Primary histiocytic sarcoma of central nervous system: a clinicopathological study of three cases
10.3760/cma.j.issn.0529-5807.2019.06.006
- VernacularTitle: 原发性中枢神经系统组织细胞肉瘤三例临床病理学观察
- Author:
Liwei SHAO
1
;
Xin SONG
;
Lu SUN
;
Qiuping GUI
Author Information
1. Department of Pathology, Chinese People′s Liberation Army General Hospital, Beijing 100853, China
- Publication Type:Journal Article
- Keywords:
Central nervous system neoplasms;
Histiocytic sarcoma;
Immunohistochemistry;
Proto-oncogene proteins B-raf
- From:
Chinese Journal of Pathology
2019;48(6):453-457
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To study the clinicopathological features, differential diagnosis and prognosis of primary histiocytic sarcoma of central nervous system(CNS).
Methods:Three cases of CNS histiocytic sarcoma were collected at Chinese People′s Liberation Army General Hospital from 2005 to 2018. Their clinicopathological characteristics were analyzed, and the related literature reviewed.
Results:The three patients included two females and one male, aged 36, 44, 58 years (median 44 years). MRI showed heterogeneously enhancing lesions which were considered meningioma, high-grade glioma or metastatic carcinoma. Histopathologically there were moderately pleomorphic, mitotically active tumor cells with a loose arrangement, effacing the normal brain tissue. These cells possess abundant eosinophilic cytoplasm, highly atypical nuclei, predominant nucleoli, and hemophagocytosis; multinucleated or spindled forms were also seen, as was background reactive inflammation. The tumor cells were typically positive for CD68, CD163, vimentin and lysozyme, S-100 protein, two of three cases were positive for BRAF V600E,one of three cases was partly positive for CD45, CD45RO, CD4, CD34, and negative for GFAP, Olig-2, CK, EMA, SSTR2, CD99, CD117, MPO, CD1a, Langerin, CD21, CD23, CD35, CD15, CD30, CD38, and CD138. The index of Ki-67 was 30%-75%. Rich reticular fiber network was seen in all cases; BRAF V600E mutation was present in two cases.
Conclusions:CNS histiocytic sarcoma is a rare malignant tumor; histopathologic and immunohistochemical examination are necessary for the diagnosis and to exclude other primary CNS and hematolymphopoietic tumors. Primary CNS histiocytic sarcoma is treated by surgery, chemotherapy and radiation therapy, but the prognosis is poor. Complete resection combined with high dose focused radiotherapy can improve the prognosis.
