AB228. SOH21AS200. A rare case of incidental functioning neck paraganglioma in a multiple myeloma patient

Background: Paragangliomas are neuro-endocrine tumours originating from neural crest chromaffin cells. Majority (80–85%) are found in the medulla of adrenal glands referred to as pheochromocytomas. Catecholamine secreting neuro-endocrine tumours arising outside of adrenal medulla referred as paragangliomas constitute only 0.6% of all head and neck tumours. In this case study, we present a rare case of a sporadic and functioning neck paraganglioma in a multiple myeloma patient, which was an incidental pick up.

Methods: A 57-year lady awaiting stem cell transplant for multiple myeloma presented with chest pain. A CT-pulmonary angiogram (CT-PA) out ruled a pulmonary embolism but picked up an incidental finding of a 3.5 cm left neck/thoracic inlet mass. This mass was further characterized using ultrasound and biopsy, confirming a paraganglioma. Further imaging in form of metaiodobenzylguanidine (MIBG) and PET-CT scans confirmed no other focus of disease except the neck.

Results: The patient had preoperative alpha and beta blockade after discussion in the endocrine MDT followed by surgery via lower neck transverse incision. The surgery was un-eventful, and the patient was discharged home on her first post-operative day. Final histo-pathology confirmed a benign paraganglioma. Her genetic testing subsequently returned negative for MEN2/RET gene mutation.

Conclusions: This case report describes the first ever reported relationship in literature between neck paraganglioma and multiple myeloma. A multidisciplinary approach is recommended for these complex and rare cases with pre-operative blockade followed by surgery as the definitive cure.

Keywords: Paraganglioma; multiple myeloma; head and neck tumours