Ben Nsir Atef, Thai Quoc-Anh, Chaieb Larbi, Jemel Hafedh
Department of Neurosurgery, Fattouma Bourguiba University Hospital - University of Medicine of Monastir, Monastir, Tunisia.
Department of Neurosurgery, The Johns Hopkins Hospital - The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
World Neurosurg. 2015 Sep;84(3):866.e11-4. doi: 10.1016/j.wneu.2015.04.024. Epub 2015 Apr 25.
Xanthogranuloma, also known as cholesterol granuloma, is an extremely rare intracranial neoplasm most commonly located in the middle ear, petrous apex, or choroid plexus. Exclusively suprasellar xanthogranulomas are exceptional and this report presents a very rare case in the pediatric population, particularly unique due to the presence of calcification.
A 17-year-old girl presented with primary amenorrhea with computed tomography and magnetic resonance imaging showing a large calcified enhancing suprasellar mass, which was presumptively diagnosed as a craniopharyngioma on the basis of its clinical and radiologic appearance. Gross total resection of a well-encapsulated, exclusively suprasellar tumor was achieved, without postoperative neurologic deficits. Histologic examination found fibrous tissue with abundant cholesterol clefts, multinucleated giant cells, and hemosiderin deposits but no epithelial cells. The final histologic diagnosis was a xanthogranuloma.
Xanthogranuloma, although extremely rare in the pediatric population, may present as a calcified suprasellar mass and manifest with primary amenorrhea. The prognosis after gross total resection is likely favorable; however, long-term follow-up is indicated for these rare neoplasms.
黄色肉芽肿，也称为胆固醇肉芽肿，是一种极其罕见的颅内肿瘤，最常见于中耳、岩尖或脉络丛。单纯位于鞍上的黄色肉芽肿极为罕见，本报告介绍了一例儿科患者中的罕见病例，因其存在钙化而尤为独特。
一名17岁女孩因原发性闭经就诊，计算机断层扫描和磁共振成像显示鞍上有一个巨大的钙化强化肿块，根据其临床和影像学表现初步诊断为颅咽管瘤。成功实现了对一个包膜完整、单纯位于鞍上的肿瘤的全切除，术后无神经功能缺损。组织学检查发现纤维组织中有丰富的胆固醇裂隙、多核巨细胞和含铁血黄素沉积，但无上皮细胞。最终组织学诊断为黄色肉芽肿。
黄色肉芽肿在儿科人群中虽然极为罕见，但可能表现为鞍上钙化肿块并伴有原发性闭经。全切除后的预后可能良好；然而，对于这些罕见肿瘤需要进行长期随访。
