Juvenile Nasopharyngeal Angiofibroma in an Adult Patient : A Rare Presentation with Fahr Syndrome and Multiple Comorbidities—A Case Report and Literature Review

Abstract

Background and Clinical Significance: Juvenile nasopharyngeal angiofibroma (JNA) is a benign but locally aggressive vascular tumor, classically affecting adolescent males. Diagnosis in adulthood is exceptionally uncommon and may mimic other vascular or malignant nasopharyngeal lesions. This patient also had chronic hypocalcemia with Fahr-like intracranial calcifications secondary to long-standing postoperative hypoparathyroidism after thyroid carcinoma treatment. To our knowledge, this coexistence has not been previously reported. Case Presentation: A 34-year-old Caucasian male with papillary thyroid carcinoma treated with total thyroidectomy developed postoperative hypoparathyroidism with chronic hypocalcemia and Fahr-like intracranial calcifications. During admission for acute respiratory insufficiency due to tracheostomy dysfunction, imaging revealed a 37 × 33 × 32 mm heterogeneous, hypervascular nasopharyngeal mass extending into the right pterygopalatine fossa (PPF) with bone remodeling and focal bony dehiscence. Digital subtraction angiography demonstrated a markedly hypervascular tumor, predominantly supplied by branches of the right internal maxillary artery (via the sphenopalatine artery). Endoscopic resection was performed, and histopathology confirmed JNA. Most JNA cases occur between 7 and 19 years of age; presentations in men older than 30 years are rare and often generate diagnostic uncertainty, particularly when differentiating from nasopharyngeal carcinoma or other lesions. In adults, magnetic resonance imaging/computed tomography for assessment of local extent and angiography for vascular mapping are key to minimizing hemorrhagic risk. The concurrent endocrine disorder emphasizes the need for multidisciplinary perioperative metabolic optimization, without implying a pathophysiological link. Conclusions: This report illustrates JNA diagnosed in adulthood in a male with Fahr-like intracranial calcifications secondary to chronic hypoparathyroidism. It highlights the necessity of considering JNA in the differential diagnosis of hypervascular nasopharyngeal masses in adults, especially in patients with complex comorbidities.