Back With a Vengeance… a Case of GIST Recurrence. - Hospital ...

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Back With a Vengeance… a Case of GIST Recurrence. - Hospital ...

Back With a Vengeance… a Case of GIST Recurrence.1 1 2 3 4GONZALEZ-IBARRA F , MUNSON E , HEKMATJOU H , ONWUZULIGBO D , RODRÍGUEZ-CASTRO CEfrom just blood tinged stools to almost a cupful of bloodABSTRACTcovering the entire bowl. Two days prior to admission, patientGastrointestinal stromal tumors (GIST) are a rare group of consulted at the Emergency Department (ED) discharged withlesions, but represent the most common type of stromal or diagnosis of hemorrhoids. Upon discharge however, themesenchymal tumors affecting the gastrointestinal tract. We patient experienced an increase in both the frequency (5-6presented a case of a 44 year old male with a recurrence of episodes per day) and amount of blood, prompting a revisit toGIST and widespread intra abdominal metastasis despite the ED. Associated symptoms included abdominal pain (rightprevious resection with adjuvant radiation and chemotherapy. lower quadrant, intermittent, cramping, nonradiating), poorKey words: Gastrointestinal stromal tumors, recurrence. appetite, weight loss of 10-15 lbs over the prior 2 weeks.INTRODUCTIONPatients past medical and surgical history were remarkable forGastrointestinal Stromal Tumor (GIST) of the small bowel,The term “gastrointestinal stromal tumor” was coined in the treated surgically via laparotomy without adjuvantearly 1980s to emphasize their uncertain lineage, although they chemotherapy or radiation therapy. The patient's social historyhad been considered smooth-muscle tumors by most observers included occasional alcohol use. The patient claimed a balancedup to that time. They are mesenchymal tumors of the diet. Patient's physical examination was remarkable for rightgastrointestinal (GI) tract often presenting as subepithelial lower quadrant tenderness with no organomegaly and noneoplasms in the stomach (often in the fundus) and proximal costovertebral angle tenderness, but otherwise benign.small intestine, but can occur in any portion of the Computed Tomography (CT) revealed multiple loculatedgastrointestinal tract. The yearly incidence of GIST has been hepatic masses. A right hepatic lobe mass with a maximalestimated to be between 4000-6000 new cases yearly. dimension of 7.4 x 7.1 cm. This mass was complex withGastrointestinal stromal tumors peak in the fifth and sixth multiple internal septations and a thick enhancing wall. Five1decades and have a male-to-female ratio of about 2:1.additional complex cystic lesions were also noted in the liver.The molecular background of these lesions is an interesting The next largest mass found measured 5.8 x 4.4 cm and wasfield of research, the therapeutic options are multiples, but still abutting the gallbladder. An 11.5 x 11.1 cm necrotic pelvic massevolving, and the outcome and prognosis of these tumors are with a 2.3-cm wide fistula to the adjacent sigmoid colon was alsovariable and unpredictable. It is described that nearly 50% of noted (Figures 1 and 2).these tumors develop metastasis, and around 7% had isolated Initial laboratory results showed a white blood cell count oflocal recurrence. Recurrence of disease after resection is a 21.3, hemoglobin of 12.9. Patient was maintained on Cefepimeproblem and it is usually intraabdominal and involves the 1g IV and Flagyl 500mg IV. Interventional Radiology guided2peritoneum and liver. We describe a case of a patient with an drainage of the hepatic and pelvic collections were done on theaggressive GIST that presented with recurrence and 2nd hospital day. Esophagogastroduodenoscopy showedintraabdominal metastasis despite resection, radiation and normal esophagus, mild non erosive gastritis and a normalchemotherapy. duodenum, firm ulcerated tumor in the sigmoid colon, at least 6CASE DESCRIPTIONcm, internal and external hemorrhoids. Biopsy of the gastricantrum showed gastric mucosa with mild chronic inflammationA 44 year old African American male presents with bright red and without the presence of H. pylori. Biopsy of ulcers at theblood per rectum beginning 4 days prior to admission with sigmoid colon revealed marked acute and chronic inflammationintermittent episodes. When it happens the patient would have and hyperplasic glandular changes. Tumor markers were all2-3 bowel movements in an hour. Blood was bright red in color normal.and admixed with stool, with varying amount of blood ranging Liver biopsy was performed for pathologic evaluation1Mount Sinai School of Medicine. Jersey City Medical Center. Department of Internal Medicine. New Jersey, USA. 2St.George's School of Medicine. Third year medical student. Grenada, W. I. 3University of Ibadan School of Medicine,Nigeria.4Texas Tech University, Health Science Center, El Paso, Texas, USA.Enviar correspondencia, observaciones y sugerencias a Fernando Pavel Gonzalez Ibarra MD. Mount Sinai School ofMedicine, Internal Medicine department, Jersey City Medical Center. 355 Grand street, Jersey City, NJ 07302, USA. E-mail:drpavelglez@gmail.com.Artículo recibido el 18 de septiembre de 2012Artículo aceptado para publicación el 19 de septiembre de 2012Este artículo podrá ser consultado en Imbiomed, Latindex, Periódica y en www.hgculiacan.comSociedad Médica del Hospital General de Culiacán “Dr. Bernardo J. Gastélum”Arch Salud Sin Vol.6 No.3 p.81-83, 201281


GONZALEZ ET ALBack With a Vengeance… a Case of GIST Recurrence.of the hepatic mass and returned a final diagnosis of metastatic typically defined by the expression of c-KIT (CD117) in thegastrointestinal stomach tumor (GIST), positive for CD117, tumor cells, as these activating KIT mutations are seen in 85-3CD34 and Dog1 by immunohistochemical staining.95% of GISTs.Originally GISTs were described histopathologically asderivatives of smooth muscles. They have been described ashaving expression of CD117 antigen, a part of the cKITtransmembrane oncogene. Mutation of the CD117 in GISTrepresents a constitutive activating mutation in thetransmembrane receptor tyrosine kinase product of cKIT, thusleading to abnormally activated oncogenic signaling in cells.Molecular mechanisms of this pathway are defined by cKITgene mutations at exon 11; however mutations in exons 9, 13,and 17 have also been documented. These mutations result inspontaneous ligand dimerization and receptor activationeffectively producing a gain of function mutation and tyrosinekinase activity. In a smaller percentage, GISTs are defined bymutations in the platelet derived growth factor receptor alpha(PDGFRA) without the expression of cKIT mutations.Histologically GISTs are characterized as 3 forms: Spindle celltype (70%), Epitheloid type (20%) and Mixed type (10%). OfFigures 1 and 2. Computed Tomography showing a complex righthepatic lobe mass with multiple internal septations and a thickenhancing wall with a maximal dimension of 7.4 x 7.1 cm, fiveadditional complex cystic lesions also noted in the liver (left). The nextlargest mass measured 5.8 x 4.4 cm and was abutting the gallbladder,along with an 11.5 x 11.1 cm necrotic pelvic mass with a 2.3-cm widefistula to the adjacent sigmoid colon (right).DISCUSSIONThese tumors are often associated with nonspecific symptoms,but may present with overt GI bleeding, abdominal mass,abdominal pain and are notorious for hematogenous metastasiscommonly to the liver and less commonly the lungs. They arethought to develop from the interstitial cells of Cajal,innervated cells associated with the Auerbach plexus. GISTs arethese the Epitheloid type is most often characterized as cKIT4negative and PDGFR positive.Clinically GISTs may present as asymptomatic or arediscovered incidentally during barium studies, or on CT scan.Contrast enhanced CT is superior to MRI for the initialscreening and staging test of choice. It aids in thecharacterization of the abdominal mass to evaluate its extent.They are normally seen as solid masses with smooth contours5and they enhance brightly with IV contrast.Treatment for GISTs is based on the assessment of tumorsize, mitotic rate, and location. These factors are also use toassess the risk of recurrence of GIST in patients. Tumors


GONZALEZ ET ALBack With a Vengeance… a Case of GIST Recurrence.patients with advanced disease has risen to 75–80% followingimatinib treatment. Recent data from the 2007 ASCO meetinghas shown a significant reduction in the rate of recurrence ofGIST tumors when treated with adjuvant treatment withimatinib following GIST tumor resection. Patients who developresistance to imatinib may respond to the multiple tyrosinekinase inhibitor sunitinib (marketed as Sutent), however theeffectiveness of either of these medications is dependent upon8the patient's genotype.In conclusion, GISTs are connective tissue tumors of thegastrointestinal tract that have assumed clinical importanceReferencesdespite their rare occurrence. This is because of the importantinsights provided into the disease biology. They are alsonotorious for intra abdominal recurrence. Currently, computedtomography scan is the preferred initial imaging study forscreening while surgical resection is the mainstay of treatment.It is however imperative that investigational protocols be put inplace post operatively in order to reduce the rate of recurrenceand to improve patient outcomes. Moreover, more effectiveadjuvant therapy still needs to become available for there to be asignificant reduction in recurrence and better treatmentoutcomes.1. Paral J, Slaninka I, Kalabova H, Hadzi-Nikolov D. Gastrointestinal stromal tumors: review on morphology, molecular pathology, diagnostics,prognosis and treatment options. Acta Gastroenterol Belg. 2010; 73(3): 349-59.2. DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns andprognostic factors for survival. Ann Surg. 2000; 231(1):51-8.3. Corless CL, Barnett CM, Heinrich MC. Gastrointestinal stromal tumours: origin and molecular oncology. Nat Rev Cancer. 2011;11(17): 865-78.4. Maleddu A, Pantaleo MA, Nannini M, Biasco G. The role of mutational analysis of KIT and PDGFRA in gastrointestinal stromal tumors in a clinicalsetting. J Transl Med. 2011; 9:75.5. Grover S, Ashley SW, Raut CP. Small intestine gastrointestinal stromal tumors. Curr Opin Gastroenterol. 2012; 28(2):113-23.6. Ganjoo KN, Patel S. Current and emerging pharmacological treatments for gastrointestinal stromal tumour. Drugs. 2011;71(3):321-30.7. Grignol VP, Termuhlen PM. Gastrointestinal stromal tumor surgery and adjuvant therapy. Surg Clin North Am. 2011; 91(5):1079-87.8. Joensuu H, Vehtari A, Riihimäki J, Nishida T, Steigen SE, Brabec P, et al. Risk of recurrence of gastrointestinal stromal tumour after surgery: ananalysis of pooled population-based cohorts. Lancet Oncol. 2012; 13(3):265-74.Conflict of Interests: The authors specified that the research was conducted in the absence of any related conflict of interest.Funding: No funding. The authors specified that the research was conducted in the absence of any study sponsor.Artículo disponible en www.imbiomed.comArch Salud Sin Vol.6 No.3 p.81-83, 201283

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