Massive Bleeding from a Small Intestinal Submucosal Tumor-like Arterial Malformation: An Unclassifiable Vascular Lesion Revealed by a Detailed Pathological Evaluation

Gastrointestinal stromal tumors (GISTs) are relatively common in neurofibromatosis type 1 (NF 1) patients. Approximately 90% of GISTs associated with NF 1 are located in the small intestine, while sporadic GISTs are most commonly located in the stomach. Here we report an extremely rare case of an NF 1 patient with multiple gastric GITs (90 or more) but without multiple small intestinal tumors. A 63-year-old female patient who had a history of NF 1 underwent surgery for a gastric neuroendocrine tumor and gastric submucosal tumor (SMT). During the operation, multiple small nodules were identified on the serosal surface of the upper stomach. SMT and multiple nodules on the serosal surface were diagnosed as GISTs consisting of spindle cells positive for KIT, CD34, and DOG-1. Both GIST and the normal gastric mucosa showed no mutations not only in the c-kitgene (exons 8, 9, 11, 13, and 17) but also in thePDGFRAgene (exons 12, 14, and 18). This patient is being followed up without the administration of a tyrosine kinase inhibitor.

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Arterial malformation of stomach: A cause of massive bleeding

Pathology ◽

10.3109/00313028209069046 ◽

1982 ◽

Vol 14 (1) ◽

pp. 81-84 ◽

Cited By ~ 10

Author(s):

Tak-Min Kung ◽

J. Wong

Keyword(s):

Massive Bleeding ◽

Arterial Malformation

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Recurrent massive bleeding from a small intestinal arteriovenous malformation after surgery for biliary atresia in an infant: a case report and literature review

Surgical Case Reports ◽

10.1186/s40792-021-01288-3 ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Wataru Kudo ◽

Katsunori Kouchi ◽

Ayako Takenouchi ◽

Aki Matsuoka ◽

Kiyoaki Yabe ◽

...

Keyword(s):

Arteriovenous Malformation ◽

Frozen Section ◽

Massive Bleeding ◽

Roux Limb ◽

Intraoperative Endoscopy ◽

Bleeding Lesion ◽

Contrast Enhanced ◽

Frozen Section Analysis ◽

Small Intestinal ◽

Section Analysis

Abstract Background Small intestinal arteriovenous malformation (AVM) can cause bleeding. Most small intestinal AVMs occur during adulthood, rarely in infancy. We report a case of an infant with hemorrhage due to small intestinal AVM early and recurrently after Kasai portoenterostomy (PE) for biliary atresia (BA). Case presentation A 51-day-old male infant was admitted to our institution for obstructive jaundice. Laparotomic cholangiography revealed BA (IIIb1μ), and Kasai PE was performed at 60 days of age. On postoperative day 17, he developed massive melena and severe anemia. Contrast-enhanced computed tomography (CT) revealed that the jejunum around the PE site was strongly enhanced with enhancing nodules in the arterial phase, and a wide area of the Roux limb wall was slightly enhanced in the venous phase. As melena continued, emergency laparotomy was performed. There were no abnormal macroscopic findings at the PE site except for a clot in the Roux limb 5 cm away from the PE site, and the Roux limb was resected 5 cm. On further investigation, a red spot was detected on the jejunal serosa 30 cm away from the Roux-en-Y anastomosis site. PE and wedge resection for the red spot were performed. Histopathologically, both specimens indicated AVM. He was jaundice-free 65 days after the first surgery. However, at 7 months of age, he developed massive melena again. Contrast-enhanced CT and upper gastrointestinal endoscopy revealed no bleeding lesions. Hemorrhagic scintigraphy showed a slight accumulation at the hepatic hilum prompting an emergency surgery. Intraoperative endoscopy detected a bleeding lesion at the PE site, and the Roux limb was resected (approximately 6 cm). Intraoperative frozen section analysis of the stump of the resected jejunum revealed no abnormal vessels. PE was performed, and permanent section analysis revealed an AVM in the resected jejunum. The postoperative course was uneventful without re-bleeding. Conclusions We experienced a case of recurrent massive bleeding from small intestinal AVM in an infant after surgery for BA. Intraoperative endoscopy and frozen section analysis helped identify the bleeding lesion and perform a complete resection of the small intestinal AVM, even after surgery, in the infant.

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Laparoscopic Splenectomy in a Patient with Sclerosing Angiomatoid Nodular Transformation

Case Reports in Gastroenterology ◽

10.1159/000506934 ◽

2020 ◽

Vol 14 (1) ◽

pp. 212-219

Author(s):

Toru Kusano ◽

Chusei Ryu ◽

Toshikazu Matsuo ◽

Hiroko Hayashi

Keyword(s):

Laparoscopic Splenectomy ◽

Submucosal Tumor ◽

Vascular Lesion ◽

Preoperative Imaging ◽

Upper Gastrointestinal Endoscopy ◽

Histopathological Diagnosis ◽

Sclerosing Angiomatoid Nodular Transformation ◽

Specific Tumor ◽

Contrast Enhanced ◽

History Of

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a specific, tumor-forming, non-neoplastic, vascular lesion with few reported cases worldwide. Herein, we describe the case of a patient who underwent laparoscopic splenectomy for SANT. A 47-year-old woman underwent upper gastrointestinal endoscopy for suspected gastric submucosal tumor. Contrast-enhanced abdominal computed tomography revealed the presence of a gradually enhancing lesion in the splenic hilum. Although we suspected splenic fibrotic hamartoma, malignancy could not be ruled out. Therefore, the patient underwent laparoscopic splenectomy, resulting in the histopathological diagnosis of SANT. Although SANT is a benign tumor, it may be difficult to obtain definitive diagnosis using preoperative imaging alone. Because the long-term natural history of SANT is unknown, we believe that splenectomy could be an appropriate technique for the diagnosis and treatment of SANT.

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