A RARE CASE OF NON-SECRETORY RETROPERITONEAL PARAGANGLIOMA POSING A MANAGEMENT CHALLENGE

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Dr. Chanda Rai
Dr. Nishant Kumar
Dr. Zachariah Chowdhury
Dr. Zikki Hasan Fatima

Abstract

Paraganglioma is a rare neuroendocrine tumour that involves neural structures and blood vessels and occurs due to abnor- mal migration of neural crest cells. These tumours secrete catecholamines, posing a significant threat to patients. Here we describe a case of a 48-year old female who came with a big abdominopelvic mass and prolapsed uterus. Following routine lab analysis and contrast-enhanced computed tomography (CECT) imaging, she underwent exploratory laparotomy, where the abdominal mass was excised, and sigmoid colectomy and covering ileostomy followed by vaginal hysterectomy. Her post-operative histo-pathology report revealed the rare diagnosis of paraganglioma, characterised by positive markers for chromogranin (diffuse), CD56 (diffuse), INSM1 (focal, weak to moderate), and GATA3 (focal, weak to moderate) while neg- ative for S-100 and PanCK. DOTA-PET was done which did not show any residual disease. After a multi-disciplinary tumour board meeting, it was decided to keep her under observation. Diagnosis of such rare endocrine neoplasms is not to be missed and it is important to bring such cases to the purview of the clinicians for improved understanding and management.

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