Primary malignant giant cell tumor of bone with predominant osteosarcoma morphology:a clinicopathological analysis of seven cases

Ting JIANG; Juan ZHOU; Chunyan CHEN; Yanrui PANG; Lan SHEN; Jie CHEN; Zhiyan LIU

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Primary malignant giant cell tumor of bone with predominant osteosarcoma morphology:a clinicopathological analysis of seven cases

VernacularTitle:以骨肉瘤形态为主的原发性恶性骨巨细胞瘤7例临床病理分析
Author: Ting JIANG ^{1
,

2} ; Juan ZHOU ; Chunyan CHEN ; Yanrui PANG ; Lan SHEN ; Jie CHEN ; Zhiyan LIU
Author Information

1. 上海交通大学医学院附属第六人民医院病理科,上海 200233
2. 上海交通大学医学院附属第六人民医院骨与软组织肿瘤综合诊治中心,上海 200233
Keywords: malignant giant cell tumor of bone; osteosarcoma; H3F3A
From: Chinese Journal of Clinical and Experimental Pathology 2024;40(8):807-812
CountryChina
Language:Chinese
Abstract: Purpose To elucidate the clinicopathological characteristics of primary malignant giant cell tumor of bone(PMGCTB)with mainly osteosarcoma-like morphology.Meth-ods Clinicopathologic features of 7 cases of PMGCTB were ret-rospectively analyzed.Results Among 7 patients with PMGCTB,there were 4 females and 3 males,aged between 9 and 66 years(mean age 39.5 years,median age 35 years).The distal femur emerged as the most frequent site to be involved(3/6).The main clinical manifestations included pain and swelling at the original site of the tumor.Radiological findings indicated osteolytic lesions,often combined with sclerotic areas;most ca-ses showed cortical bone destruction and soft tissue masses(5/7).Histologically,the majority of tumors exhibited typical mor-phological features of osteosarcoma with a few or without osteo-clast-like multinucleated giant cells.Positive immunoreaction with H3F3A G34W was confirmed in 6 cases and with H3F3A G34V in 1 case.SATB2 and p63 were positive in all cases,p53 was proved to be wild type,the Ki67 proliferation index ranged approximately from 10％to 50％.H3F3A p.G34W mutation was detected in 6 cases and only 1 case harboring H3F3A p.G34V mutation.Conclusion PMGCTB is exceedingly rare and difficult for accurate diagnosis,especially for those with atypical morphological features.A comprehensive analysis involving ra-diological,immunophenotypic,and molecular detection is neces-sary to rule out other high-grade sarcomas.