Ismael Farhan, Khawar Sidrah, Hamza Ameer
Dow University of Health Sciences Karachi, Department of Pathology, Karachi, Pakistan.
University of Kansas, University of Kansas Medical Center, Department of Pathology, Kansas City, Kansas, USA.
Autops Case Rep. 2021 Oct 22;11:e2021338. doi: 10.4322/acr.2021.338. eCollection 2021.
Mucosal Schwann cell hamartoma (MSCH) is a rare benign neurogenic tumor characterized by pure S100p positive spindle cell proliferation. Most cases occur in the distal colon. Involvement of the gall bladder is exceedingly rare. There have been no reports of recurrence or a syndromic association with MSCH. Herein, we describe a case of MSCH of the gallbladder in a 55-year-old female patient with prior history of gastrointestinal neurofibromas who presented with abdominal pain. MR imaging revealed choledocholithiasis, gallbladder thickening, and marked biliary and pancreatic ductal dilation. The patient subsequently underwent cholecystectomy with choledochoduodenostomy. Histologic evaluation of the gallbladder showed diffuse expansion of the mucosa with S100p positive cells with spindly nuclei and indistinct cytoplasmic borders and diagnosis of MSCH of the gallbladder was rendered.
黏膜施万细胞瘤（MSCH）是一种罕见的良性神经源性肿瘤，其特征为单纯S100p阳性梭形细胞增殖。大多数病例发生在结肠远端。胆囊受累极为罕见。目前尚无MSCH复发或综合征相关性的报道。在此，我们描述一例55岁女性胆囊MSCH病例，该患者既往有胃肠道神经纤维瘤病史，表现为腹痛。磁共振成像显示胆总管结石、胆囊增厚以及显著的胆管和胰管扩张。该患者随后接受了胆囊切除术及胆总管十二指肠吻合术。胆囊组织学评估显示黏膜弥漫性扩张，有S100p阳性细胞，细胞核呈梭形，胞质边界不清，诊断为胆囊MSCH。
