An Isolated Soft Tissue Ectopic Paraganglioma in the Head and Neck

ABSTRACT Background Paragangliomas are rare neuroendocrine tumors derived from autonomic paraganglia of neural crest origin. While typically found at predictable sites in the head and neck, ectopic paragangliomas in unusual locations are exceptionally rare and diagnostically challenging. Methods We describe a novel presentation of an ectopic paraganglioma found as a discrete soft tissue mass in the neck. Results A 45‐year‐old female with a pathogenic mutation in the SDHB gene was diagnosed with an isolated ectopic paraganglioma in left level III. Intraoperatively, the soft tissue mass was anatomically separate from the carotid sheath and all thyroid and laryngeal neurovascular structures. Histopathology revealed the absence of lymph node architecture, excluding the diagnosis of metastasis. Conclusions This case represents a previously unreported location of an ectopic head and neck paraganglioma, unrelated to known neurovascular structures or nodal metastasis, highlighting key diagnostic and management considerations—especially in the context of hereditary disease.