Unusual Gastrointestinal Bleeding due to an Aberrant Artery Neighboring a Jejunal Gastrointestinal Stromal Tumor

Abstract

Tumor rupture and/or ulceration of the intestinal mucosa are well-known causes of gastrointestinal stromal tumor (GIST) hemorrhage; however, bleeding from an aberrant artery is exceedingly rare. An elderly female presented with profound anemia (nadir Hgb 4.8 g/dL) and ongoing melena over the past week. Esophagogastoduodenoscopy, colonoscopy, and conventional angiography were negative. Tagged red blood cell scintigraphy localized the bleed to the proximal jejunum prompting diagnostic bedside push enteroscopy. Intraoperative examination revealed diverticular bleeding from a large-caliber aberrant artery arising from a shared arterial trunk supplying a jejunal GIST. Histopathology consistent with low-grade combined type GIST with immunochemistry positive for CD117 (c-KIT). This case highlights an unusual presentation of small bowel bleeding originating from an aberrant artery in continuity with a hypervascular jejunal GIST.

INTRODUCTION

Small bowel bleeding accounts for only ∼10% of all gastrointestinal (GI) hemorrhage but often requires an extensive workup when esophagogastroduodenoscopy and colonoscopy are unrevealing.¹ Even with advancements in device-assisted endoscopy, the small intestine's length and relative inaccessibility often leads to diagnostic delay. Current guidelines recommend diagnostic video capsule endoscopy or device-assisted enteroscopy for suspected small bowel bleeding, except in cases of ongoing hemorrhage, where an emergent computed tomography (CT) angiogram is advised.¹

GI stromal tumors (GISTs) are mesenchymal stromal tumors that make up less than 2% of all GI neoplasms; however, hemorrhage is the most frequent complication of these highly vascular tumors. GISTs arise most frequently in the stomach, followed by the small intestine with approximately 30%–35% of cases.² Within the small intestine, the jejunum has been reported as the most frequent site of GI hemorrhage.²

We describe an unusual case of overt small bowel bleeding in an elderly woman, ultimately found to originate from an aberrant arterial branch in continuity with a jejunal GIST.

CASE REPORT

An 85-year-old woman with dementia and prior Helicobacter pylori eradication presented with ongoing melena for the past week and a syncopal episode. On arrival, she was afebrile, normotensive, and tachycardic; examination revealed a soft, nontender abdomen, and digital rectal examination revealed melena. The patient's initial hemoglobin of 6.5 g/dL continued to decline requiring multiple transfusions of packed red blood cells (PRBCs).

Esophagogastroduodenoscopy revealed erosive gastritis and duodenal erosions without an active bleeding source; antral biopsies were negative for H. pylori. Both colonoscopies showed scattered melena with 1-cm sessile cecal polyp but was negative for active bleed.

The patient clinically deteriorated with worsening melena and required 5 units of PRBCs, 3 units of fresh frozen plasma, and 1 unit of platelets. CT angiography identified an active contrast blush within a jejunal loop and an adjacent enhancing lesion suspicious for a vascular malformation (Figure 1).

Figure 1.

Computed tomography angiography showing active arterial extravasation near an adjacent enhancing mass of the jejunum.

Thereafter, conventional angiography was performed for selective embolization; however, no bleeding was identified. Tagged red blood cell scintigraphy localized bleeding to the proximal jejunum, prompting diagnostic bedside push enteroscopy. A submucosal jejunal mass was identified with an adjacent diverticulum demonstrating stigmata of recent hemorrhage (Figure 2). No additional diverticula were present in the surrounding jejunum; the site was tattooed to guide surgical resection.

Figure 2.

Push enteroscopy (PENTAX EC-3490LI) showing a submucosal jejunal mass and an adjacent diverticulum with stigmata of recent hemorrhage status postclipping.

The patient underwent laparoscopic-assisted small bowel resection with primary anastomosis. Intraoperatively, diverticular bleeding was found to originate from a large tortuous artery arising from a shared arterial trunk in connection with a 4.0 × 2.5 × 2.2-cm exophytic jejunal mass (Figure 3).

Figure 3.

Intraoperative photograph showing an exophytic mass of the jejunum with bleeding from an aberrant artery during laparoscopic-assisted small bowel resection.

Histopathology confirmed a GI stromal tumor (GIST) beneath intact intestinal mucosa with preserved villous architecture (Figure 4). The tumor consisted of a mixed histologic pattern of bland spindle cells arranged in fascicles with scattered epithelioid cells with no evidence of necrosis (Figure 5). The tumor was classified as low grade (G1) with a mitotic rate ≤5 per 5 mm². Immunohistochemistry showed positivity for CD117 (c-KIT) consistent with the diagnosis (Figure 6).

Figure 4.

Histopathology revealed intact jejunal mucosa overlying a submucosal spindle cell neoplasm with intratumoral hemorrhage consistent with a gastrointestinal stromal tumor (hematoxylin and eosin stain, original magnification ×40).

Figure 5.

Mixed histology pattern featuring spindle cells and epithelioid cells characteristic of a combined-type gastrointestinal stromal tumor (hematoxylin and eosin stain, original magnification ×200).

Figure 6.

Immunohistochemical staining showing expression of CD117 (c-KIT) marker in tumor cells.

Based on tumor size and mitotic index, the neoplasm was categorized as low risk, with an estimated malignant potential of 4.3%. At the 6-month follow-up, the patient remains symptom-free and CT scan showed no evidence of recurrent or residual GIST.

DISCUSSION

Small bowel bleeding caused by a GIST is a rare presentation, with an estimated incidence of 10–15 cases per million.³ GIST bleeding often occurs due to tumor rupture or direct erosion of the overlying intestinal mucosa.² However, our patient's bleeding originated from a large-caliber aberrant artery arising from the same arterial trunk that supplied the GIST, which is an uncommon vessel-driven mechanism.

Meta-analysis shows that the risk of bleeding in patients with a GIST is increased with age, small intestine location, tumor diameter ≥5 cm, mitotic index ≥ 5/50 HPF, and tumor rupture.² In our elderly patient, the size of the GIST measured 2–4 cm and had a mitotic index ≤5 per/50 HPF, without evidence of tumor rupture or necrosis.

GISTs are known to be highly vascular stromal tumors due to tumor-associated angiogenesis, with vascular endothelial growth factor signaling being one of the most implicated pathways. Hypertrophied and prominent feeding arteries in GISTs are thought to arise from an “angiogenic switch,” in which a predominance of proangiogenic factors drives vascular remodeling of feeding vessels.⁴ In our patient, a shared arterial trunk gave rise to both the tumor-feeding branches and an adjacent large aberrant artery. Given the relative caliber of the aberrant artery compared with the adjacent feeder vessels, increasing blood flow demands associated with tumor growth through the shared arterial trunk may have contributed to hypertrophy of the adjacent vessel and subsequent hemorrhage.

This aberrant artery-driven bleeding in our patient may resemble that of a caliber-persistent artery (Dieulafoy lesion), where pulsations of a large-caliber vessel erode the overlying mucosa presenting as GI bleeding in the absence of ulceration.³ This mechanism can cause massive and/or intermittent hemorrhage that may elude detection.⁵ This presentation is consistent with our patient's progressive melena and profound anemia (nadir Hb 4.8 g/dL), necessitating a total of 16 units of PRBCs. In addition, the intermittency of the bleeding in our patient may support the absence of active extravasation on selective mesenteric angiography. These findings further support that our patient's acute GI bleeding originated from an aberrant artery neighboring the GIST.

The literature has not shown a well-established relationship between GI stromal tumors and arterial anomalies; however, rare reports have described Dieulafoy-like arterial bleeding in association with GIST as well as GISTs occurring synchronously with arteriovenous malformations.^6–8 This may suggest that the hypervascularity of GISTs may have associated vascular anomalies in rare circumstances. To the best of our knowledge, hemorrhage arising from an aberrant artery in direct continuity with a GIST, as observed in this case, has not been previously reported.

Diagnosis was further challenged by the GIST's location in the jejunum, which is relatively inaccessible to standard endoscopy and often delays identification of small bowel sources.⁹ In our clinically deteriorating and frail patient, bedside push enteroscopy offered a rapid and practical evaluation of the proximal jejunum with reduced anesthetic burden. By contrast, device-assisted enteroscopy (single or double balloon) entails longer procedural times, requires general anesthesia, and necessitates specialized personnel.¹ Targeted push enteroscopy promptly identified the submucosal mass with an adjacent diverticulum and enabled tattooing to guide laparoscopic resection.

Definitive management of localized GIST is complete (R0) resection with preservation of the pseudocapsule; routine lymphadenectomy is not indicated because nodal metastases are rare.¹⁰ For jejunal GISTs measuring 2–5 cm with ≤5 mitoses per 5 mm², the risk category is low.¹¹ Surveillance imaging at 6 months in our patient showed no evidence of residual or recurrent GIST.

While uncommon, jejunal GISTs can present with life-threatening GI hemorrhage and should be considered in the differential for cases of suspected small bowel bleeding. For proximal jejunal lesions, push enteroscopy offers a rapid and effective way to identify and mark the tumor to guide surgery. Management of GISTs includes R0 resection with negative margins to reduce risk of local recurrence.¹²

Our patient's case highlights an unusual presentation of GIST-related bleeding due to a neighboring aberrant artery without evidence of tumor rupture or mucosal ulceration. This suggests that the frequent complication of GI bleeding secondary to a GIST may be less understood than previously appreciated.

DISCLOSURES

Author contributions: K. Desai and A. Magasic: Wrote the manuscript; M. Alkomos: Edited and reviewed the manuscript; W. Baddoura and A. El-Sedfy: Reviewed and gave final approval of manuscript. K. Desai is the article guarantor.

Financial disclosure: None to report.

Informed consent was obtained for this case report.

ABBREVIATIONS:

CD117 (c-KIT)

cluster of differentiation 117 (KIT receptor tyrosine kinase)

CT

computed tomography

EGD

esophagogastroduodenoscopy

FFP

fresh frozen plasma

GI

gastrointestinal

GIST

gastrointestinal stromal tumor

H&E

hematoxylin and eosin

Hb

hemoglobin

HPF

high-power field

PRBCs

packed red blood cells

RBC

red blood cell

VCE

video capsule endoscopy