成年患者中的青少年鼻咽血管纤维瘤：伴Fahr综合征及多种合并症的罕见表现——病例报告与文献综述
Juvenile Nasopharyngeal Angiofibroma in an Adult Patient: A Rare Presentation with Fahr Syndrome and Multiple Comorbidities-A Case Report and Literature Review.
摘要(中文译文)
青少年鼻咽血管纤维瘤（JNA）是一种良性但具有局部侵袭性的血管性肿瘤，典型发病于青春期男性。成年期诊断极为罕见，且可能与其他血管性或恶性鼻咽病变相混淆。本例患者还患有慢性低钙血症，并伴有Fahr样颅内钙化，继发于甲状腺癌治疗后长期术后甲状旁腺功能减退。据我们所知，这种共存情况此前尚未有报道。一名34岁白人男性，因甲状腺乳头状癌行全甲状腺切除术后出现甲状旁腺功能减退，伴慢性低钙血症及Fahr样颅内钙化。因气管造口功能障碍导致急性呼吸功能不全入院期间，影像学检查发现一个37×33×32 mm的异质性富血供鼻咽部肿块，延伸至右侧翼腭窝（PPF），伴有骨重塑及局灶性骨缺损。数字减影血管造影显示肿瘤血供丰富，主要由右侧上颌内动脉分支（经蝶腭动脉）供血。行内镜下切除术，组织病理学确诊为JNA。大多数JNA病例发生于7至19岁；30岁以上男性发病罕见，常导致诊断不确定性，尤其是在与鼻咽癌或其他病变鉴别时。在成人中，磁共振成像/计算机断层扫描评估局部范围及血管造影进行血管定位是降低出血风险的关键。合并的内分泌疾病强调了围手术期多学科代谢优化的必要性，但并不意味着存在病理生理学关联。本报告描述了一例成年男性JNA诊断，该患者伴有继发于慢性甲状旁腺功能减退的Fahr样颅内钙化。它强调了在成人富血供鼻咽部肿块的鉴别诊断中考虑JNA的必要性，尤其是在合并复杂基础疾病的患者中。
Abstract
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. 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. 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.
如何引用
AMA
Sigita Zālīte, Karīna Čudare, Kalvis Vērzemnieks, Sergejs Pavlovičs, Kārlis Kupčs, Ingus Vilks, et al. Juvenile Nasopharyngeal Angiofibroma in an Adult Patient: A Rare Presentation with Fahr Syndrome and Multiple Comorbidities-A Case Report and Literature Review.. Diagnostics (Basel, Switzerland). 2026; doi:10.3390/diagnostics16091327.
APA
Sigita Zālīte, Karīna Čudare, Kalvis Vērzemnieks, Sergejs Pavlovičs, Kārlis Kupčs, Ingus Vilks, et al (2026). Juvenile Nasopharyngeal Angiofibroma in an Adult Patient: A Rare Presentation with Fahr Syndrome and Multiple Comorbidities-A Case Report and Literature Review.. Diagnostics (Basel, Switzerland). https://doi.org/10.3390/diagnostics16091327
GB/T 7714
Sigita Zālīte, Karīna Čudare, Kalvis Vērzemnieks, Sergejs Pavlovičs, Kārlis Kupčs, Ingus Vilks, et al. Juvenile Nasopharyngeal Angiofibroma in an Adult Patient: A Rare Presentation with Fahr Syndrome and Multiple Comorbidities-A Case Report and Literature Review.[J]. Diagnostics (Basel, Switzerland), 2026 doi:10.3390/diagnostics16091327.
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