Neurofibromatosis type 1 (NF1) is associated with an increased risk of gastrointestinal stromal tumors (GISTs). Vasculopathy, such as aneurysms, is a recognized complication of NF1; however, it is little known among gastroenterologists. We report a 65-year-old woman with NF1 who developed hemorrhagic shock due to rupture of an intercostal artery aneurysm during sunitinib therapy. The patient underwent distal gastrectomy for duodenal GIST at age 49, but later developed hepatic and peritoneal recurrence. Because of failure of imatinib therapy and multiple transarterial embolization procedures, sunitinib therapy was initiated, stabilizing the disease for nine months. During treatment, she developed acute back pain with severe hypertension (220/106 mmHg) and was immediately admitted, where intravenous nicardipine was administered. Despite initial stabilization, the patient abruptly went into shock the following day. Dynamic computed tomography (CT) revealed a massive right hemothorax, posterior mediastinal hematoma, and contrast extravasation from a right intercostal artery aneurysm. Emergency angiography confirmed rupture of the ninth intercostal artery aneurysm measuring 18 mm. Embolization with microcoils achieved complete hemostasis. The patient recovered without sequelae and was discharged on day 34. Through a literature review, we identified 16 cases of intercostal artery aneurysm rupture in NF1. Of these, 11 were treated with embolization, although two experienced rebleeding. Sunitinib inhibits vascular endothelial growth factor signaling, which can induce hypertension and destabilize vascular integrity. Sunitinib therapy in NF1 patients requires strict blood pressure control. Additionally, pre-treatment vascular screening with CT angiography may be warranted.
INTRODUCTION: Gallbladder varices (GBV) are a rare form of ectopic varices associated with portal hypertension. They are often difficult to diagnose prior to rupture, which carries a high mortality rate. We report a case of ruptured GBV successfully treated with laparoscopic cholecystectomy following a definitive preoperative diagnosis by endoscopic ultrasonography (EUS). CASE PRESENTATION: A 43-year-old male with a history of alcoholic cirrhosis presented with epigastric pain. Contrast-enhanced CT revealed massive ascites and extravasation from the gallbladder wall, indicating active intra-abdominal hemorrhage. However, the specific etiology remained unclear. Transabdominal ultrasonography was suboptimal due to massive ascites and intestinal gas. Given the patient's high risk for emergency laparotomy in a resource-limited setting and stable hemodynamics, we opted for overnight conservative management. The following morning, EUS was performed to definitively identify the bleeding source and exclude other upper gastrointestinal bleeding. EUS revealed prominent blood flow around the gallbladder neck suggestive of varices. Based on these comprehensive findings, a diagnosis of ruptured GBV was established. This definitive diagnosis allowed us to select a minimally invasive laparoscopic cholecystectomy. The postoperative course was uneventful, and the patient was discharged on POD 7. Although strict follow-up was planned, the patient was lost to follow-up. He died 1 year later from another gastrointestinal hemorrhage. CONCLUSIONS: Blood flow evaluation with EUS is valuable forthe definitive diagnosis of ruptured GBV, especially when other imaging modalities are inconclusive. Accurate preoperative diagnosis by EUS enables the selection of laparoscopic cholecystectomy as a safe and effective therapeutic option, avoiding high-risk emergency open surgery in patients with liver cirrhosis.
PURPOSE:In radiotherapy for bleeding gastric cancer, no standard response definition exists; studies have used transfusion-only or transfusion-plus-hemoglobin criteria. After inter-facility transfer or during home care-sometimes following palliative radiotherapy-laboratory results from other facilities are often unavailable, leaving patients inevaluable. We therefore tested whether omitting hemoglobin compromises the precision of response assessment. METHODS AND MATERIALS:In criteria A, referring to our original study, patients were diagnosed as responders when all the following three conditions were met: (1) hemoglobin levels ≥ 8.0 g/dL; (2) 14 consecutive days without blood transfusion before blood sampling; and (3) no salvage treatment. In criteria B, patients were diagnosed as responders when only (2) and (3) of the above conditions were met. The strength of agreement between criteria A and B was estimated by calculating Gwet's first-order agreement coefficient (AC1) at 4- and 8-week follow-up for patients completing planned radiotherapy with assessable response. RESULTS:In 36 evaluable patients at 4-week follow-up, response rates were 69% (25/36) by criteria A vs. 78% (28/36) by criteria B (AC1, 0.86; almost perfect agreement). In 30 evaluable patients at 8-week follow-up, response rates were 83% (25/30) by criteria A vs. 90% (27/30) by criteria B (AC1, 0.91; almost perfect agreement). CONCLUSIONS:Criteria A and B showed almost perfect agreement at 4- and 8-week assessments among evaluable patients with available hemoglobin data. ADVANCES IN KNOWLEDGE:Among patients with available hemoglobin data, criteria A and B showed almost perfect agreement. The observed agreement may not be fully generalizable to patients without hemoglobin assessment.
We report the case of a 23-year-old nulligravida woman with low-grade endometrial stromal sarcoma (LGESS) diagnosed using ultrasonography-guided transvaginal needle biopsy. The patient presented with severe anemia and heavy menstrual bleeding. MRI revealed a 10-cm uterine tumor with irregular signal intensity. Due to her virginal status and the intramural location of the tumor, conventional endometrial sampling was not feasible. Transvaginal ultrasound-guided needle biopsy was successfully performed under general anesthesia, providing an accurate preoperative diagnosis. Following oocyte cryopreservation, she underwent modified radical hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy. Pathological examination confirmed LGESS with stage IIB disease. The patient remains disease-free at 30 months post-surgery with adjuvant letrozole therapy. This case demonstrates the efficacy of ultrasound-guided needle biopsy for preoperative diagnosis of uterine sarcoma in young women when conventional sampling methods are not applicable.
