VULVAR PARAURETRAL EKSTRAGASTROINTESTİNAL STROMAL TUMOR; A CASE REPORT

Abstract

SUMMARY

Gastrointestinal Stromal Tumors (GISTs) are mesenchymal tumors that are arises from the wall of the gastrointestinal tract and is throught to originate from the Interstitial Cells of Cajal (ICC), which regulate the motility of the gastrointestinal tract. More rarely neoplasms with histology and immunohistochemistry similar to gastrointestinal stromal tumors may ocur outside the gastrointestinal tract and are so-called Extra-gastrointestinal Stromal Tumors (EGISTs). Surgical removal  is the gold standard treatment for non-metastatic Extragastrointestinal stromal tumors. We reported a very rare case of ekstragastrointestinal stromal tumor arising from the vulva in this article.

Keywords

• EKSTRA GASTROINTESTINAL STROMAL TUMOR,GASTROINTESTINAL STROMAL TUMOR,PARAURETRAL MASS,VULVAR MASS

VULVADA PARAÜRETRAL EKSTRAGASTROİNTESTİNAL STROMAL TÜMÖR; OLGU SUNUMU

Öz

ÖZET

Gastrointestinal Stromal Tümörler (GİST), gastrointestinal sistem duvarı peristaltizmini düzenleyen İnterstisyel Cajal Hücreleri’nden (İCH) kaynaklandığı düşünülen mezenkimal tümörlerdir. Ekstragastrointestinal  Stromal Tümörler (EGİST) daha nadir görülen, histoloji ve immünohistokimlyasal açıdan gastrointestinal stromal tümörler ile benzer ancak gastro intestinal yolak dışında oluşan tümörlerdir. Metastatik olmayan ekstragastrointestinal stromal tümörlerin cerrahi olarak çıkarılması altın standart tedavidir. Bu makalede, nadiren vulvadan köken alan ekstragastrointestinal stromal tümörlü bir olgu sunduk.

Anahtar Kelimeler

• EKSTRA GASTROİNTESTİNAL STROMAL TÜMÖR,GASTROİNTESTİNAL STROMAL TÜMÖR,PARAÜTERRAL KİTLE,VULVAR KİTLE

Kaynakça

1. 1. Gold JS,Dematteo RP. Combined surgıcal and molecular therapy: the gastrointestinal stromal tumor model. Ann Surg 2006;244:176-84.
2. 2. Kindblom LG, Remotti HE, Aldenborg F. Gastrointestinal pacemaker cell tumor (GIPACT). Gastrointestinal stromal tumors show phenotypic characteristics of intestinal cell of Cajal. Am J Pathol. 1998:152(5):1259-69.
3. 3. Heinrich MC, Corless CL, Duensing A, Chen CJ, Joseph N, et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science. 2003;299:708-10.
4. 4. Hirota S, Ohashi A, Nishida T, Isozaki K, Kinoshita K, Shinomura Y, et al. Gain-of-function mutations of platelet-derived growht factor receptor alpha gene in gastrointestinal stromal tumors. Gastroenterology. 2003;125:660-7.
5. 5. Mortensen MB, Larsen KE, Fristrup CW, Nielsen HO. Gastrointestinal stromal tumor: clinical and pathological presentation. Ungeskr Laeger. 2007;169:2776-9.
6. 6. Yamamoto H, Oda Y, Kawaguchi K, et al. C-kit and PDGFRA mutations in extragastrointestinal stromal tumor (gastrointestinal stromal tumor of the soft tissue) Am J Surg Pathol, 2004;28(4):479-88.
7. 7. Gun BD, Gun MO, Karamanoglu Z. Primary stromal tumor of the omentum: report of a case. Surg Today 2006:36:994-6.
8. 8. Li J, Gong JF, Wu AW, Shen L. Post-operative imatinib in patients with intermediate or high risk gastrointestinal stromal tumor. Eur J Cancer Surg 2001:37:319-24.

9. 9. Nilsson B, Sjölund K, Kindblom LG, Meis-Kindblom JM, Bümming P, Nilsson O, Anderson J and Ahlman H: Adjuvant imatinib treatment improves recurrence-free survival in patients with high-risk gastrointestinal stromal tumors (GIST). Br J Cancer 96: 1656-1658, 2007.
10. 10. De Matteo RP, Ballman KV, Antonescu CR, Maki RG, Pisters PW, Demetri GD et al. Adjuvant imatinib mesylate after resection of localized primary gastrointestinal stromal tumour: a randomized, double-blind, placebo-controlled trial. Lancet. 2009;373;1097-104.
11. 11. Hirota S, Isozaki K, Moriyama Y, Hashimato K, Nishida T, Ishiguro S et al. Gain of function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998:279(5350):577-80.
12. 12. Fletcher CDM, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol. 2002:33:459-65.
13. 13. Connoly EM, Gaffney E, Reynolds JV. Gastrointestinal stromal tumors. Br J Surg. 2003:90:1178-86.
14. 14. Miettinen M, Lasota J. Gastrointestinal stromal tumors: definition, clinical, histological, immunohistochemical and molecular genetic features and differential diagnosis. Virchows Arch. 2001:438:1-12.
15. 15. Reith JD, Goldblum JR, Lyles RH, Weiss SW. Extragastrointestinal (soft-tissue) stromal tumors: an analysis of 48 cases with emphasis on histologic predictors of outcome. Mod Pathol. 2000:13(5):577-85.
16. 16. Park SS, Min BW, Kim WB, Choi JW, Lee JH, Chae YS, et al. Malignant extragastrointestinal stromal tumors of retroperitoneum. Acta Oncol. 2005;44(5):497-9.
17. 17. Lognoro R, Jones DV, Faruqi S, Bhutani MS. Recent advances in cell biology, diagnosis and therapy of gastrointestinal stromal tumor (GIST) . Cancer Biol Ther. 2004;3:251-8.
18. 18. Barreda-Bolanos F, Liu Bejarano H, Sanchez Lihon J, Landeo Aliaga I, Sanchez Rodriguez Z. Survival factors in 152 patients with gastrointestinal stromal tumor . Rev Gastroenterol Peru. 2010;30:305-23.
19. 19. Barros A, Linhares E, Valadao M, et al. Extragastrointestinal stromal tumor (EGIST) : a series of case reports. Hepatogastroenterology 2011:58:865-868.
20. 20. Yamamato H, Kojima A, Nagata S, Tomita Y, Takahashi S, Oda Y. KIT negative gastrointestinal stromal tumor of the abdominal soft tissue : a clinicopathologic and genetic study of 10 cases. Am J Surg Pathol. 2011;35:1287-1295.
21. 21. Sakurai S, Hishima T, Takazawa Y, Sano T, Nakajima T, Saito K, et al. Gastrointestinal stromal tumors and KIT-positive mesenchymal cells in the omentum. Pathol Int. 2001:51:524-31.
22. 22. Ruiz-Tovar J, Diez-Tabernilla M, Housari G, Martinez-Molina E, Sanjuanbenito A. Gastrointestinal stromal tumor: actin expression, a new prognostic factors? Am Surg. 2010:76:1244-9.