Research Article

EWSR1-NFATC2 and FUS-NFATC2 Gene Fusion-Associated Mesenchymal Tumors: Clinicopathologic Correlation and Literature Review

Table 1

Published data of patients with tumors containing EWSR1-NFATC2 fusion.

Literature case nrReferenceAge (y)GenderSiteOriginal diagnosisFollow-up/outcomeTherapy

1[22, 31]39MHumerus RESLT15 mo after surgery lung metResection post-op Chth EuroEwing 99
26 mo after surgery bone met spine
40 mo after surgery 2nd lung met
48 mo after surgery ANED
216MFemur RESLTNDND
321MThigh RESLTNDND
425MFemur RESLTNDND
5[29]32MLower limbMyoepithelial-like “MFH”
of bone 7 y before radiation due to lymphoma
64 mo; ANED (never metastasized)ND
6[28]12MFemurES11 mo; ANED (never metastasized)Pre-op Chth resection
728MHumerusLymphoma4 y local recurrence 4 y lung metastasis “possible”Chth (“standard protocol”)
8[12]42MFemur RNDND
9[30]30MFemur LAggressive osteoblastoma
osteosarcoma (recurrence)
2.5 y post-op local rec 3.5 y ANEDCurettage and bone graft 2.5 y post-op Chth after local recurrence
10[27]24FCalf subcutisExtraskeletal ES12 mo; ANED (never recurrence or metastases)Pre-op Chth Ewing protocol
11[32]NDNDFibulaESLTANED (how long?)ND
12[26]32.7MHumerusESNDND
1312.7FTibiaESNDND
1461.5MCalfESNDND
1523.6MFemurESNDND
16Current study34FFemur RSEF4.5 y skin metastasis 10.5 y lung metastasis 11 y ANEDPre-op Chth EURAMOS protocol resection
1742MTibia LMyoepithelal tumor8.5 y ANEDCurettage resection amputation (due to surgical complications)
1860FAbdomenMyoepithelal tumor or EMC8 mo ANEDResection
19–24[20]NDNDNDES/ESLTNDND
25[23]NDNDNDES/ESLTNDND
26–32[24]NDNDNDES/ESLTNDND

ANED: alive, no evidence of disease; Chth: chemotherapy; EMC: extraskeletal myxoid chondrosarcoma; ES: Ewing sarcoma; ESLT: Ewing sarcoma-like tumor; F: female; L: left; ND: no data; M: male; mo: months; R: right; SEF: sclerosing epithelioid fibrosarcoma; y: years.