The relationship between RMS and HH signaling pathway was first identified in mice heterozygous for Ptch1 as these mutants exhibited a high incidence of ERMS.349 The consistent activation of the HH pathway has been associated with RMS, though there have been relatively few RMS cases in patients with Gorlin syndrome.211 According to the Children’s Oncology Group, 18% of patients with fusion-negative RMS harbor a mutation in HH signaling pathway.350. The gene discussed is PTCH1; the disease is nevoid basal cell carcinoma syndrome.