Periampullary and duodenal neoplasms in neurofibromatosis type 1: two cases and an updated 20-year review of the literature yielding 76 cases.

BACKGROUND: Patients with neurofibromatosis type 1 (NF1) are at increased risk to develop tumors throughout the gastrointestinal tract, including neuromas, gastrointestinal stromal tumors (GIST), and periampullary somatostatin-rich carcinoids. Here, we briefly describe two male patients with NF1 and review the recent literature on this topic. METHODS: Databases for PubMed and MEDLINE were searched for English-language articles since 1989 using a list of keywords, as well as references from review articles. RESULTS: The results generated by the search yielded 50 articles and 74 cases. Patients most commonly presented with jaundice, weight loss, GI bleeding, or anemia. The mean age at presentation was 47.9 years, with 59% of patients being female. Mean tumor size was 3.8 cm (range 0.9-27 cm). Tumor location was the duodenum (60%), ampulla (31%), pancreas (5%), or bile duct/gallbladder (4%). Tumor type was reported as somatostatinoma (40%), GIST (34%), adenocarcinoma (8%), carcinoid (6%), neurofibroma (5%), schwannoma (4%), or gangliocytic paraganglioma (3%). Treatment included classic Whipple procedure (42%), local excision (25%), pylorus-preserving pancreaticoduodenectomy (17%), and other resection (6%). Mean follow-up was 31 months postresection (range 0-99 months): 75% of patients were alive with no evidence of disease. CONCLUSIONS: These results underscore the importance of a thorough evaluation for tumors in NF1 patients with gastrointestinal symptoms, as well as subsequent surgical management when findings suggest a tumor in the periampullary region, as resection remains the mainstay of treatment.

Long-term results of combined common femoral endarterectomy and iliac stenting/stent grafting for occlusive disease.

BACKGROUND: Common femoral artery (CFA) endarterectomy with iliac stenting or stent grafting can be an alternative to traditional open surgery in patients with aortoiliac occlusive disease. We report the long-term outcomes of this approach. METHODS: Patients undergoing CFA endarterectomy with simultaneous iliac stenting/stent grafting between 1997 and 2006 were retrospectively reviewed. Technical success, clinical and hemodynamic outcomes, and 5-year patency using life-table methodology were determined. Factors associated with reintervention and mortality were determined by logistic regression analysis. RESULTS: A total of 171 patients (mean age, 67 +/- 10 years; 38% female; 35% diabetic) underwent 193 CFA endarterectomies and iliac stent/stent grafting. Indications were rest pain (32%), tissue loss (22%), and claudication (46%). External iliac artery (EIA) lesions were present in 39%, and combined common iliac artery (CIA) and EIA lesions were seen in 61% of patients. Complete CIA/EIA occlusions were present in 41% of patients. Stent grafts were used in 41% of patients. Technical success occurred in 98% of patients. Clinical improvement was seen in 92% of patients. Mean ankle-brachial index increased from 0.38 +/- 0.32 to 0.72 +/- 0.24. Median length of stay was 2 days (range, 1-51 days). Thirty-day mortality was 2.3% and 5-year survival was 60%. Five-year primary, primary-assisted, and secondary patencies were 60%, 97%, and 98% respectively. Endovascular reintervention was required in 14% of patients; inflow surgical procedures were required in 10%. By logistic regression analysis, use of stent grafts compared with bare stents was associated with significantly higher primary patency (87% +/- 5% vs 53% +/- 7%; P < .01). CONCLUSION: Combined CFA endarterectomy with iliac intervention yield acceptable long-term results. The use of stent grafts compared with bare stents is associated with improved primary patency.