Division of Hematology-Oncology, Department of Internal Medicine, Chang Gung Memorial Hospital, Keelung, Taiwan; Institute of Medicine, Chung Shan Medical University, Taichung, Taiwan.
Division of Hematology-Oncology, Department of Internal Medicine, Chang Gung Memorial Hospital, Keelung, Taiwan.
J Clin Neurosci. 2022 Jul;101:37-46. doi: 10.1016/j.jocn.2022.03.037. Epub 2022 May 5.
Solitary type primary intracranial malignant melanoma (PIMM) is extremely rare but fatal. The optimal treatment algorithm according to clinical relevance of symptoms and outcomes is unclear. This series emphasized the prognostic factors of solitary PIMM and established the treatment algorithm for this rare disease.
Patients with solitary PIMMs were pathologically verified and treated with neurosurgical tumor resection. All solitary PIMMs recruited at our institute received multidisciplinary team care. We analyzed the clinical findings and prognostic factors.
The study cohort included 10 patients. PIMMs in solitary type impacted middle-aged populations with male predominance in Taiwan. Most patients (80%) presented a single tumor initially. Six patients had progressed to multiplicity after the initial treatment. Rates of tumor bleeding and leptomeningeal metastasis seeding (LS) are high in solitary PIMMs. Patients who had gross-total resection (GTR) had better survival than those who had incomplete resection, with median overall survival (OS) rates of 170.4 months vs. 5.23 months (p = 0.004). Multiplicity, eloquent area involvement, initial tumor bleeding, LS, hydrocephalus, and Karnofsky Performance Score < 80 at diagnosis were associated with negative outcomes in progression-free survival and OS. Adjuvant radiotherapy for patients who had LS and for those who cannot undergo grossly total tumor removal resulted in a good outcome.
GTR demonstrated better outcomes for solitary PIMM. For recurrent tumors, aggressively repeated surgical resection remained beneficial for selected cases. Adjuvant radiotherapy was a treatment option for LS following operation. We proposed a possible treatment algorithm for solitary PIMM.
孤立型原发性颅内恶性黑色素瘤（PIMM）极为罕见但致命。根据症状和结果的临床相关性，最佳治疗方案尚不清楚。本系列强调了孤立型 PIMM 的预后因素，并为这种罕见疾病制定了治疗方案。
经病理证实为孤立型 PIMM 的患者接受神经外科肿瘤切除术。我院所有收治的孤立型 PIMM 患者均接受多学科团队治疗。我们分析了临床发现和预后因素。
本研究队列包括 10 名患者。PIMM 在孤立型中影响中年人群，在台湾以男性为主。大多数患者（80%）最初表现为单个肿瘤。6 例患者在初始治疗后发展为多发性。肿瘤出血和软脑膜转移种植（LS）在孤立型 PIMM 中发生率较高。行肿瘤全切除（GTR）的患者比未行完全切除的患者生存更好，中位总生存期（OS）分别为 170.4 个月和 5.23 个月（p=0.004）。多发性、重要功能区受累、初始肿瘤出血、LS、脑积水和诊断时 Karnofsky 表现状态评分（KPS）<80 与无进展生存期和 OS 的不良预后相关。对于有 LS 且无法进行大体全切除的患者，辅助放疗可获得良好的效果。
GTR 对孤立型 PIMM 显示出更好的结果。对于复发性肿瘤，积极重复手术切除对选定病例仍有益。术后 LS 可行辅助放疗。我们提出了一种治疗孤立型 PIMM 的可能方案。
