Other neoplasms with rhabdoid features possibly that need to be considered before making the diagnosis of RHB include epithelioid angiomyolipoma (HMB-45+ and Melan-A+), malignant melanoma with rhabdoid features (HMB-45+ and Melan-A+) [13], paraganglioma (synaptophysin+, chromogranin+ and a-inhibin-), epithelioid leiomyosarcoma with rhabdoid features (SMA+, desmin+ and S100-) [14], and epithelioid malignant peripheral nerve sheath tumor (a-inhibin-) [15]. The gene discussed is SMN1; the disease is malignant peripheral nerve sheath tumor.