Clinicopathological characteristics of myxoid solitary fibrous tumor: analysis of seven cases
10.3760/cma.j.cn112151-20200201-00060
- VernacularTitle:黏液型孤立性纤维性肿瘤七例临床病理特征分析
- Author:
Juanjuan CAI
1
;
Xianglei HE
;
Ming ZHAO
Author Information
1. 浙江省人民医院/杭州医学院附属人民医院病理科 310014
- Keywords:
Soft tissue neoplasms;
Immunohistochemistry;
Diagnosis, differential;
Solitary fibrous tumor;
Myxoid
- From:
Chinese Journal of Pathology
2020;49(9):910-915
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinicopathological characteristics, immunophenotypes, and diagnostic and differential diagnostic features of myxoid solitary fibrous tumor (SFT).Methods:Seven cases of myxoid SFT were collected from the archives of Zhejiang Provincial People′s Hospital from January 2014 to December 2019. The clinical features, histomorphology, immunohistochemistry, molecular genetics and prognosis were analyzed and the relevant literature was reviewed.Results:There were three male and four female patients ranging from 32 to 67 years. Locations included the pleura (three cases), pelvic cavity, vagina, parotid gland, and nasal cavity(one each). Tumor size ranged from 2.7 to 13.5 cm. Histologically, all cases were characterized predominantly by the presence of myxoid stroma comprising 55% to 90% of the tumor (mean 72%). The tumors were composed of predominantly stellated, spindled or ovoid cells disposed haphazardly, in loose fascicles, or in anastomosing strands imparting a microcystic/reticular appearance in a extensively myxoid, richly vascularized stroma. Staghorn-shaped branching vessels and thin strands of collagen were commonly seen between tumors cells amidst the myxoid background. These myxoid areas were punctuated by small cellular areas showing diagnostic features of classical SFT, which were present in all seven cases. Areas showing giant cell angifibroma-like change were noted in 2 cases and focal lipomatous metaplasia was identified in 1 case. Atypical features suggestive of aggressive behavior were present in 2 cases and in one of the cases myxoid SFT with high-grade sarcomatous overgrowth was noted. Immunohistochemically, tumor cells in all cases stained positively for STAT6 and CD34. Polymerase chain reaction technique showed in both the examined cases the characteristic NAB2ex4-STAT6ex2 fusion gene. According to the Demicco′s risk assessment model, four cases were classfied as low, one was classified as moderate and 2 was classified as high. Follow-up information was obtained in 4 cases. One tumor recurred 3 times within 48 months after operation, and the other 3 cases had no tumor recurrence and metastasis.Conclusions:Myxoid SFT represents a rare morphologic variant of SFT with biological behaviors ranging from indolent to aggressive. Myxoid SFT should be included in the differential diagnostic spectrums of soft tissue tumors with significantly myxoid change. Carefully searching for the typical SFT histomorphology with the use of immunohistochemistry and if necessary, molecularly testing for NAB2-STAT6 fusion can help to distinguish myxoid SFT from its many mimickers.
