Department of ENT, Strasbourg University Hospital, 1, avenue de Molière, Strasbourg, France.
Department of Neurosurgery, Strasbourg University Hospital, 1, avenue de Molière, Strasbourg, France.
Neurochirurgie. 2022 Oct;68(5):e34-e39. doi: 10.1016/j.neuchi.2022.03.005. Epub 2022 Apr 25.
Cutaneous Central Follicular Lymphoma (CCFL) is a type B cutaneous lymphoma with a usually indolent course. Scalp localization of CCFL is extremely rare, we report a new case mimicking an epidural hematoma, and showing a rapid progression with aggressive infiltration of skin, calvaria, dura and brain parenchyma.
A 58-year-old patient with an unlabeled polymalformative syndrome was admitted to the Emergency department following a head injury secondary to a self-resolving tonic-clonic epileptic seizure. The initial CT-scan was interpreted as a minor subcutaneous and epidural hematoma initially deemed for conservative management. Within 4 days, the patient showed a progressive neurological deterioration culminating into a stuporous status which prompted a constrast-enhanced brain MRI. The scan revealed a multilayered solid lesion, extending from the subgaleal compartment to the subdural space, threatening the integrity of overlying skin and causing infiltration of the brain parenchyma. Following emergency neurosurgical excision a definitive histology diagnosis of central follicular lymphoma was made. A focused chemotherapy with high-dose Methotrexate with R-CHOP protocol led to disease control until the latest follow up at 2 years.
To our knowledge, this case represents the first CCFL invading the brain parenchyma and the second extending to the dura. Although such tumor is usually indolent the aggressive behavior herein reported extend the differential diagnosis to high-grade meningiomas, sarcomas, and metastases. Prognostication and appropriate adjuvant treatment require prompt surgical excision and histological confirmation.
皮肤中心滤泡淋巴瘤(CCFL)是一种 B 型皮肤淋巴瘤,通常呈惰性病程。头皮 CCFL 定位极为罕见,我们报告了一例新病例,该病例模仿硬膜外血肿,表现为快速进展,皮肤、颅骨、硬脑膜和脑实质侵袭性浸润。
一位 58 岁患者,患有未标记的多形性发育不良综合征,因自限性强直阵挛性癫痫发作继发头部受伤而被收入急诊部。初始 CT 扫描被解释为轻度皮下和硬膜外血肿,最初被认为需要保守治疗。在 4 天内,患者出现进行性神经功能恶化,最终导致昏迷状态,促使进行对比增强脑 MRI。扫描显示了一个从皮下间隙延伸到硬膜下腔的多层实性病变,威胁到覆盖皮肤的完整性并导致脑实质浸润。在紧急神经外科切除后,明确了中央滤泡淋巴瘤的组织学诊断。随后进行了高剂量甲氨蝶呤联合 R-CHOP 方案的聚焦化疗,直到 2 年的最新随访,疾病得到控制。
据我们所知,该病例代表了首例侵犯脑实质的 CCFL,也是第二例扩展到硬脑膜的病例。尽管此类肿瘤通常呈惰性,但在此处报告的侵袭性行为将鉴别诊断扩展为高级别脑膜瘤、肉瘤和转移瘤。预后和适当的辅助治疗需要及时进行手术切除和组织学确认。