LETTER

Duodenal gastrointestinal stromal tumor presenting as massivegastrointestinal bleed

Mallikarjun Patil & Keyur A. Sheth & C. K. Adarsh &

Suraj Manjunath & Harshad Devarbhavi

Published online: 17 September 2013# Indian Society of Gastroenterology 2013

EditorGastrointestinal stromal tumors (GISTs) are the most commonmesenchymal tumors of the gastrointestinal (GI) tract, derivedfrom the malignant transformation of the interstitial cells ofCajal or their precursors. GISTs are more common in the 50–60 years age group and occur commonly in the stomach (60%to 70 %) and small intestine (25 % to 35 %) [1]. DuodenalGISTs are rare, comprising about 12 % to 18 % of smallintestine GIST and less than 4 % of all GISTs. They usuallypresent with anemia due to chronic GI blood loss, but duode-nal GIST presenting as massive GI bleed with shock is rare.Duodenal GISTs most frequently involve the second portionof the duodenum in close relationship to the ampulla of Vater,followed by the third, fourth, and first portions. The relation-ship to the ampulla determines surgical treatment strategies.

We have treated four patients of GIST presenting withmassive upper GI bleed and shock in the last 18 months. Theirclinical, laboratory, operative, and histopathology details areoutlined in Table 1. All four were between 40–60 years of age.Three out of four presented with hematemesis, and all fourwere critically ill with hemodynamic instability. Laboratoryevaluations revealed severe microcytic hypochromic anemiain all four patients with hemoglobin ranging from 5.6 to 6.8 g/dL. Coagulation parameters were normal.

Gastroduodenoscopy showed submucosal nodule with sur-face ulceration and active ooze in the second part of the

duodenum in three patients and submucosal nodule withactive spurt of blood in the third part of the duodenum inone patient (Fig. 1). Hemostasis was achieved with injectionof the diluted adrenaline. Contrast-enhanced computed to-mography of abdomen confirmed intramural mass in duode-num with distinct fat planes and no lymph node enlargement.All four were operated with limited resection and primaryanastomosis. All patients had small size tumors whichpresented early due to endoenteric growth and had mitosisless than 5 per 50 high-power fields. Their small size,endoenteric growth, complete resection, and lower mitosiswere good prognostic signs in our patients. Immunohisto-chemical analysis revealed a positive CD117 in all four pa-tients, positive CD34 in three patients, and positive smoothmuscle actin (SMA) in one. All are alive with no recurrence,with a mean follow up of 10 months ranging from 5 to18 months.

Massive GI bleeding, as in our patients, is unusual induodenal GIST. In a large study of 156 duodenal GIST pa-tients, 75 cases presented with anemia due to chronic GI loss,but massive GI bleed with hemodynamic instability was onlyoccasional [2]. In the same study, occasional massive GI bleedpresented with melena, but none had hematemesis [2]. Thetypical features of GISTs on duodenoscopy include grossulceration in the mucosa or an intramural mass with centralulceration. The most specific diagnostic criterion is strong anddiffuse positive staining for CD117. GISTs harbor positivityfor vimentin in nearly all cases, CD34 in 50 % to 70 %,smooth muscle actin in 30 % to 40 %, and platelet-derivedgrowth factor receptor alpha (PDGFR-α) in about 5 %, whiledesmin (intermediate filament typical for muscle) and S-100(a neural cell marker) are usually negative [3]. Poor prognosticparameters of GISTs include extragastric location, size greaterthan 5 cm, central necrosis, extension into adjacent organs,

M. Patil (*) :K. A. Sheth :C. K. Adarsh :H. DevarbhaviDepartment of Gastroenterology, St. John’s Medical College,Bangalore 560 034, Indiae-mail: drmalli_arjun@yahoo.co.in

S. ManjunathDepartment of Surgical Oncology, St. John’s Medical College,Bangalore 560 034, India

Indian J Gastroenterol (March–April 2014) 33(2):192–194DOI 10.1007/s12664-013-0384-4

metastases occurring in the liver and peritoneum [4], tumorrupture [5], positive p53 immunoreactivity [6], and alterationof P16INK4A function [6]. Favorable prognostic indicators ofGISTs in the duodenum includes the lower prevalence of p16loss, lower Ki-67 levels, smaller size of the lesion, lowermitotic count, and mutational status [3]. Risk stratification inGISTs is based on tumor size and mitotic activity; size lessthan 5 cm and mitotic count less than 5 per 50 high-powerfields are categorized as low risk [5]. Segmentalduodenectomy is indicated for small (less than 1 cm) on thesecond part when they are more than 2 cm away from theampulla or large (more than 3 cm) tumors located on the thirdor fourth part of the duodenum. Reconstruction in such casesis performed using a side-to-side duodenojejunostomy oppo-site to the ampulla. Pancreaticoduodenectomy is indicated forperiampullary GISTs or large tumors of first or second part ofthe duodenum, which may be inadequately resected through a

Table 1 Clinical, laboratory, operative, and histopathology details of four GIST patients

No. Age Sex Presentation Laboratoryresults

Gastroduodenoscopy Imaging Operative and pathologic data Immunohistochemicaldata

Followup data

Hb PLT INR Tumor site(part ofduodenum)

Size(cm)

Surgical procedure(limited resection)

Mitosis,50 hpf

CD117 CD34 SMA Desmin Ki-67 LI(%)

1 42 Male Hematemesis,melena,shock

6.1 2.17 0.9 Duodenalsubmucosalnodulemeasuring1×1 cm withsummit ulcerand active oozeon the secondpart

Mural mass of 1×1 cm seen atthe junction ofthe first andsecond part ofthe duodenumon lateralaspect

1st–2nd 1×1 Primary closure 2 + + – – <10

2 50 Female Melena,shock

5.6 1.56 0.9 Duodenalsubmucosalnodulemeasuring3×3 cm withsummit ulcerand active oozeon the third partof the duodenum

Hypervascularmural lesionof 3.8×2.7×3 cm seen onthe third partof theduodenumwith nolymph nodesand distinctfat planes

3rd 4×3 Duodenojejunostomy <5 + – – – <5

3 61 Male Hematemesis,melena,shock

5.8 1.4 1.1 Duodenalsubmucosalnodule of 1×2 cm seen at thejunction of thesecond and thirdpart of theduodenum withactive spurt

Mural lesion1×2 cm seenwithoutnecrosis at thejunction of thesecond andthird partof theduodenum.

2nd–3rd 1×2 Duodenojejunostomy <5 + + + – <5

4 60 Female Hematemesis,melena,shock

6.8 1.9 1.0 Duodenalsubmucosalnodule of 1×2 cm withsummit ulcerand activeooze seen onthe medialwall of thesecond part ofthe duodenumaway frompapilla

Mural soft tissuelesion 1×2 cm withoutnecrosis seenon the secondpart of theduodenum

2nd 1×2 Duodenojejunostomy <2 + + – – <2

Fig. 1 Esophagogastroduodenoscopy showing gastrointestinal stromaltumor with active spurt

Indian J Gastroenterol (March–April 2014) 33(2):192–194 193

pancreas-preserving duodenectomy. Patients with completelyresected primary duodenal GISTs have a more favorableprognosis with 1- and 3-year recurrence-free survival ratesof 100 % and 95.2 % [5]. Imatinib mesylate, a tyrosine kinaseinhibitor, has been used as adjuvant or neoadjuvant therapy aswell as in the setting of metastatic and recurrent disease. Wepresent this series of four cases to demonstrate that duodenalGIST may present with massive GI bleeding.

References

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2. Miettinen M, Kopczynski J, Makhlouf HR, et al. Gastrointestinalstromal tumors, intramural leiomyomas, and leiomyosarcomas inthe duodenum: a clinicopathologic, immunohistochemical, and mo-lecular genetic study of 167 cases. Am J Surg Pathol. 2003;27:625–41.

3. Machado NO, Chopra PJ, Al-Haddabi IH, Al-Qadhi H. Largeduodenal gastrointestinal stromal tumor presenting with acutebleeding managed by a whipple resection. A review of surgicaloptions and the prognostic indicators of outcome. JOP. 2011;12:194–9.

4. Ashkzaran H, Coenegrachts K, Steyaert L, et al. Duodenalstromal tumor detected by CTenteroclysis. JBR–BTR. 2006;89:306–7.

5. Ghidirim G, Mishin I, Gagauz I, Vozian M, Cernii A, Cernat M.Duodenal gastrointestinal stromal tumor. Rom J Morphol Embryol.2011;52:1121–5.

6. Yang WL, Yu JR, Wu YJ, et al. Duodenal gastrointestinal stromaltumor: clinical, pathologic, immunohistochemical characteristics, andsurgical prognosis. J Surg Oncol. 2009;100:606–10.

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