Unusual Presentation of Gastrointestinal Stromal Tumor of Stomach in Neurofibromatosis Type 1: A Case Report

Swain, Sudeepta Kumar; Smile, Robinson; Arul, Tirou; David, Dipak

doi:10.1007/s12262-012-0572-y

Cited by 7 publications

(5 citation statements)

References 8 publications

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“…Five of 7 reported cases had a tumor with an ulcer or fistula in the lumen on macroscopic findings, and tumor necrosis was observed in 5 cases on histological examination. The presence of an ulcer, fistula, and necrosis may increase the risk of abscess formation; however, the present case and the case reported by Swain et al [ 9 ] had no ulcer, fistula, or necrosis. The precise reason for abscess formation in these cases was unclear.…”

Section: Discussioncontrasting

confidence: 76%

“…Cases of GISTs involving the stomach presenting as an intra-abdominal abscess are extremely rare, and only 7 cases have been reported in the English literature (table 1 ) [ 8 , 9 , 10 , 11 , 12 , 13 , 14 ]. In some cases with GISTs, tumor degeneration or necrosis can result in bleeding into the gastrointestinal lumen or peritoneum space, and bleeding into the intra-abdominal space can cause an intra-abdominal abscess.…”

Section: Discussionmentioning

confidence: 99%

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Gastrointestinal Stromal Tumor of the Stomach with an Abscess Excised by Laparoscopic Surgery

Maeda¹,

Shinohara²,

Katayama³

et al. 2016

Case Rep Gastroenterol

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Gastrointestinal stromal tumors (GISTs) of the stomach presenting as an intra-abdominal abscess are extremely rare. We herein report a case that underwent successful laparoscopic resection of gastric GIST presenting with an intra-abdominal abscess. A 70-year-old man presented with a 3-day history of acute upper abdominal pain with a fever. Laboratory data showed an elevated white blood cell count and C-reactive protein level. A CT scan revealed a mass of mixed solid and fluid components, measuring 5.5 cm, in the adjacent lesser curvature of the stomach. He was diagnosed as having an intra-abdominal abscess, and antibiotic therapy was administered. Consequently, his symptoms were relieved and a CT scan on the 7th day showed that the mass reduced and became homogenous. According to the results of a fine-needle aspiration biopsy, the tumor was suspected to be a GIST. Laparoscopic surgery with the 5-port approach was performed. The tumor existed in the lesser curvature of the stomach, and was excised en bloc with the omentum and gastric wall by wedge resection. The tumor measured 3.0 cm in diameter and originated from the serosal layer of the stomach. Histological findings showed spindle cells with a stromal growth pattern that was positive for c-kit (CD 117) and CD 34. The postoperative course was uneventful, and no recurrence has been noted in the 2 years since the operation. This is the first report of a case with gastric GIST with an intra-abdominal abscess that was successfully treated by laparoscopic surgery.

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Section: Discussioncontrasting

confidence: 76%

Section: Discussionmentioning

confidence: 99%

Gastrointestinal Stromal Tumor of the Stomach with an Abscess Excised by Laparoscopic Surgery

Maeda¹,

Shinohara²,

Katayama³

et al. 2016

Case Rep Gastroenterol

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“…A PubMed search of the literature revealed 126 case reports concerning GISTs associated with NF 1 (keywords: “Gastrointestinal stromal tumor” and “Neurofibromatosis 1”; language: English). Only 11 (8.7%) patients had gastric GISTs ( Table 1 ) [ 1 , 3 , 7 – 12 ], of whom seven had multiple GISTs in the stomach and four had only one gastric GIST. Six patients also had GISTs in the small intestine.…”

Section: Discussionmentioning

confidence: 99%

Multiple Gastric Gastrointestinal Stromal Tumors in a Patient with Neurofibromatosis Type 1

Tomatsu

Isogaki

Watanabe

et al. 2016

Case Reports in Surgery

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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-kit gene (exons 8, 9, 11, 13, and 17) but also in the PDGFRA gene (exons 12, 14, and 18). This patient is being followed up without the administration of a tyrosine kinase inhibitor.

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“…Regarding GISTs, they are not uncommon in NF-1 patients. However, recent reports have suggested that some patients can present symptomatically with bleeding and anemia, perforation, obstruction or pain with invasion into adjacent structures, suggesting that the risk of complications and aggressive disease biology should not be underestimated in NF-1 patients [57][58][59][60]. These NF-1-associated GISTs are more frequent in the small bowel than the stomach and account for 1.5% of all GISTs [53].…”

Section: Neurofibromatosis Typementioning

confidence: 99%

Gastrointestinal stromal tumors in the setting of multiple tumor syndromes

Burgoyne¹,

Somaiah²,

Sicklick³

2014

Current Opinion in Oncology

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Unusual Presentation of Gastrointestinal Stromal Tumor of Stomach in Neurofibromatosis Type 1: A Case Report

Cited by 7 publications

References 8 publications

Gastrointestinal Stromal Tumor of the Stomach with an Abscess Excised by Laparoscopic Surgery

Gastrointestinal Stromal Tumor of the Stomach with an Abscess Excised by Laparoscopic Surgery

Multiple Gastric Gastrointestinal Stromal Tumors in a Patient with Neurofibromatosis Type 1

Gastrointestinal stromal tumors in the setting of multiple tumor syndromes

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