A Case of Gastrointestinal Stromal Tumor of the Urinary Bladder (30 cm in Diameter) and the Jejunum (7.5cm) Associated with Neurofibromatosis Type I

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  • 径30cmの膀胱GISTと径7.5cmの空腸GISTを合併した神経線維腫症1型の1例
  • 症例 径30cmの膀胱GISTと径7.5cmの空腸GISTを合併した神経線維腫症1型の1例
  • ショウレイ ケイ 30cm ノ ボウコウ GIST ト ケイ 7.5cm ノ ソラ チョウ GIST オ ガッペイ シタ シンケイ センイ シュショウ 1ガタ ノ 1レイ

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Abstract

Gastrointestinal stromal tumors (GIST) arisen from the urinary bladder wall is extremely rare. We have experienced surgical intervention for a patient who had a GIST (30 cm in diameter) arisen from the bladder, and a GIST (7.5 cm in diameter) arisen from the jejunum. Besides, he was simultaneously diagnosed as having neurofibromatosis type I. A 58-year-old male patient was referred to our institute because of abdominal distention. Abdominal and pelvic CT scans showed a huge solid and cystic tumor 30 cm in diameter in the pelvic to abdominal cavity and an abnormal up-take to the solid component in this tumor was found on the positron emission tomography (PET) CT. At the same time, we confirmed café au lait spots in his body trunk and schwannoma at the dorsum of the hand. Neurofibromatosis type I was diagnosed. He underwent surgery to improve his symptom and to prevent progressive anemia. Operative findings indicated that the tumor was seemingly arisen from the urinary bladder wall and we resected the tumor combined with a part of the bladder wall. We also found another tumor of the jejunum, and resected it. Histopathological examination demonstrated that the both tumors were c-kit positive and were diagnosed with GIST. Proliferation of spindle tumor cells which continued from the proper muscle of the bladder in the main tumor demonstrated the GIST to have arisen from the bladder.

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