Case Report: Pseudo-Mirizzi Syndrome as a Rare Initial Clinical Presentation of MEN1 Syndrome

Bhimsi Kandoriya, Hirdaya Hulas Nag, Anurita Srivastava, Surbhi Goyal and Pritesh Kumar N

Department of Surgical Gastroenterology, GB Pant Institute of Postgraduate Medical Education and Research, Delhi University, India Department of Radiation Oncology, Lok Nayak Hospital, Delhi University, India Department of Pathology, GB Pant Institute of Postgraduate Medical Education and Research, Delhi University, India

^*Correspondance to: Hirdaya Hulas Nag 

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Abstract:

Introduction: Pseudo Mirizzi Syndrome (PMS) refers to extrinsic compression of the bile duct without calculi. PMS due to metastatic lymph node in Multiple Endocrine Neoplasia type 1 (MEN1) has not previously been described, to our knowledge, this is the first reported case of PMS in MEN 1 syndrome. Case Presentation: A 48-year female presented with upper abdominal pain and fever. Examination revealed tenderness in the right upper abdomen; the rest of the abdomen was unremarkable. Ultrasound and MR imaging suggested acute cholecystitis with features of Mirizzi syndrome. Laparoscopic cholecystectomy was performed due to persistent pain and ultrasound evidence of Gallbladder (GB) perforation. Intraoperatively, the GB was distended, and a large pericholedochal lymph node was found compressing the mid common hepatic duct. Histopathology confirmed necrotizing cholecystitis, and the lymph node showed metastatic, Neuroendocrine Tumor (NET). Further evaluation revealed a Ga-68 DOTANOC avid pancreatic and liver lesion, pituitary macroadenoma and parathyroid adenomas, consistent with MEN1 diagnosis. Genetic testing confirmed a pathogenic MEN1 mutation. Conclusion: This report underscores the importance of recognizing atypical causes of biliary obstruction, such as Pseudo-Mirizzi syndrome due to metastatic lymph node involvement, particularly in patients with syndromic conditions like MEN1. Clinicians should maintain a high index of suspicion when encountering unusual biliary manifestations.

Keywords:

Acute cholecystitis; Gastrinoma; MEN1 syndrome; Pancreatic neuroendocrine tumor; Pituitary macroadenoma; Pseudo mirizzi syndrome

Cite the Article:

Kandoriya B, Hulas Nag H, Srivastava A, Goyal S, Pritesh Kumar N. Case Report: Pseudo-Mirizzi Syndrome as a Rare Initial Clinical Presentation of MEN1 Syndrome. Clin Oncol. 2025; 10: 2127. ORCID ID: https://orchid. org/0009-0006-7072-8015.