Naeim Ali, Larijani Amirhossein, Naeimi Arvin, Ghorbani Jahangir, Sadeghipour Alireza, Shirani Mohammad
Department of Neurosurgery, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Gamma Knife Center, Yas Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Childs Nerv Syst. 2025 Sep 12;41(1):279. doi: 10.1007/s00381-025-06932-z.
Brown tumors are rare focal giant-cell lesions resulting from the direct effects of parathyroid hormone (PTH) on bone tissue in hyperparathyroidism. Brown tumors most commonly affect long bones, pelvis, and ribs, whereas craniofacial involvement is uncommon, with pterygopalatine fossa involvement being exceedingly rare. This report presents a case of a 13-year-old girl with a rapidly progressing lesion in the pterygopalatine fossa, with involvement of the sphenoid and maxilla. The patient exhibited significant facial pain and V2 paresthesia, with imaging studies suggesting differential diagnoses such as craniopharyngioma and glioma. Pre-operative digital subtraction angiography ruled out highly vascular lesions, and the patient underwent complete tumor resection via an expanded endoscopic transnasal approach. Histopathological evaluation initially revealed a solid-variant aneurysmal bone cyst (ABC). Nevertheless, routine postoperative laboratory evaluation incidentally detected a significant increase in PTH and hypercalcemia, prompting further pathological reassessment and confirming the diagnosis of a brown tumor. Finally, the patient underwent a parathyroidectomy, followed by further management and regular follow-ups. This case highlights the diagnostic challenges posed by the histopathological overlap between brown tumors and other giant-cell lesions, such as ABCs and giant-cell tumors, particularly in their rare presentation in the pterygopalatine fossa. The choice of surgical approach was guided by the lesion's location, symptom severity, and rapid progression, demonstrating the utility of ipsilateral transmaxillary endoscopic techniques in accessing complex anatomical regions. Furthermore, this case underscores the importance of routine calcium and PTH testing in preoperative evaluations of cranial bone lesions to prevent potential misdiagnosis and ensure accurate, effective, and timely management.
棕色瘤是一种罕见的局灶性巨细胞病变，由甲状旁腺激素（PTH）对甲状旁腺功能亢进症患者骨组织的直接作用引起。棕色瘤最常累及长骨、骨盆和肋骨，而颅面部受累并不常见，翼腭窝受累极为罕见。本报告介绍了一名13岁女孩的病例，其翼腭窝有一个快速进展的病变，并累及蝶骨和上颌骨。患者表现出明显的面部疼痛和V2感觉异常，影像学检查提示有颅咽管瘤和神经胶质瘤等鉴别诊断。术前数字减影血管造影排除了高血管性病变，患者通过扩大的鼻内镜经鼻入路接受了肿瘤全切术。组织病理学评估最初显示为实性型动脉瘤样骨囊肿（ABC）。然而，术后常规实验室检查偶然发现PTH显著升高和高钙血症，促使进一步进行病理重新评估并确诊为棕色瘤。最后，患者接受了甲状旁腺切除术，随后进行了进一步治疗和定期随访。该病例突出了棕色瘤与其他巨细胞病变（如ABC和巨细胞瘤）之间组织病理学重叠所带来的诊断挑战，尤其是在它们罕见地出现在翼腭窝时。手术入路的选择取决于病变的位置、症状严重程度和快速进展情况，证明了同侧经上颌内镜技术在进入复杂解剖区域方面的实用性。此外，该病例强调了在颅骨病变术前评估中进行常规钙和PTH检测的重要性，以防止潜在的误诊，并确保准确、有效和及时的治疗。
