Concurrent head and neck paragangliomas and pulmonary carcinoid tumor: A rare clinical entity.
Faria Nisar, Hoo Jung Rhim, Roman Finocchiaro, Vikas Jain
Abstract
Open AccessWe report the first documented case of a 61-year-old female presenting with concurrent multiple head and neck paragangliomas (HNPGLs) and a pulmonary carcinoid tumor, representing an unprecedented association of synchronous neuroendocrine tumors (NETs). This rare coexistence presented unique diagnostic challenges requiring multimodal imaging, including FDG PET-CT, contrast-enhanced CT, MRI, and Cu-64 DOTATATE PET-CT for comprehensive tumor characterization. Management employed a tailored multidisciplinary approach: surgical resection of the dominant carotid body tumor with excellent local control, stereotactic radiation therapy for remaining cervical lesions (45 Gy), and active surveillance with octreotide therapy for the pulmonary carcinoid. At 24-month follow-up, all lesions remained stable with no evidence of progression or new neurological deficits. Despite declining genetic testing, the patient's multiple paragangliomas strongly suggest hereditary predisposition, emphasizing the critical importance of genetic counseling in such presentations. The successful management approach provides a framework for similar complex presentations and highlights the potential for shared molecular mechanisms underlying synchronous neuroendocrine tumorigenesis.