Shah Darsh S, Sharma Himanshu, Patel Prem, Shetty Arya, English Collin William, Goodman J Clay, Viswanathan Ashwin, Patel Akash J
Department of Neurosurgery, Dell Medical School, Austin.
Jan and Dan Duncan Neurological Research Institute, Texas Children's Hospital.
Surg Neurol Int. 2022 Sep 2;13:395. doi: 10.25259/SNI_513_2022. eCollection 2022.
Liponeurocytomas are rare neurocytic neoplasms that most often arise in the posterior fossa and affect individuals in the third and fifth decades of life. Most reported cases of this unique tumor in the literature have described a favorable clinical prognosis without recurrence. However, increasing reports of recurrent cases prompted the World Health Organization, in 2016, to recategorize the tumor from Grade I to the less favorable Grade II classification. We conducted a systematic review to identify recurrent cases of this unique tumor and to summarize differences between the primary and recurrent cases of liponeurocytoma.
A systematic review exploring recurrent liponeurocytoma cases was conducted by searching the PubMed, Google Scholar, and Scopus databases for articles in English. Abstracts from articles were read and selected for full-text review according to a priori criteria. Relevant full-text articles were analyzed for symptoms, imaging, location, histological, pathological, treatment, and recurrence-free time between the primary and recurrent cases.
Of 4392 articles, 15 articles accounting for 18 patients were included (level of evidence: IV) in the study. Recurrence-free time decreased from an average of 82 months between the primary tumor resection to first recurrence to 31.3 months between the first and second recurrence. Recurrent tumors demonstrated increased pleomorphic neural cells, necrosis, vascular proliferation, and MIB-1 index when compared to the primary tumor. Several cases also demonstrated decreased lipidizing components when compared to the primary tumor, further indicating increased dedifferentiation. The primary treatment for this tumor was surgical resection with occasional adjunctive radiotherapy.
Recurrent cases of liponeurocytoma have features of increased malignant proliferation compared to the primary cases. The standard treatment for these primary and recurrent tumors is gross total resection. The role of adjunctive radiotherapy remains a matter of debate.
脂肪神经细胞瘤是一种罕见的神经细胞瘤，最常发生于后颅窝，好发于30至50岁人群。文献中报道的大多数此类独特肿瘤病例的临床预后良好，无复发。然而，复发病例报告的增加促使世界卫生组织在2016年将该肿瘤从I级重新分类为预后较差的II级。我们进行了一项系统综述，以确定这种独特肿瘤的复发病例，并总结脂肪神经细胞瘤原发和复发病例之间的差异。
通过在PubMed、谷歌学术和Scopus数据库中搜索英文文章，对复发性脂肪神经细胞瘤病例进行系统综述。根据预先设定的标准阅读文章摘要并选择进行全文审查。对相关全文文章进行分析，以了解原发和复发病例之间的症状、影像学、位置、组织学、病理学、治疗及无复发生存时间。
在4392篇文章中，15篇文章纳入了本研究，共18例患者（证据级别：IV）。无复发生存时间从原发肿瘤切除至首次复发的平均82个月降至首次复发至第二次复发的31.3个月。与原发肿瘤相比，复发性肿瘤显示多形性神经细胞增加、坏死、血管增生和MIB-1指数升高。与原发肿瘤相比，一些病例还显示脂质化成分减少，进一步表明去分化增加。该肿瘤的主要治疗方法是手术切除，偶尔辅助放疗。
与原发病例相比，脂肪神经细胞瘤复发病例具有恶性增殖增加的特征。这些原发和复发性肿瘤的标准治疗方法是全切除。辅助放疗的作用仍存在争议。
