Bhandari Ramesh, Riddiough Georgina, Lokan Julie, Weinberg Laurence, Efthymiou Marios, Nikfarjam Mehrdad
Department of Surgery, University of Melbourne, Austin Health. Heidelberg, Melbourne, Victoria, Australia.
JOP. 2015 Jan 31;16(1):81-4. doi: 10.6092/1590-8577/2906.
Somatostatinoma arising from the minor papilla in a patient with neurofibromatosis type 1 (NF1) is a known but very rare condition, which may cause non-specific symptoms and can present because of its mass effect.
A fifty-year-old female presenting with ongoing non-specific abdominal pain for a few months duration was found to have a mass involving the minor papilla. She had a history of NF1 but was otherwise well. Magnetic resonance imaging showed a dilated pancreatic duct and the finding of pancreatic divisum. The lesion was (18)fluorine-fluoro-2-deoxyglucose positron emission tomography/computed tomography and (68)gallium (Ga) DOTATATE negative. Endoscopic ultrasound revealed a 1.7 cm lesion confined to the minor ampulla. Endoscopic retrograde pancreatography attempts with biopsy and endoscopic ultrasound fine needle aspiration biopsy were inconclusive and resulted in mild pancreatitis on two occasions. Open local excision of the minor papilla was undertaken without complications. Histology confirmed a completely excised grade 1 neuroendocrine tumor with intense diffuse somatostatin staining.
Somatostatinoma of the minor papilla is a rare tumor that most commonly occurs in the setting of NF1 and may be amenable to local excision.
1型神经纤维瘤病（NF1）患者发生于小乳头的生长抑素瘤是一种已知但极为罕见的病症，可引起非特异性症状，并因其占位效应而表现出来。
一名50岁女性持续数月出现非特异性腹痛，检查发现有一个累及小乳头的肿块。她有NF1病史，但其他方面健康。磁共振成像显示胰管扩张及胰腺分裂症表现。该病变氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描（18F-FDG PET/CT）及镓（68Ga） DOTATATE显像均为阴性。内镜超声显示一个局限于小壶腹的1.7厘米病变。经内镜逆行胰胆管造影术活检及内镜超声引导下细针穿刺活检均未得出明确结果，且两次均导致轻度胰腺炎。遂对小乳头进行开放性局部切除，未出现并发症。组织学检查证实为完全切除的1级神经内分泌肿瘤，生长抑素呈强烈弥漫性染色。
小乳头生长抑素瘤是一种罕见肿瘤，最常发生于NF1背景下，可能适合局部切除。
