Vatavu Octavian, Crisà Francesco Maria, Leocata Filippo, Arienti Virginia, Cenzato Marco, La Camera Alessandro
Gamma Knife Unit, Neurosurgery, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy.
Radiotherapy, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy.
Brain Spine. 2025 Aug 27;5:104396. doi: 10.1016/j.bas.2025.104396. eCollection 2025.
Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine skin malignancy with an increasing incidence. Although it frequently presents with loco-regional metastases, brain metastases (BMs) are exceptionally rare, lacking standardized treatment protocols.
This report details the case of a 75-year-old male diagnosed with MCC following surgical excision of an inguinal mass, with subsequent adjuvant radiotherapy. Four months post-surgery, imaging revealed a pancreatic nodule and two cerebral lesions. The patient underwent Gamma Knife® stereotactic radiosurgery (GK-SRS) followed by adjuvant immunotherapy with avelumab. MRI follow-ups showed a significant reduction of the cerebellar lesion and full remission of the frontal lesion, with stable remission noted at one year. The patient remained asymptomatic and continued immunotherapy without neurological deficits.
A systematic review conducted in parallel included 17 papers representing 20 patients with MCC BMs. Treatment strategies varied, including surgery, radiotherapy, and SRS, sometimes combined with immunotherapy. Analysis revealed that SRS provided high rates of local control, highlighting its value as a primary treatment option. Surgical interventions were typically reserved for symptomatic cases or diagnostic uncertainty. Cases involving leptomeningeal spread correlated with poor outcomes, particularly when not coupled with immunotherapy. The results of analysis support the efficacy of SRS in managing MCC brain metastases, recommending its use as a first-line option when feasible. Immunotherapy appears to enhance disease control, especially in preventing leptomeningeal complications. These data suggest to incorporate radiosurgery and immunotherapy into treatment paradigms for improving prognosis in MCC patients with brain metastases.
默克尔细胞癌(MCC)是一种罕见且侵袭性强的神经内分泌皮肤恶性肿瘤,发病率呈上升趋势。尽管它常出现局部区域转移,但脑转移(BMs)极为罕见,且缺乏标准化的治疗方案。
本报告详细介绍了一名75岁男性的病例,该患者在腹股沟肿块手术切除后被诊断为MCC,随后接受了辅助放疗。术后四个月,影像学检查发现胰腺有一个结节和两个脑部病变。患者接受了伽玛刀®立体定向放射外科治疗(GK-SRS),随后用阿维鲁单抗进行辅助免疫治疗。MRI随访显示小脑病变明显缩小,额叶病变完全缓解,一年时仍处于稳定缓解状态。患者无症状,继续接受免疫治疗,无神经功能缺损。
同时进行的一项系统综述纳入了17篇论文,代表20例MCC脑转移患者。治疗策略各不相同,包括手术、放疗和SRS,有时还联合免疫治疗。分析表明,SRS提供了较高的局部控制率,凸显了其作为主要治疗选择的价值。手术干预通常仅用于有症状的病例或诊断不确定的情况。涉及软脑膜播散的病例预后较差,尤其是在未联合免疫治疗时。分析结果支持SRS在治疗MCC脑转移中的疗效,建议在可行时将其作为一线选择。免疫治疗似乎能增强疾病控制,尤其是在预防软脑膜并发症方面。这些数据表明,应将放射外科和免疫治疗纳入治疗模式,以改善MCC脑转移患者的预后。