Madsen Peter J, Kundishora Adam J, Reeves Benjamin C, Coyle Anne M, Nagasawa Daniel T, Wong Judith M, Yang Isaac, Tucker Alexander M
1Department of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
2Department of Neurosurgery, Yale University School of Medicine, New Haven, Connecticut.
J Neurosurg Case Lessons. 2024 May 13;7(20). doi: 10.3171/CASE23640.
Skull lesions are a common finding in children, with dermoid cysts and eosinophilic granulomas observed most frequently. However, primary intraosseous xanthomas of the calvaria, which are lytic, expansile lesions that develop without underlying hyperlipidemic disease, are rare in children, with only one prior case reported.
The authors describe the case of a healthy 6-year-old male who presented with a 2-month history of an enlarging midline skull mass that developed after a recent minor trauma. Imaging showed a full-thickness, lytic frontal bone lesion with an aggressive appearance and heterogeneous contrast enhancement. The patient underwent gross-total resection of the lesion with placement of a mesh cranioplasty. Histopathology revealed a primary intraosseous xanthoma. The patient was discharged on postoperative day 2 and required no further treatment at the 1-month follow-up.
This is the first reported case of a primary intraosseous xanthoma in the frontal bone of a pediatric patient. It emphasizes the need to include primary xanthomas in the differential diagnosis for pediatric skull lesions, particularly when the lesion has an aggressive radiographic appearance or the patient has a history of focal trauma. Furthermore, our findings indicate that resection, together with subsequent monitoring for lesion reccurrence, is an adequate first-line treatment.
颅骨病变在儿童中很常见，最常观察到的是皮样囊肿和嗜酸性肉芽肿。然而，颅骨原发性骨内黄瘤是一种溶骨性、膨胀性病变，在无潜在高脂血症的情况下发生，在儿童中罕见，此前仅报道过一例。
作者描述了一名健康6岁男性的病例，该患者有一个中线颅骨肿块，在最近一次轻微创伤后出现，持续增大2个月。影像学检查显示为全层溶骨性额骨病变，外观侵袭性，对比增强不均匀。患者接受了病变的全切除并进行了网状颅骨成形术。组织病理学检查显示为原发性骨内黄瘤。患者术后第2天出院，1个月随访时无需进一步治疗。
这是首次报道的小儿患者额骨原发性骨内黄瘤病例。它强调在小儿颅骨病变的鉴别诊断中需要考虑原发性黄瘤，特别是当病变具有侵袭性影像学表现或患者有局灶性创伤史时。此外，我们的研究结果表明，切除并随后监测病变复发是一种合适的一线治疗方法。
