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The Journal of Emergency Medicine, Vol. 40, No. 5, pp. e93–e95, 2011Copyright © 2011 Elsevier Inc.

Printed in the USA. All rights reserved0736-4679/$–see front matter

doi:10.1016/j.jemermed.2007.11.111

ClinicalCommunications: Adults

UNUSUAL ETIOLOGY OF EPIGASTRIC PAIN

Rita Slim, MD,* Tarek Smayra, MD,† Cyril Tohme, MD,‡ Elia Samaha, MD,* Cesar Yaghi, MD,*and Raymond Sayegh, MD*

*Department of Gastroenterology, †Department of Radiology, and ‡Department of General Surgery, Hotel-Dieu de France Hospital,Beirut, Lebanon

Reprint Address: Rita Slim, MD, Department of Gastroenterology, Hotel-Dieu de France Hospital, Beirut, Lebanon

e Abstract—Background: Epigastric pain is a commonpresenting complaint encountered in urgent care settings.Although peptic, biliary, and pancreatic pathologies are themost frequent findings, other rare diagnoses also can befound. Objectives: We report an unusual case of acuteepigastric pain in which abdominal ultrasound was of greatsupport in revealing the diagnosis. Case Report: A 64-year-old man presented to the Emergency Department afterrapid onset of acute epigastric pain. Abdominal ultrasoundshowed a multi-cystic heterogeneous mass between thestomach and the liver. Abdominal computed tomographyscan confirmed the gastric origin of the mass and showedtorsion signs. Urgent laparotomy was performed with tu-mor excision. The diagnosis of pedunculated exophytic gastricstromal tumor was made and long-term follow-up was ar-ranged. Conclusions: Acute presentation revealed the pres-ence of the tumor, which was excised surgically. © 2011Elsevier Inc.

e Keywords—epigastric pain; torsion; GIST

INTRODUCTION

Gastrointestinal stromal tumors (GIST) were describedin 1983 as a mesenchymal neoplasm closely related tothe interstitial cells of Cajal (1). Diagnosis of GIST relieson standard histological examination. In a typical case,the diagnosis is definitively established when CD34 andc-Kit are positive while alpha-smooth muscle actin andS-100 are negative (2).

RECEIVED: 30 September 2007; FINAL SUBMISSION RECEIVE

CCEPTED: 16 November 2007

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Cystic stromal degeneration and stromal hemorrhagerepresent prominent features of GIST with spindle celltype. We describe a case of acute epigastric pain due tothe pedicle torsion of an exophytic gastric stromal tumor,asymptomatic before this acute presentation.

CASE REPORT

A 64-year-old man with an unremarkable past medicalhistory presented with acute abdominal pain. Sixteenhours before admission, he reported an acute epigastricpain of rapidly increasing intensity unrelated to meals,with no referred pain. There were neither changes inbowel habits nor signs of gastrointestinal hemorrhage.His clinical examination revealed stable hemodynamicswith a temperature of 36.9°C (98.4°F) axillary, pulse 70beats/min, blood pressure 130/80 mm Hg, and respira-tory rate 18 breaths/min. The heart and lungs were nor-mal on auscultation, and abdominal examination showedlocalized epigastric tenderness with a negative Murphysign. Routine laboratory data were normal, includingblood count and cardiac and liver enzymes. Electrocar-diogram and chest radiograph were also normal.

Abdominal sonography showed a 6-cm heterogeneousmulti-cystic abdominal mass located between the stom-ach and the left lobe of the liver (Figure 1). Abdominalcomputed tomography (CT) scan revealed a solid massattached to the anterior wall of the stomach with torsionsigns (Figure 2).

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On laparotomy, the tumor appeared polylobated witha pseudocapsule, attached to the anterior gastric wall bya thin pedicle. There was a peduncular torsion with signsof intra-tumoral swelling and hemorrhage. Excision ofthe tumor and of the gastric wall tissue where the tumorwas attached to the pedicle was performed.

Macroscopic examination revealed a 6-cm tumor withan area of liquefied necrosis and intra-tumoral hemor-rhage (Figure 3). Microscopic examination revealed ar-

Figure 2. Abdominal computed tomography scan revealing asolid mass attached to the anterior wall of the stomach with

Figure 1. Abdominal ultasonography showing a heteroge-neous multi-cystic abdominal mass located between thestomach and the left lobe of the liver.

torsion signs.

eas of necrosis and hemorrhage, and the rest of the tumorwas composed of spindle cells with a weak mitotic index.Immunohistochemistry showed marked positivity forCD34 and CD117 (c-Kit).

The final diagnosis was that of a gastric exophyticpedunculated stromal tumor with pedicle torsion.

CONCLUSION

There are many causes of acute epigastric pain. This caseis presented to increase awareness among emergencyphysicians that pedicle torsion of a GIST can causesudden onset of abdominal pain who present to the ED.

DISCUSSION

GISTs, defined as immunohistochemically CD117-positiveand c-Kit signaling-driven primary mesenchymal tumorsof the gastrointestinal (GI) tract, comprise the great ma-jority of primary mesenchymal tumors of the digestivesystem (1). In a population-based study, approximately70% of patients with GIST were symptomatic, 20% wereasymptomatic, and 10% of the tumors were detected atautopsy. Symptoms caused by GISTs are commonly dueto their location, with both mass effect and intraluminalbleeding being reported. Large GISTs can cause vagueabdominal discomfort, pain, bloating, early satiety, orincreased abdominal girth. Erosion into the GI tract caninduce significant hemorrhage, causing hematemesis,melena, or anemia from occult bleeding. Other rare pre-sentations described in the literature include hypoglyce-mia, abdominal pain due to torsion of an exophytictumor, presentation as content in a hernial sac, intraperi-

Figure 3. Macroscopic appearance of the tumor.

toneal bleed, and mimicking acute appendicitis (3).

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Unusual Etiology of Epigastric Pain e95

GIST occurs most frequently in the stomach, andgastric stromal tumors account for approximately 3.6%of all gastric tumors. The ratio of male to female cases is3:1, and the average age of patients presenting with thetumor is 40–60 years. They originate from gastric an-trum and body in 70–90% of cases. They grow in anexophytic pattern in 30–40%, intraluminal pattern in29–44%, endoluminal pattern in 18–22%, and a mixedpattern in 16–20% of all cases (4).

Currently available imaging techniques to evaluateGIST include endoscopic ultrasound, CT, magnetic res-onance imaging, and fluorine-18-fluorodeoxyglucosepositron emission tomography. Owing to the frequentexophytic growth of these lesions, differentiation ofthese tumors from non-digestive lesions of a differentnature is a common diagnostic problem. Imaging find-ings usually allow differentiation from gastrointestinalepithelial tumors but not from non-epithelial tumors, forwhich histological confirmation is necessary. CT is con-sidered the most useful imaging tool in these tumors,more useful than endoscopy or barium studies to evalu-ate their real size and local extension because it canassess endo- and exophytic components of the tumorregardless of its size (5). Before surgery of a primaryGIST, abdominal CT study excludes liver or peritonealmetastasis and evaluates the extension of the primarylesion.

GIST pedunculated externally from the stomach, as inour patient, is very rare, and only one case of acuteclinical presentation due to peduncle torsion and devel-opment of hemoperitoneum has been reported previouslyin the literature (6). Diagnosis was not suspected until theabdominal ultrasound showed the multi-cystic mass. Ab-dominal CT scan confirmed the gastric origin of thetumor and excluded metastasis.

Standard treatment of resectable GIST is surgery. Thegoal of surgery is complete resection of visible andmicroscopic disease, possibly avoiding the occurrence oftumor rupture and achieving negative margins. Laparo-scopic surgery should be avoided, owing to the higherrisk of tumor rupture and subsequent peritoneal seeding.Wedge resection of the stomach is considered an ade-quate treatment in case of gastric stromal tumor (2,7). Areoperative pathological diagnosis for intramural le-ions of the gastrointestinal tube is indicated providing itoes not induce tumor spillage. Gu et al. evaluated thefficacy and accuracy of endoscopic ultrasound (EUS)-uided fine needle aspiration (FNA) biopsy in the diag-

osis of GIST and described its cytomorphologic fea-

tures (8). They demonstrated that when combiningsmears and cell blocks, EUS-guided FNA is an accurateand efficient way of diagnosing GIST (8). In our case,although the diagnosis of a GIST tumor was suspectedbut not confirmed, urgent surgery was mandatory due tothe acute presentation.

The prognostic factors of a localized tumor are size �5 cm and mitotic count � 1–5 per 50 high-powered fields5). In this group, the risk of local recurrence or devel-pment of metastasis is estimated to be higher than 50%9). In these cases, experts recommend a radiologic follow-p with a CT scan every 3–4 months for 3 years, thenvery 6 months until 5 years, and yearly thereafter (2).reatment with imatinib after complete resection in pa-

ients who have a substantial risk of relapse is the subjectf ongoing clinical trials and it is not recommendedutside these trials (2). We think that the size of theumor in our patient was overestimated due to tumorongestion from the pedicle torsion. He was not includedn any ongoing clinical trial but he was advised to un-ergo a strict long-term follow-up.

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