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颅内原发性恶性黑色素瘤:病例系列及文献复习。

Primary intracranial malignant melanomas: A case series with literature review.

机构信息

Department of Neurosurgery, The First Medical Center of the Chinese PLA General Hospital, Beijing, China.

Department of Neurology, The Second Medical Center of the Chinese PLA General Hospital, Beijing, China.

出版信息

Medicine (Baltimore). 2024 Nov 1;103(44):e40334. doi: 10.1097/MD.0000000000040334.

Abstract

RATIONALE

There is a high chance of misdiagnosis and limited knowledge regarding therapeutic strategies owing to the rarity of primary intracranial malignant melanoma (PIMM). The objective of the present study was to evaluate the clinical features, treatment modalities, and outcomes of patients with histologically proven PIMM.

PATIENT CONCERNS

Data of 15 patients with PIMM admitted to the Chinese People's Liberation Army General Hospital in a 14-year period between January 2005 and January 2019 were collected. Clinical presentations, pathology, surgical strategies, adjuvant treatment, and prognosis were retrospectively analyzed.

DIAGNOSES

CT showed iso- or high-density lesions in 12 cases (80%). MRI revealed short T1 and slightly short T2 in 14 cases (93.3%).The tumors showed mild or no enhancement on enhanced MRI. The patients were eventually diagnosed with PIMM through pathological examination.

INTERVENTIONS

The treatment modalities included radical resection followed by conventional radiotherapy (RT, n = 12) and subtotal resection followed by stereotactic radiosurgery (n = 3).

OUTCOMES

All 15 patients had either recurrence or metastasis at an average of 14.7 months (range, 6-23 months) after surgery. In total, 14 patients (93.3%) succumbed to disease, with a mean overall survival of 22 months (range, 6-36 months). The median survival time was 23 months. The overall survival rates at 1, 2, and 3 years were 80, 47, and 13%, respectively. Radical resection with RT was associated with longer overall survival (log-rank, P < .05) than subtotal resection followed by stereotactic radiosurgery.

LESSONS

PIMM is an extremely rare tumor with a poor prognosis. Radical resection with RT may result in a longer overall survival rate. Targeted immunotherapy may be a promising treatment option for PIMM.

摘要

背景

由于原发性颅内恶性黑色素瘤(PIMM)罕见,因此误诊的可能性很高,且对治疗策略的了解有限。本研究旨在评估经组织学证实的 PIMM 患者的临床特征、治疗方式和结局。

患者关注

收集了 2005 年 1 月至 2019 年 1 月期间在中国人民解放军总医院就诊的 15 例 PIMM 患者的数据。回顾性分析了临床表现、病理学、手术策略、辅助治疗和预后。

诊断

CT 显示 12 例(80%)为等或高密度病变。MRI 显示 14 例(93.3%)T1 短和略短 T2。增强 MRI 上肿瘤呈轻度或无强化。通过病理检查最终诊断为 PIMM。

干预措施

治疗方式包括根治性切除联合常规放疗(n=12)和次全切除联合立体定向放疗(n=3)。

结果

所有 15 例患者术后平均 14.7 个月(6-23 个月)出现复发或转移。共有 14 例患者(93.3%)死于疾病,平均总生存期为 22 个月(6-36 个月)。中位生存期为 23 个月。1、2 和 3 年的总生存率分别为 80%、47%和 13%。根治性切除联合放疗的总生存率高于次全切除联合立体定向放疗(log-rank,P<0.05)。

结论

PIMM 是一种预后极差的罕见肿瘤。根治性切除联合放疗可能会提高总生存率。靶向免疫治疗可能是 PIMM 的一种有前途的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b569/11537647/6b9acd3c367a/medi-103-e40334-g005.jpg

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