Response to sunitinib in a patient affected by intraperitoneal metastases from an extraskeletal myxoid chondrosarcoma (EMC) primary of the thigh. (A) Hematoxylin and eosin stain of extraskeletal myxoid chondrosarcoma arising from the thigh; (B) Fluorescence in situ hybridization FISH analysis confirming NR4A3 rearrangement (split signals: centromere, orange; telomere, green, in contrast to normal fused signals); (C) Peritoneal metastases (red circles) treated with sunitinib 37.5 mg/day with complete response (green arrow; no more tumor lesion detectable in (D) maintained over 2 years. After two years of treatment, sunitinib was discontinued with evidence of progression (red arrow) after 8 months from discontinuation, as confirmed by the appearance of a new peritoneal lesion ((E), red circle). A new complete response was achieved after rechallenging sunitinib (green arrow, no tumor lesion in (F)).