Synchronous Gastrointestinal Stromal Tumor and Serous Adenocarcinoma Associated with Neurofibromatosis Type 1: A Case Report

초록

Gastrointestinal stromal tumor is a tumor that typically arises in the gastrointestinal tract and peritoneum and is the most common mesenchymal neoplasm of the gastrointestinal tract. In patients with neurofibromatosis type 1, GIST is the most common non-neurological tumor. When GISTs occur in patients with NF1, surgical treatment is the first-line treatment of choice. In this patient, a small bowel resection was performed to remove the GIST and a hernia repair was performed for the hernia due to a previous surgery. However, serous adenocarcinoma (SA) was also found in the hernia sac and is reported here. Materials and methods: A 78-year-old woman diagnosed with NF1 was referred to surgery after an outpatient visit to the gastroenterology department for a proximal jejunal area mass-like lesion on computed tomography performed for abdominal pain. The patient was diagnosed with hypertension, diabetes, peripheral arterial disease, and NF1 Patient had undergone laparoscopic cholecystectomy for acute cholecystitis and salphingo-oophorectomy for both ovarian cysts. She had an incisional hernia at the site of her previous surgery. She also had a history of endoscopic retrograde biliary drainage insertion for ampullar of vater adenoma. Physical examination of the patient revealed multiple cafe-au-lait patches and numerous subcutaneous soft nodular masses on the skin of the entire body, including the head, arms, legs, and trunk. The patient's abdomen was tender and non-painful, but the masses were not directly palpable. The patient underwent small bowel resection and anastomosis and incisional hernia repair and was discharged on day 7 without any complications. Postoperative biopsy confirmed intermediate risk GIST and high-grade serous adenocarcinoma on hernia sac.