Castillejo Luis A, de Jesús Julian José, González Pedro, Román Rafael
Department of Neurosurgery, Instituto Mexicano del Seguro Social, Mexico City, Mexico.
J Neurosurg Case Lessons. 2021 May 24;1(21):CASE21127. doi: 10.3171/CASE21127.
Solitary extradural plasmacytoma of the skull (SEPS) is an extremely rare entity with only 35 cases reported in the English-language literature. SEPS is a rare presentation of plasma cell dyscrasias, accounting for 4% of plasma cell tumors. The diagnosis of solitary plasmacytoma requires exclusion of multiple myeloma (MM) and prompt diagnosis and treatment.
The authors describe the case of a 52-year-old man with SEPS. He presented with a painless, progressive, soft swelling mass in the left parietal region. Magnetic resonance imaging revealed a left frontotemporal extra-axial lesion that involved the ipsilateral orbital apex and posterior ethmoidal cells. Biological studies did not reveal features suggestive of MM. A diagnosis of SEPS was based on microscopic examination and immunohistochemical analysis after surgery. The patient had an excellent recovery and was discharged the day after surgery without neurological deficit.
SEPS is a potentially curable disease, and total resection with or without radiotherapy is associated with a good prognosis and long-term recurrence-free survival. Distinction between SEPS and MM is of paramount importance because the prognosis and treatment differ.
颅骨孤立性硬膜外浆细胞瘤(SEPS)是一种极其罕见的疾病,英文文献中仅报道了35例。SEPS是浆细胞发育异常的一种罕见表现,占浆细胞肿瘤的4%。孤立性浆细胞瘤的诊断需要排除多发性骨髓瘤(MM),并需及时诊断和治疗。
作者描述了一例52岁患有SEPS的男性病例。他表现为左侧顶叶区域无痛性、进行性、软性肿胀肿块。磁共振成像显示左侧额颞叶轴外病变,累及同侧眶尖和筛窦后组气房。生物学检查未发现提示MM的特征。SEPS的诊断基于手术后的显微镜检查和免疫组织化学分析。患者恢复良好,术后次日出院,无神经功能缺损。
SEPS是一种潜在可治愈的疾病,无论是否进行放疗,全切除术后预后良好,长期无复发存活。区分SEPS和MM至关重要,因为两者的预后和治疗方法不同。