Case Study TUMOR STROMAL JEJUNAL MAY BE COMPLICATED BY SHOCK HEMORRHAGIC?ABOUT A CASE

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1 Case Study TUMOR STROMAL JEJUNAL MAY BE COMPLICATED BY SHOCK HEMORRHAGIC?ABOUT A CASE ABSTRACT. Stromal tumors of the small bowel are mesenchymal tumors of uncertain prognosis, developed in the wall of the digestive tract. They are usually asymptomatic incidentally discovered during endoscopy or during surgery. identifying gastrointestinal stromal tumor is facilitated by a relatively specific marker, c-kit. diagnosis is confirmed by histological examination of the surgical specimen. They pose two problems to confirm the diagnosis and assess their evolutionary potential to adapt the therapeutic management. We report the case of a patient of 34 years with no pathological history, admitted there was a year to emergencies in an array of hemorrhagic shock with moelena abundance evolving for three days. The patient underwent a formal condition (remlissage and transfusions) and an endoscopic assessment (UGIE AND colonoscopy) that did not reveal any abnormalities Surgical exploration objectified tumor at 1 meter from the ligament of Treitz, the rest of the exploration was unremarkable. Resection taking process with Grelo small bowel and anastomosis was performed. Postoperative suites were simples.study pathology of the surgical specimen confirmed that this is a stromal tumor with low potential of malignité.le staging did not reveal any secondary lesions and the patient was sent to oncology serve where she received the IMATINIB. stromal tumors are asymptomatic long. Low digestive bleeding is an exceptional way of revelation. complete resection of the tumor is the treatment of choice.the recent development of targeted therapies molecular biology is a new hope in the treatment of these tumors. the interest of this observation is to review the diagnostic and therapeutic aspects of this disease Keywords: hail, lower GI bleeding, c-kit, imatinib

2 INTRODUCTION gastrointestinal stromal tumors are mesenchymal tumors that developed on conjonctifs tissus of the wall of the digestive tract organs. they account for 85% of the mesenchymal tumors of the gastrointestinal tract. these rare tumors often are localized in the stomach (70%) and small intestine (20-30%) their origin derived from cajal cells forming a network interposed between the muscular and nervous plexus of the digestive tract involved in the regulation of cell motility. stromal tumors may be asymptomatic, incidentally discovered during a morphological examination or surgery, they can be revealed by hemorrhage presumption endoscopic diagnosis is usually confirmed by the typical echoendoscopic aspect. the diagnosis is confirmed by histological study. these tumors have a known malignancy potential, and their prognosis is poor when the tumor size is important and the mitotic index is high 2. CASE we report the case of a patient of 34 years with no pathological individuals history, admitted there was a year to emergencies in an array of hemorrhagic shock with high abundance moelena evolving for three days before his admission. clinical examination revealed a patient obnubilated in poor general condition,generalized cutaneous pallor mucosa, pulse thready, respiratory rate 40 cycles / min, blood pressure impregnable, the rest of the physical examination was unremarkable. the patient underwent a formal requirement (filling and transfusions) and endoscopic assessment ( echoendoscopic & colonoscopy) did not reveal any abnormalities. given the persistence of moelena a artérigraphie was indicated but could not be made due to its unavailability in the hospital, so the insatable condition of the patient does not allow its movement. surgical exploration was decided seen the evolution of the patient to hemodynamic instability. this exploration revealed a tumor process 3 cm long axis at 1 meter depends on the angle of treitz may be associated with a stromal tumor, the rest of the exploration was unremarkable. resection carrying the process with small bowel anastomosis grelo small bowel. (figure 1) post-operative suites were marked by the cessation of moelena with favorable evolution of the patient. the anapth of the surgical specimen showed that it is a stromal tumor expressing cd117 gene immunohistochemistry, low malignant potential with limits resection R0.le staging did not reveal any lesions secondary and the patient was referred to the oncology department where she received imatinib

3 figure 1 : jejunal tumor located one meter from the first jejunal loop 3. DISCUSSION gastrointestinal stromal tumors are solid tumors,although limited, developed in the thickness of the wall of the digestive tract the age of onset of these tumors is between 50 and 70 years, with a male predominance. type 1 neurofibromatosis and triad carney are predisposing factors. we must emphasize the existence of familial forms (1) they can grow on the entire digestive tract with a predominance of gastric and small bowel locations, other locations can be extradigestive but very rare way: greater omentum, mesentery, retroperitoneum.stromal digestive tumors(sdt) most often interesting the jejunum, ileum and duodenum. the presentation of gastrointestinal stromal tumors varies according to their origin and their aggressiveness site. thus, the sdt may be asymptomatic and discovered incidentally or manifest as gastrointestinal bleeding or abdominal pain, or more rarely by occlusive syndromes or perforation of the gastrointestinal tract (2-4). the anemia associated with occult bleeding can be a call sign ( 5). a biological inflammatory syndrome, cytolysis and cholestasis or if secondary liver damage, mild leukocytosis in cases of infectious complication tumor can be observed. imaging has an important place in the management of sdt. despite their various aspects, these tumors have a very evocative presentation that allows most often adopt appropriate care, and in particular to avoid transmural biopsy source peritoneal swarming. allows imagery after treatment to evaluate the therapeutic efficacy and detect relapse (6). abdominal ultrasound can highlight a sdt if it is large endoscopic ultrasonography can be used to determine the local extension in the case of esophageal tumors, gastric and rectal. in the tdm sdt appear as exoluminales masses of sharp edges and variable size. their density is variable and tissue homogeneity. aggressive sdt is suggested by a large, irregular contours, invasion of adjacent organs ains a densification of the adjacent fat (7)

4 64 MRI allows the study of mesenteric extension and research of liver metastases positron emission tomography (5) is a very sensitive test for the assessment of tumor metabolism and therapeutic response to imatinib, and the initial staging (7,8). endoscopy is useful for diagnosing colic or gastric stromal tumors, and often allows the diagnosis in case of appearance of intraluminal growth. the capsule video, entero mri, ct enteroclysis, double-balloon enteroscopy are the most sensitive tests to visualize stromal tumors hail (9). the diagnosis is based on natomopathologique study. macroscopically stromal tumors develop from the muscularis of the gastrointestinal tract. they are usually very limited, consisting of a fabric, fibrous, sometimes surrounded by a pseudo capsule. they are often associated with ulceration of the mucosa may explain their mode of presentation in the form of gastrointestinal bleeding. they may have an endophytic growth towards the light, exophytic, or mixed. their size is variable from a few millimeters to more than 30 cm ( 3.7 ). microscopically the tumor consists of a proliferation of spindle cells, epithelioid rarely strongly positive immunohistochemistry for c -kit ( cd117 ). c-kit is responsible for receptor tyrosine kinases (kit or cd117 ) that is widely involved in étiogenèse gist gene. for mutations in the target genes is only necessary to tumor histology but negative evocative kit. (2, 10) the malignant potential is difficult to assess for sdt, even for small tumors less than 2 cm in size, several prognostic factors have been reported :(5) the location, gastric stromal tumors have a better prognosis tumor size: tumors larger than 5 cm are usually malignant the intratumoral necrosis the size of the tumor cells, the number of mitoses, aneuploidy and cell proliferation index with a threshold of 2 to 3mitoses 10 high power fields (x 400). in practice, below mitosis per 10 fields, tumors are considered benign, more than 4 mitoses per 10 fields these tumors are considered malignant existence of metastases at diagnosis. nevertheless, there are many tumors "border line" and any stromal tumor should be considered to have malignant potential even very low the treatment of stromal tumors based on complete surgical resection remains the only potentially curative treatment. there is consensus on the optimal margins of resection. a margin of 1 to 2 cm is desirable and lymphadenectomy is not as systematic,because lymph node metastases are rare (less than 10%) and the risk of lymph node recurrence is limited (less than 5%) (11). the surgical procedure depends known tumor site. for small bowel tumor resection more or less extensive is made with immediate restoration of continuity.

5 imatinib is a selective inhibitor of tyrosine protein kinases, especially c- kit. it is indicated in stromal tumors locally advanced or metastatic earnings as adjuvant or neo adjuvant to surgery is being evaluated (12, 13). the evolution of aggressive stromal tumor is not modified by radiation and by the standard chemotherapy which is not confirmed. the recent development of specific and potent inhibitors of tyrosine kinases (st1570, su11248) is a good alternative. 4. CONCLUSION Gastrointestinal stromal tumors are the most common mesenchymal tumors of the digestive tract.they are long asymptomatic and most often manifested by a complication. Jejunal localization is rare.the lower GI bleeding is a way of exceptional revelation of these tumors, given their exoluminal development. With the current immunohistochemical means, their diagnosis has become relatively easy. Their prognostic evaluation is not easy against. Complete resection of the tumor is the treatment of choice. The recent development of targeted therapies molecular biology is a new hope in the treatment of these tumors COMPETING INTERESTS Authors have declared that no competing interests exist. CONSENT All authors declare that written informed consent was obtained from the patient for publication of this case report and accompanying images ETHICAL APPROVAL All authors declare that this manuscript have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

6 REFERENCES 1.Gupta P,Pewari M,Shukla HS.gastrointestinal stromal tumor,surg oncol 2008 ;17 : Balaton J, Coindre JM, Cvitkovic F. tumeurs stromales digestives. gastroentérol clin biol 2001 ; 25 : Miettinen M,Lasota J. gastrointestinal stromal tumors - definition, clinical, histological,immunohistochemicaland molecular genetic features and differential diagnosis. virchows 4.Tateishi U,Hasegawa TI, Satak M,Moriyama N gastrointestinal stromal tumor: correlation of computed tomography findings with tumor grade and mortality. journal of computer assisted tomography. 2003, vol. 27, no5, pp Lapalus M-G, Hervieu V, Crombe A, Scoazec J-Y, Valette P-J,Stremsdoerfer N, Ponrt G.tumeur stromale de l'intestin grêle : une approche diagnostique multitechnique par vidéo-capsule et entéroscanner. gastroentérologie clinique et biologique 2005, vol. 29, no11, pp arch 2001 ; 438 : 1-12térol clin biol 2001 ; 25 : (janvier 2008)a consensus approach. human pathol 2002 ; 33 : Bensimhon D et al. tumeurs stromales digestives : rôle de la tomodensitométrie avant et après traitement ; gastroentérologie clinique et biologique 2008 volume 32, n 1 7.Burkill. malignant gastrointestinal stromal tumor: distribution, imaging features, and pattern of metastatic spread.gj, badran m, al-muderis o, meirion thomas j, judson ir, fisher c, moskovic ec. radiology feb;226(2): Kumaresan Sandrasegaran; Arumugam Rajesh; Daniel A. gastrointestinal stromal tumors ;clinical,radiologic and pathologic features ;ajr ;184.march Cobrin GM, Pittman RH, Lewis BS. increased diagnostic yield of small bowel tumors with capsule endoscopy. cancer jul 1;107(1): Fletcher CDM, Berman JJ, Gorstein F, Longley BJ, Oleary T, Rubin BP et al. diagnosis of gastrointestinal stromal tumors : 11.Dematteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. ann surg 2000 ; 231 : Demetri GD. targeting c-kit mutations in solid tumors: scientific rationale and novel therapeutic options. semin oncol oct;28(5 suppl 17): Savage DG, Antman KH. imatinib mesylate--a new oral targeted therapy. n engl j med feb 28;346(9):

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