Abstract
A 32-year-old man with sudden onset of right abdominal pain and anemia was immediately admitted because abdominal contrast enhanced CT showed an 8-cm mass with internal hemorrhage, which was located in the duodenal second portion. After establishing conservative hemostasis, a biopsy of the duodenal submucosal tumor via upper gastrointestinal endoscopy was performed, leading to the diagnosis of gastrointestinal stromal tumor (GIST). A thorough examination of hemostasis was carried out for a prolonged prothrombin time (PT), resulting in the diagnosis of congenital factor VII (FVII) deficiency. We conducted subtotal stomach-preserving pancreaticoduodenectomy with careful attention to the following point. With a goal of keeping PT-international normalized ratio ≤1, the patient was perioperatively managed by the administration of recombinant activated FVII at intervals of 4 to 6 hours. The duodenal GIST caused a large amount of bleeding due to tumor congestion, but good hemostasis was achieved after the completion of tumor extirpation. The patient had no postoperative complications, and was discharged 16 days after surgery. Histopathological examination of the resected specimen showed that the tumor cells did not invade the surrounding tissue. Immunohistochemically, the tumor had an MIB-1 labeling index of 30 to 40%, was positive for c-kit and DC-34, but negative for desmin and S-100. On the basis of the above findings, the present case was diagnosed as high-risk GIST. We reported a very rare case of hemorrhagic duodenal GIST with congenital FVII deficiency, and mentioned a management of congenital FVII deficiency when performing major surgery.
