Gastrointestinal autonomic nerve tumors Jejunum - Gastrointestinal tract, Hemorrhage Embolization, Interventional therapy

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The patient was admitted with 2-day history of melena, abdominal discomfort and dizziness. Physical examination revealed conjunctival and mucosal pallor. Laboratory data documented iron deficiency anemia with the serum hemoglobin level of 7.7 g/dL, and hematocrit 22.4%. Ten months and 3 months earlier, he had a history of melena. Esophagogastroduodenal endoscopy revealed reflux of fresh blood in third and fourth portion of the duodenum from jejunum below the Treiz ligament. After emergency coil embolization of lower GI bleeding of jejunum, segmental resection of proximal jejunum was performed.

Lower GI bleeding from gastrointestinal autonomic nerve (GAN) tumor in proximal jejunum

Emergency mesenteric angiography was performed using 5-F Cobra shaped catheter (Cook, Bloomington, U.S.A.) with right femoral arterial approach, which showed hypervascular staining in proximal jejunum, and enlargement of arterial branch supplying the lesion (Fig. 1A). Superselective arteriogram was performed to precisely define the more peripheral arterial anatomy. It showed active bleeding focus, intraluminal leak of contrast media into the proximal jejunum and early visualization of draining veins (Fig. 1B). Superselective catheterization was attempted to embolize the arterial feeder using a microcatheter (Progreat; Terumo, Tokyo, Japan) and a 0.014-inch guide wire. After successful superselective catheterization of the jejunal arterial recta supplying the site of hemorrhage, we performed transcatheter embolization using two Vortex diamond shaped, 3 mm ×23 mm sized microcoils (Target Therapeutics, Fremont, CA) (Fig. 2A). Post-procedure angiogram showed total occlusion of the feeding vessel without evidence of contrast media extravasations (Fig. 2B). After the procedure, the patient stopped melena. His hemoglobin and hematocrit had increased to 10.5g/dL, 30.7%, respectively. Contrast enhanced computed tomography (CT) scan of the abdomen performed one day after the coil embolization showed lobulating contoured soft tissue mass in the region of proximal jejunum with foreign body artifact induced by microcoils adjacent to small bowel mesentery (Fig. 3A, B). The mass was well marginated, homogeneously low attenuated and not enhanced. Small bowel follow-through study revealed smooth marginated intraluminal filling defect in proximal jejunum (Fig. 3C). Overlying mucosal surface was intact and small ulcer niche was noted in central portion of the filling defect. The radiologic diagnosis was a submucosal tumor of proximal jejunum such as GIST. Segmental resection of proximal jejunum was performed. On gross examination, the tumor mass was measured 3.5×3.5×3.5 cm in size and was exposed to the serosal surface of the proximal jejunum. The epithelial lining was intact, but there was an umbilicated deep ulcer in the mid-portion of exophytic mass. The histopathologic diagnosis was gastrointestinal autonomic nerve (GAN) tumor of jejunum with high probability of malignancy.

Lower GI bleeding is hemorrhage distal to the ligament of Treitz and includes small bowel and colonic hemorrhage. The common etiologies include diverticulosis, angiodysplasia, neoplasm, and inflammatory bowel disease. The therapeutic options are medical management, endoscopic coagulation, transcatheter therapy, and surgery. Endoscopy is often the first method that is used to investigate and treat lower GI bleeding. Whereas endoscopy is feasible in cases of bleeding within the upper GI and colon, hemorrhage originating in the small bowel, when inaccessible by interventional techniques, can only be treated surgically. And also, failure of endoscopic diagnosis and therapy may occur as a result of massive bleeding, which limits precise localization of the site of hemorrhage. Surgery is still considered the mainstay of treatment; however, it is associated with significant morbidity and mortality. The reported mortality rates after emergency colonic resection for bleeding range from 10-36%. There are two transcatheter options available to the interventional radiologist for the control of lower GI bleeding: (I) pharmacologic control with use of vasopressin and (ii) embolization. Although vasopressin infusion is associated with a high initial control rate, the rebleeding rates after termination of infusion are high (20-50%) and it is associated with a high rate of cardiovascular complications (40% or so). Transcatheter embolization avoids the potential problems of catheter dislodgement and systemic complications. However, the small bowel, and especially the large bowel, do not have the rich collateral supply characteristic of stomach and duodenum. The potential risk of gut infarction after percutaneous embolization is therefore greater. Recently the development of finer coaxial micro catheters, guidewires, and digital angiographic equipment have enabled more peripheral superselective catheterization of distal vessels, permitting more selective vascular intervention. We can reduce the arterial pressure to the bleeding site, while preserving the potential for collateral arterial supply to prevent ischemic damage to the intestinal wall. Studies in the 1980s showed a 10-20% incidence of postembolization infarction, whereas later studies in the 1990s demonstrated no or fewer cases of postembolization infarction. In this respect, microcoils would be the agents of choice, which are easy to use and theoretically pose a lesser threat of ischemia than PVA particles, which may disseminate to the smaller vessels. Embolization may not be possible in cases of vessel spasm, spontaneous cessation of bleeding, and vascular tortuosity. Embolization can be the definitive and only treatment used in about half the cases of embolization. In these cases, patients can avoid the morbidity and mortality associated with emergency surgery. However, when bleeding is controlled, all patients should undergo colonoscopy to determine the underlying pathology. Angiodysplasia had a high rate of repeated bleeding despite initial angiographic control and may warrant elective surgical resection. Also resectable neoplasms require curative surgery. Embolization in these instances will allow safer and more elective surgery. In our case, emergency embolization was attempted to stop bleeding and a jejunal mass was found on the following CT. And then surgical resection was performed and the histopathologic report revealed GAN tumors. This is a case that we successfully treated bleeding of jejunal GAN tumors with transcatheter coil embolization technique. GAN tumors are extremely rare neoplasms that compose a distinct subcategory of GIST, arising from autonomic nervous system plexuses of GI tract such as those of Meissner or Auerbach, also termed plexosarcoma. A symptomatology of GAN tumor is variable and often nonspecific. Generalized symptoms of low-grade fever, malaise, fatigue, and pallor can be seen. A minority of cases may present with GI bleeding. It is not possible to distinguish GAN tumors from other GIST based on radiologic techniques. In summary, although very rare, lower GI bleeding can be caused by GAN tumors. We successfully performed emergency transcatheter coil embolization for a jejunal GAN tumor. So, in cases of lower GI bleeding, this disease entity should be born in mind, and transcatheter coil embolization can be the treatment of choice for emergency bleeding control in such a case.

1. Herrera GA, de Morales HP, Grizzle WE, Han SG. Malignant small bowel neoplasm of enteric plexus derivation (plexosarcoma): light and electron microscopic study confirming the origin of the neoplasm. Dig Dis Sci 1984;29:275-284 2. Rueda O, Escribano J, Vicente JM, Garcia F, Villeta R. Gastrointestinal autonomic nerve tumors (plexosarcomas). Is a radiological diagnosis possible? Eur Radiol 1998;8:458-460 3. Bandi R, Shetty PC, Sharma RP, Burke TH, Burke MW, Kastan D. Superselective arterial embolization for the treatment of lower gastrointestinal hemorrhage. J Vasc Interv Radiol 2001; 12:1399-1405

A. Superior mesenteric angiogram showed hypervascular staining in proximal jejunum, and enlargement of arterial branch supplying the lesion. B. Superselective arteriogram showed active bleeding focus in the proximal jejunum and intraluminal leak of contrast material (arrow).

A. Transcatheter embolization was performed with two microcoils (3 mm×23 mm in size) (arrow). B. Post-embolization angiogram showed total occlusion of the feeding vessel without evidence of contrast material extravasations.

A, B. A contrast enhanced computed tomography (CT) of the abdomen showed lobulating contoured soft tissue mass in the region of proximal jejunum with foreign body artifact induced by microcoils adjacent to small bowel mesentery. The mass was well marginated, homogeneously low attenuated and not enhanced.

Small bowel follow-through study revealed smooth marginated intraluminal filling defect.