Clinicopathological features of dedifferentiated liposarcomas with meningothelial-like whorls: report of six cases
10.3760/cma.j.issn.0529-5807.2020.02.007
- VernacularTitle: 伴有脑膜上皮细胞样旋涡状特征的去分化脂肪肉瘤六例临床病理学观察
- Author:
Hongjin HUA
1
;
Jing WU
;
Haoran SUN
;
Cheng XU
;
Xiao LI
;
Chong LIU
;
Zhihong ZHANG
;
Qinhe FAN
;
Hai LI
Author Information
1. Department of Pathology, Jiangsu Province Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China
- Publication Type:Journal Article
- Keywords:
Liposarcoma;
Osteogenesis;
Proto-oncogene proteins c-mdm2;
In situ hybridization, fluorescence
- From:
Chinese Journal of Pathology
2020;49(2):139-144
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the histopathologic, immunohistochemical, molecular genetic characteristics of dedifferentiated liposarcomas with meningothelial-like whorls(DDLMW).
Methods:Six cases of DDLMW diagnosed at Jiangsu Province Hospital(the First Affiliated Hospital of Nanjing Medical University) from March 2012 to August 2018 were enrolled. The cases were analyzed by routine HE staining, immunohistochemistry(MDM2, CDK4 and p16) and fluorescent in-situ hybridization(FISH) on MDM2 gene. Related literatures were also reviewed.
Results:Three of the 6 patients were male.The patient ages ranged from 40 to 77 years (mean, 58 years). Four tumors occurred in the retroperitoneum and two in the mediastinum. Histologically, the tumors showed, in addition to foci of well-differentied liposarcoma, characteristic, scattered meningothelial-like concentrical whorls. The whorls were composed of tightly, concentrically arranged, spindle to ovoid cells with mild to mederate cytological atypia. Metaplastic bone was present within or in their immediate vicinity in four case. The tumors cell also showed strong and diffuse immunoreactivity to MDM2, CDK4 and p16, but no immunoreactivity to S-100 protein, SMA, SOX10, EMA, CD21, CD23 or CD35. The Ki-67 labeling indexes were low, while FISH showed high levels of MDM2 amplification in all cases.
Conclusions:DDLMW is a rare morphologic variant of dedifferentiated liposarcoma. The whorls in DDLMW do not represent perineurial or follicular dendritic differentiation. Recognition and familiarity with its existence, as well as combined application of immunohistochemical staining and MDM FISH, are important to avoid confusion with other lesions.
