Multi-cystic gist of stomach: an unusual presentation as obstructive jaundice.

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1 Multi-cystic gist of stomach: an unusual presentation as obstructive jaundice. Dr. Kirankumar P. Jadhav *, Prof. Dr. Sanjiv S. Thakur**Prof. Dr. Ajay S. Chandanwalle*** Dr. Schin M. Kharat****, Dr. Chhaya E. Ahire***** * Assistant Prof., Surgery Dept., B.J.Gov. Medical college, Pune (India) ** Prof & Head of Surgery Dept., B.J.Gov. Medical college, Pune (India) *** Dean, B.J.Gov. Medical college, Pune (India) **** MBBS, Medical Officer, Health services, Maharashtra (India) ***** MD Physiology, B.J.Gov. Medical college, Pune (India) Correspondence:Dr. Kirankumar P. Jadhav, Mail ID: drkpjadhav@gmail.com.. Abstract: A 42 yr old male presented with pain in upper abdominal fullness and pain and jaundice.. Upper GI endoscopy suggestive of extraluminal compressing mass at antrum. On laparotomy there was multiple cystic swelling along the lesser curvature largest cyst 6 x 4 cms. There was three sub-mucosal swelling near antrum causing narrowing. The patient was histo-pathologically reported as gastrointestinal stomal tumor(gist), which is present 0.1% of all colorectal cancer. GIST is mostly identified incidentally during endoscopic screening in the absence of symptom. GIST in comparison with leiomyoma and leiomyosarcoma is on the basis clinic-pathologic, immunohistochemical and molecular genetic study. Mostly malignant GIST run a very slow course and have recurrence and metastasis over years. Thus long term follow-up is necessary. Unless proved other-wise gastric and colonic lesions are considered as malignant. Key words: Gastrointestinal stomal tumor, gastric malignancy, CD117 antigen, Cajal cell tumor, S Background: The gastrointestinal stomal tumors (GIST) may arise any-where in the tubular gastrointestinal tract, from esophagus to the rectum. In-addition, it has been appreciated in extra-gi locations, principally mesentery, omentum and retro-peritoneum. In terms of detailed distribution, 5% in the esophagus 1, 50-60% in the stomach, 20-30% in the small intestine 2, 10% in the large bowel, 3,4 5% else-where in the abdominal cavity (gallbladder, bladder, mesentery, omentum and retro-peritonium). 5,6,7,8 Case Report: A 42 yr old male presented with pain in upper abdominal fullness and pain And jaundice. Patient was investigated for that, had normal hematological reports except raised serum bilirubin 6 IU whisch was suggestive of obstructive jaundice. Upper GI-scopy shows narrowing at antrum of stomach with normal mucosa. CT abdomen suggestive of cystic swelling6 x 4 cm size between head of pancreas and lesser curvature of stomach. Large cyst compressing over lower common bile duct and partial narrowing at antrum. 81 On laparotomy, there was multiple cystic swelling along the lesser curvature largest cyst 6 x 4 cms. There was three sub-mucosal swelling near antrum causing narrowing. Distal gastrectomy with Bilroth II procedure was done. There was no rupture of any cyst during surgery. The cysts contained clear fluid. On HPR the cyst was positive for low grade Malignant GIST. Immunohistochemistry showed c-kit-positive CD34-

2 positive smooth muscle actin (SMA)-negative, and S-100-negative staining of tumor cells. The final unclear. 13 But prognostic relevance of cell type is seems limited. CT-abdomen is used to define correctly the site, diagnosis was gastrointestinal stromal tumor size and structure of lesions in all cases and to identify (GIST). Postoperative period was uneventful and patient discharged on 8 th day. Since last 8 months patient is on Imatinb. After Six month CT not show any evidence of recurrence. signs of invasion of neighboring structures in some cases. 14 On immune-histochemisry, in contrast to true smooth muscle tumors, GISTs are usually positive for expression of the KIT receptor tyrosine kinas (detected as CD117 antigen) and CD34, variably positive for smooth Discussion: The GIST is present 0.1% of all cancer. muscle actin and usually negative for desmin. Unlike Sixty five % of cases are identified incidentally Shwanomas GISTs are usually negative for S100 protein. during endoscopic screening in the absence of The prevalence of c-kit mutation in GIST is as high as symptom. Most of the tumors are silent until it 90%. 15 Imatinb (STI-571) selectively inhibits the ABL, reaches a large size, at which point it may cause BRC-ABL, KIT and PDGF receptor tyrosine kinas. The non-specific abdominal pain, discomfort or become Imatinb is a targeted therapy directed against the apparent recognized as a palpable lump. The diagnosis of fundamental and pathogenic defect in the GIST. GIST is often suspected histological & imunohistochemestry, because the majority of cases have Adjuvant therapy with STI-571 will improve outcome if applied earlier in course of GIST. 15, Radiotherapy (5040 remarkably uniform appearance. The cystic GIST cgy) has limited role, in positive microscopic margins in have a benign to malignant ratio of 1:2. 4,9 Unless gastric and rectal GIST. 16,17 proved otherwise gastric and colonic lesions are considered as malignant. 10,11 Mucosal invasion is The primary goal of surgery is complete resection very rare and it can be diagnosed as extra-luminal compression on endoscopy. Invasion between smooth muscle fibers and bundles is a characteristic growth pattern. This may be related to the GISTs origin from Cajal cells or related stem cells, the non smooth muscle elements between the layers of the of the disease with avoidance of tumor rupture. Unlike intestinal adeno-carcinoma GIST rarely metastases to lymp-nodes, so lymphadenectomy is seldom warranted. The disease specific survival rate of asymptomatic GIST patient is 81.7% at 5 years. The patient with primary tumor without metastasis underwent complete gross muscularis propria. 12 The low cellularity has resection having 54% 5 year disease specific survival emerged as a favorable prognostic feature. The mitotic activity >5/50 HPFs or the tumor >5 cm size have a high risk for intra abdominal or hepatic rate, but if the tumor of size >10 cm, it will only 20%. 16 Prognosis of the GISTs depends on anatomic sites, tumor size, mitotic rate and patient age and tumor rupture metastasis. Nuclear atypia is generally not during surgery. 11,16 Most malignant GISTs run a slow prominent in GISTs, and its significance is course, with recurrence and metastasis developing over 82

3 years, give rise to peritoneal cavity or hepatic metastasis, Sometimes it may be 10 to 15 yrs after primary surgery. Thus long term followup is necessary. Photograph I: CT abdomen with contrast. Photograph II: Cystic lump between stomach & Pancreas. Photograph III: Clinical specimen of distal gastrectomy with multiple cysts on lesser curvature. References: 1. Miettinem M, Sarlomo-Rikala M.SobinLH, et al: Esophagial stromal tumors, A clinicpathologic, immuno-histrochemical and molecular genetic study of 17 cases and comparison with esophageal leiomyoma and leiomyosarcoma. Am. Jr Surg Pathol, 24; ,

4 2. Christopher D. M. Fletcher, Jules J. Berman, Christopher Corless, et al.: Diagnosis of gastrointestinal stromal tumors- A consensus approach, published by Elsevier Science, USA. Human pathology, 33 (2); , Tworek JA, Goldblum JR, Weiss SW, et al.: Stromal tumor of the abdominal colon: Clinic-pathologic study of 20 cases. Am. Jr Surg Pathol,23; , Tworek JA, Goldblum JR, Weiss SW, et al.: Stromal tumor of the anorectum: Clinicopathologic study of 22 cases. Am. Jr Surg Pathol, 23; , Ortiz-Hidalgo C, De Leon Bojorge B, Albores-Saavedra J: Stromal tumor of the gallbladder with phyno-type of intestinal cell of Cajal, A previously unrecognized neoplasm. Am. Jr Surg Pathol, 24; , Lasota J, Miettienen M, Carson JA: Stomal tumor of the urinary bladder serosa with phynotype and genotypic feature of GIST. Arch Pathol Lab Med 124; , Miettienen M, Monihan JM, Salromo-Rikala M et al.: GISTs primary in the omentum and mesentery, clinic-pathologic, immuno-hitochemical and molecular genetic study of 26 cases. Am. Jr Surg Pathol, 23; 1109,-1118, Reith JD, Goldblum JR, Lytes RH, et al.: Extra-gastrointestinal stromal tumors: An analysis of 48 cases with emphasis on histological outcome. Mod Pathol 13; , Miettienen M, Salromo-Rikala M, Sobin et al: GISTs and leiomyosarcomas in the colon, A clinic-pathologic, immuno-histrochemical and molecular genetic study of 20 cases. Am. Jr Surg Pathol 24; , Miettienen M, Lasota J: GISTs- Definition, clinical, histological, immuno-hitochemical and molecular genetic feature and differential diagnosis. Virchows Arch 438; 1-12, Emosy TS, Sobin LH, Lukes I, et al.: Prognosis of GISTs, Depends on anatomical site. Am. Jr Surg Pathol 23; 82-87, Miettienen M, Lasota J: GISTs -Defination, clinical, histological, immuno-hitochemical and molecular genetic feature and differential diagnosis. Science 279; , Braid JA, Goldblum JR: Stromal tumors of the jejunum and ileum. A clinical study of t39 cases. Am J Surg Pathol 21; , Rimondini A, Belgrano M, Favretto G, et al: Contribution of CT to treatment planning in patient with GIST. Med. 112(5): ,

5 15. Blanke CD, von Mehren M, Joensuu H, et al.: Evaluation of the molecularly targeted therapy STI-571 in patient with unresectable or metastatic GIST expressing KIT. ASCO Proc 20; 2, Ng EH, Polock RE, Munsell MF, et al.: Prognostic factors influencing survival in gastrointestinal liomyosarcomas. Implication for surgical management and staging. Ann surg. 215; 68-77, Pollock J, Morgan D, Denobile J, et al.: Adjuvant radiotherapy for GIST of the rectum. Dig. Dis. Sci. 46; , Manuscript submission: 18 November 2012 Peer review approval: 07 December 2012 Final Proof approval: 28 December 2012 Date of Publication: 05 January 2013 Source of support: Nil Conflict of Interest: Nil 85