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A Case of mistaken identity: Rhabdomyosarcoma development from endothelial progenitor cells

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Article: e1448246 | Received 27 Feb 2018, Accepted 28 Feb 2018, Published online: 16 May 2018

Figures & data

Figure 1. Role for endothelial progenitor reprogramming by Hedgehog in rhabdomyosarcoma oncogenesis. A) Model of endothelial cell and skeletal muscle development from Kinase insert domain receptor positive (KDR+) progenitors in the pharyngeal mesoderm on a Waddington epigenetic landscape. B) Proposed model of fusion-negative rhabdomyosarcoma tumorigenesis. Constitutive activation of Hedgehog signaling (Shh) by expression of constitutively active oncogenic SMOOTHENED (SmoM2) in aP2-Cre expressing endothelial progenitor cells results in their embryonic expansion, reprogramming and myogenic transdifferentiation into fusion-negative rhabdomyosarcoma. Immunostaining for Tomato (Red), Myosin Heavy Chain (Green) and DAPI (Blue). Abbreviations in figure as follows: adipose protein 2 (aP2), T-box 1 (TBX1), Paired-like homeodomain transcription factor 2 (PITX2), Transcription factor 21 (TCF21), rhabdomyosarcoma (RMS). Adapted from.Citation9

Figure 1. Role for endothelial progenitor reprogramming by Hedgehog in rhabdomyosarcoma oncogenesis. A) Model of endothelial cell and skeletal muscle development from Kinase insert domain receptor positive (KDR+) progenitors in the pharyngeal mesoderm on a Waddington epigenetic landscape. B) Proposed model of fusion-negative rhabdomyosarcoma tumorigenesis. Constitutive activation of Hedgehog signaling (Shh) by expression of constitutively active oncogenic SMOOTHENED (SmoM2) in aP2-Cre expressing endothelial progenitor cells results in their embryonic expansion, reprogramming and myogenic transdifferentiation into fusion-negative rhabdomyosarcoma. Immunostaining for Tomato (Red), Myosin Heavy Chain (Green) and DAPI (Blue). Abbreviations in figure as follows: adipose protein 2 (aP2), T-box 1 (TBX1), Paired-like homeodomain transcription factor 2 (PITX2), Transcription factor 21 (TCF21), rhabdomyosarcoma (RMS). Adapted from.Citation9