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. Author manuscript; available in PMC: 2023 May 31.
Published in final edited form as: Genes Chromosomes Cancer. 2022 Jul 21;62(1):5–16. doi: 10.1002/gcc.23082

Table 2:

Immunohistochemical and molecular findings in RREB1::MRTFB fusion positive extra-glossal neoplasms

No Case/reference Reported as Histology patterns Mitoses/10 hpfs Positive IHC Negative IHC Fusion findings
1 Siegfried et al, 2018 [13] Biphenotypic sarcoma Monomorphic spindle cells fibrosarcoma-like 4 SMA, S100, desmin, myogenin h-caldesmon, SOX10, EMA, p63, MUC4, AE1/AE3, CD34, STAT6, MDM2 RREB1 exon 8/ MRTFB exon 11
2 Makise et al, 2020 [14] Unclassified/extra-glossal EMCMT Round > > spindle <1 S100, GFAP, SMA, EMA, AE1/3, ER Desmin, myogenin, SOX10, HMB45, MUC4, STAT6, CD34, BCOR, NKX2–2 RREB1 exon 8/ MRTFB exon 11
3 Makise et al, 2020 [14] Unclassified/extra-glossal EMCMT Spindle >> round > myxoid <1 S100, GFAP, SMA, EMA, AE1/3, pan-TRK, ER AE1/AE3, desmin, myogenin, CD34, STAT-6, MUC4, SOX10, GLUT1, claudin-1, MDM2, DOG1, SSTR2a, ALK RREB1 exon 8/ MRTFB exon 11
4 Bubola et al (2020) [15] EMCMT EMCMT 0 SMA, S100, CD56, desmin, GFAP SOX10, keratin (AE1/ AE3), chromogranin, calponin RREB1 exon 8/ MRTFB exon 11
5 Mechtersheimer et al, 2021 [16] Biphenotypic sarcoma Monomorphic spindle cells fibrosarcoma-like 1 SMA, S100, EMA, CD34 Desmin, myogenin, GFAP, STAT6, AE1/AE3 RREB1 exon 8/ MRTFB exon 11
6 Current Case 1 Unclassified fibromyxoid neoplasm Fibromyxoid, perineurioma-like whorls 5 CD34, EMA (F+), claudin1 (F+) Pankeratin, STAT6, SMA, desmin, GLUT1, S100, SOX10, GFAP, ERG, MUC4, INI1 retained RREB1 exon 8/ MRTFB exon 11
7 Current Case 2 Unclassified fibromyxoid neoplasm Fibromyxoid, chondromyxoid lobules 0 S100, CD68, EMA (F+) Pankeratin, Brachyury, Claudin1, GFAP, MUC4, CD34, SOX10. INI1 retained RREB1 exon 8/ MRTFB exon 11
8 Current Case 3 Chondromyxoid sinonasal hamartoma Chondromyxoid hamartoma-like 0 S100, CD56 (focal), SATB2 (wk.) Pankeratin, ERG, ALK, Brachyury, MUC4, Pan-Trk, SSTR2A, Desmin, MDM2 RREB1 exon 8/ MRTFB exon 11
9 Current Case 4 Unclassified spindle and round cell neoplasm Monomorphic spindle and round cell, highly cellular 2 Synaptophysin, GFAP, CD56, patchy CD99 and PAX8 MUC4, NUT, Pan-TRK, STAT6, SS18 and ALK, EMA, p63, CK19, TLE1, S100, SMA, SOX10, CD34, CK34BetaE12, desmin, myogenin, Myo-D1, WT1, Chromogranin-A, Calcitonin, TTF1 RREB1 exon 8/ MRTFB exon 11
10 Current Case 5 Low-grade mesenchymal neoplasm with rhabdomyoblastic differentiation and RREB::MRTFB fusion, not RMS Monomorphic ovoid and round cells 1 MyoD1, desmin (focal), myogenin (focal) GFAP, S100, betacatenin, CD34, CD117, CD3, CD19, CD45 RREB1 exon 8/ MRTFB exon 11

EMCMT=ectomesenchymal chondromyxoid tumor; F=focal; IHC=immunohistochemistry; hpf=high power field; RMS=rhabdomyosarcoma.