Vassos Nikolaos, Perrakis Aristotelis, Schmid Axel, Croner Roland S, Gruetzmann Robert, Agaimy Abbas
Department of Surgery, University Hospital Erlangen, Erlangen, Germany.
Division of Surgical Oncology and Thoracic Surgery, Department of Surgery, Mannheim University Medical Centre, University of Heidelberg, Mannheim, Germany.
Visc Med. 2021 Jun;37(3):226-232. doi: 10.1159/000510486. Epub 2020 Sep 30.
Neuroma of the biliary tree is extremely rare with no more than 100 cases reported so far. They mostly present with obstructive jaundice and have been commonly described after surgery or abdominal trauma. Although involvement of the extrahepatic bile duct is far more common, occurrence in the intrahepatic ducts has not so far been reported.
We describe 3 cases of diffuse biliary tree neuroma affecting 3 females aged 53-68 years. None had a history of neurofibromatosis type1. All presented with progressive obstructive jaundice with no evidence of gallstones. A history of previous surgery was noted in 2 patients. Initial impression on clinical and imaging examination was highly suspicious for bile duct carcinoma in 2 patients. Histology showed diffuse neuromatous proliferation replacing and thickening the bile duct walls. The third patient had concurrent neuroma and recurrent cholangiocarcinoma causing great clinical confusion as initial biopsy showed only benign neuroma, but CA 19-9 was steadily increasing, necessitating a second biopsy which then confirmed recurrent carcinoma.
This uncommon cause of long-distance bile duct stenosis and progressive jaundice should be included in the differential diagnosis of bile duct neoplasms, in particular when there is a previous surgical history in this abdominal region.
胆管神经瘤极为罕见，迄今为止报告的病例不超过100例。它们大多表现为梗阻性黄疸，常见于手术后或腹部外伤后。虽然肝外胆管受累更为常见，但肝内胆管发生神经瘤迄今尚未见报道。
我们描述了3例弥漫性胆管神经瘤病例，患者均为53 - 68岁女性。均无1型神经纤维瘤病病史。所有患者均表现为进行性梗阻性黄疸，无胆结石证据。2例患者有既往手术史。2例患者临床及影像学检查初步印象高度怀疑为胆管癌。组织学检查显示弥漫性神经瘤样增生，取代并增厚胆管壁。第3例患者同时患有神经瘤和复发性胆管癌，造成了很大的临床困惑，因为初次活检仅显示良性神经瘤，但CA 19 - 9持续升高，需要再次活检，随后证实为复发性癌。
这种导致远距离胆管狭窄和进行性黄疸的罕见病因应纳入胆管肿瘤的鉴别诊断，特别是当该腹部区域有既往手术史时。
