and were involved in drafting the manuscript. VK contributed in the selection of slides for illustration of the case. All authors read and approved the final manuscript.
Gastrointestinal stromal tumor is the most common sarcoma of the gastrointestinal tract. We report a case of gastrointestinal stromal tumor in a small intestine, initially suspected for leiomyosarcoma given that gastrointestinal stromal tumors in young adult patients are limited due to their rarity.
A 30-year-old Caucasian ethnic Albanian woman from Kosovo presented with abdominal pain, nausea and vomiting. Subsequently, the tumor was detected in her small intestine, as an infiltrating mass approximately 10cm in diameter. The tumor was resected en bloc and duodenojejunal terminal-terminal anastomosis was performed. The tumor was a large, bulky, intramural mass, with fish-flesh to tan-brown appearance, as well as with foci of hemorrhage and necrosis. On histological examination the tumor showed transmural growth, deep infiltrative pattern and malignant feature, with mitotic count >5 per 50 high-power field, dense cellularity with plump spindle cells, and with eosinophilic cytoplasm within variably hyalinized and edematous stroma, skeinoid fibers (extracellular collagen globules) and foci of hemorrhage. In addition, the tumor was composed of areas with epithelioid morphology. The immunohistochemistry results showed high expression of proto-oncogene c-kit, CD117, CD34 and vimentin, whereas α-smooth muscle actin was focally positive. Desmin and S-100 protein were negative.
Gastrointestinal stromal tumor should be included in the differential diagnoses of intestinal mesenchymal tumors presenting as a single mass in young female adults. Given that gastrointestinal stromal tumors in young adults represent a more heterogeneous group than gastrointestinal stromal tumor in pediatric cases, more effort should be made to investigate its pathogenesis and potentially more specific treatment.
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Hirota S, Isozaki K, Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, Kawano K, Hanada M, Kurata A, Takeda M, Muhammad Tunio G, Matsuzawa Y, Kanakura Y, Shinomura Y, Kitamura Y: Gain-of-function mutations of c- kit in human gastrointestinal stromal tumors. Science. 1998, 279 (5350): 577-580. 10.1126/science.279.5350.577. CrossRefPubMed
Bosman FT, Carneiro F, Hruban RH, Theise ND: IARC WHO Classification of Tumours of the Digestive System. 2010, Geneva: World Health Organization, 4
Yen CC, Yeh CN, Cheng CT, Jung SM, Huang SC, Chang TW, Jan YY, Tzeng CH, Chao TC, Chen YY, Yang CY, Ho CL, Fletcher JA: Integrating bioinformatics and clinicopathological research of gastrointestinal stromal tumors: identification of aurora kinase A as a poor risk marker. Ann Surg Oncol. 2012, 19 (11): 3491-3499. 10.1245/s10434-012-2389-0. CrossRefPubMed
Corless CL, Barnett CM, Heinrich MC: Gastrointestinal stromal tumours: origin and molecular oncology. Nat Rev Cancer. 2011, 11 (12): 865-878. PubMed
Italiano A, Bui B: Gastrointestinal stromal tumors: molecular aspects and therapeutic implications. Bull Cancer. 2008, 95 (1): 107-116. PubMed
Shiba K, Matsumoto T, Hirota S: Small-molecule inhibitors against KIT and PDGFRs especially in GISTs. Gan To Kagaku Ryoho. 2009, 36 (7): 1080-1085. PubMed
Liang H: Treatment strategy for recurrent and metastatic gastrointestinal stromal tumors. Zhonghua Wei Chang Wai Ke Za Zhi. 2014, 17 (4): 309-311. PubMed
Uno F, Fujiwara Y, Fujiwara T: A long-term control of gastrointestinal stromal tumor with sunitinib. Gan To Kagaku Ryoho. 2013, 40 (9): 1241-1244. PubMed
Heinrich MC, Maki RG, Corless CL, Antonescu CR, Harlow A, Griffith D, Town A, McKinley A, Ou WB, Fletcher JA, Fletcher CD, Huang X, Cohen DP, Baum CM, Demetri GD: Primary and secondary kinase genotypes correlate with the biological and clinical activity of sunitinib in imatinib-resistant gastrointestinal stromal tumor. J ClinOncol. 2008, 26 (33): 5352-5359. 10.1200/JCO.2007.15.7461. CrossRef
Fayet Y, Chasles V, Ducimetière F, Collard O, Berger C, Meeus P, Ranchère-Vince D, Thiesse P, Sunyach MP, Marec-Bérard P, Poncet L, Cousin P, Blay JY, Ray-Coquard I: To answer rare cancer issues. Geographical analysis of EMS sarcoma cohort in the Rhône-Alpes region. Bull Cancer. 2014, 101 (2): 127-136. PubMed
Starczewska Amelio JM, Cid Ruzafa J, Desai K, Tzivelekis S, Muston D, Khalid JM, Ashman P, Maguire A: Prevalence of gastrointestinal stromal tumour (GIST) in the United Kingdom at different therapeutic lines: an epidemiologic model. BMC Cancer. 2014, 14 (1): 364-10.1186/1471-2407-14-364. CrossRefPubMedPubMedCentral
Wang ZH, Liang XB, Wang Y, Ma GL, Qu YQ, Tian XW: Epidemiology survey of gastrointestinal stromal tumor in Shanxi Province in 2011. Zhonghua Yi Xue Za Zhi. 2013, N93 (32): 2541-2544.
Wang ZQ, Wang S, Ye YJ, Kang YL, Sun KK, Zheng HF: Gastrointestinal mesenchymal tumors: a clinical pathologic and immunohistochemical study of 210 cases. Zhonghua Wei Chang Wai Ke Za Zhi. 2007, 10 (1): 11-16. PubMed
Folgado AS, Sánchez P, Oliveira M, Cuesta L, Gomes F, Figueiredo A, Pinheiro N, Ramos de Deus J: Gastrointestinal stromal tumors – a retrospective study of 43 cases. Rev Esp Enferm Dig. 2008, 100 (11): 696-700.
Machado NO, Chopra PJ, Al-Haddabi IH, Al-Qadhi H: Large duodenal gastrointestinal stromal tumor presenting with acute bleeding managed by a whipple resection. A review of surgical options and the prognostic indicators of outcome. JOP. 2011, 12 (2): 194-199. PubMed
Lv A, Li Z, Tian X, Guan X, Zhao M, Dong B, Hao C: SKP2 high expression, KIT exon 11 deletions, and gastrointestinal bleeding as predictors of poor prognosis in primary gastrointestinal stromal tumors. PLoS One. 2013, 17: 8(5)-
Liang YM, Li XH, Lü YY, Lü YL, Zhong M, Pu XL, Li WM: Prognostic significance of clinicopathologic parameters in gastrointestinal stromal tumor: a study of 156 cases. Zhonghua Bing Li Xue Za Zhi. 2007, 36 (4): 233-238. PubMed
Prakash S, Sarran L, Socci N, DeMatteo RP, Eisenstat J, Greco AM, Maki RG, Wexler LH, LaQuaglia MP, Besmer P, Antonescu CR: Gastrointestinal stromal tumors in children and young adults: a clinicopathologic, molecular, and genomic study of 15 cases and review of the literature. J Pediatr Hematol Oncol. 2005, 27 (4): 179-187. 10.1097/01.mph.0000157790.81329.47. CrossRefPubMed
Shimomura M, Ikeda S, Takakura Y, Kawaguchi Y, Tokunaga M, Takeda H, Sumitani D, Yoshimitsu M, Hinoi T, Okajima M, Ohdan H: Gastrointestinal stromal tumors of the small intestine in pediatric populations: a case report and literature review. Pediatr Surg Int. 2010, 26 (6): 649-654. 10.1007/s00383-010-2596-3. CrossRefPubMed
Agaimy A, Vassos N, Wunsch PH, Hohenberger W, Hartmann A, Croner RS: Impact of serosal involvement/extramural growth on the risk of synchronous and metachronous peritoneal spread in gastrointestinal stromal tumors: proposal for a macroscopic classification of GIST. Int J Clin Exp Pathol. 2012, 5 (1): 12-22. PubMedPubMedCentral
Skipworth J, Fanshawe A, West M, Al-Bahrani A: Perforation as a rare presentation of gastric gastrointestinal stromal tumours: a case report and review of the literature. Ann R Coll Surg Engl. 2014, 96 (1): 96-100. 10.1308/003588414X13824511650010. CrossRef
Boudabous H, Chaker Y, Nouira R, Dziri C: Stromal gastrointestinal tumors: retrospective study about 24 cases. Tunis Med. 2013, 91 (11): 661-667. PubMed
Menéndez P, Padilla D, Cubo T, Villarejo P, Gambí D, Menéndez JM, Colina F, García M, Lora D, Martín J: Biological behavior due to cell proliferation markers of gastrointestinal stromal tumors. Hepatogastroenterology. 2011, 58 (105): 76-80. PubMed
Bauer S, Rutkowski P, Hohenberger P, Miceli R, Fumagalli E, Siedlecki JA, Nguyen BP, Kerst M, Fiore M, Nyckowski P, Hoiczyk M, Cats A, Casali PG, Treckmann J, van Coevorden F, Gronchi A: Long-term follow-up of patients with GIST undergoing metastasectomy in the era of imatinib – analysis of prognostic factors (EORTC-STBSG collaborative study. Eur J Surg Oncol. 2014, 40 (4): 412-419. 10.1016/j.ejso.2013.12.020. CrossRefPubMed
- Small intestinal gastrointestinal stromal tumor in a young adult woman: a case report and review of the literature
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