In previous studies, FANCM, TEX14, NR5A1, and WT1 were implicated as monogenic causes of SCOS, and NANOS2, PLK4, WNK3, and FANCA were reported in a single study and require additional cases to establish a monogenic causative link to SCOS [15]. The gene discussed is NANOS2; the disease is small cell osteogenic sarcoma.