A Case of Type 1 Neurofibromatosis Associated with Gastrointestinal Stromal Tumor of the Duodenum and Pheochromocytoma

WAKABAYASHI Toshiki, SATO Tsutomu, OUCHI Shinichiro, KOTANAGI Hitoshi

doi:10.3919/jjsa.75.2622

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Other Title

神経線維腫症1型に多房性褐色細胞腫（径22cm）と十二指腸GISTを併存した1例
症例神経線維腫症1型に多房性褐色細胞腫(径22cm)と十二指腸GISTを併存した1例
ショウレイシンケイセンイシュショウ 1ガタニタボウセイカッショクサイボウシュ(ケイ 22cm)トジュウニシチョウ GIST オヘイソンシタ 1レイ

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Abstract

A 55-year-old woman with type 1 neurofibromatosis (NF-1) was referred to our hospital for a liver tumor that was observed during a medical check-up. Abdominal computed tomography (CT) showed a multilocular 22 × 20-cm cystic tumor compressing the diaphragm and kidney with a poorly defined hepatic border. A 3-cm hypervascular tumor was also noted in the duodenum. Upper gastrointestinal tract endoscopy showed a submucosal tumor in the anterior wall of the second portion of the duodenum, which was diagnosed as a gastrointestinal stromal tumor (GIST). The site corresponded to the hypervascular tumor seen on CT. The positional relationship between the duodenum and cystic tumor seen on CT suggested that the latter was a retroperitoneal tumor. While a definitive diagnosis was not made preoperatively, the possibility of hepatic metastases of the GIST was ruled out based on differences in characteristics. Intraoperatively, the retroperitoneal tumor showed extensive adhesion to the right hepatic lobe, and the posterior segment of the liver was concurrently resected. Partial duodenectomy was performed for the duodenal GIST. The retroperitoneal tumor was pathologically diagnosed as pheochromocytoma. There are few case reports of NF-1 with pheochromocytoma and GIST, and we report this patient with a discussion of the relevant literature.

Journal

Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association)

Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 75 (9), 2622-2627, 2014

Japan Surgical Association

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