A related and even rarer disorder named Maffucci syndrome is a very similar condition that is characterized by the presence of multiple enchondromas with hemangiomas and occasionally lymphangiomas usually near the hands and feet but not limited to the skull, ribs, and spinal bones. This disorder is also sporadic and nonhereditary and usually detected during childhood despite being a congenital condition. Maffucci syndrome has also similarly been linked to IDH1 and IDH2 mutations specifically the IDH1 R132C hotspot mutation. This hotspot mutation is presumed to be responsible for the spindle cell hemangiomas and enchondromas in cases of Maffucci syndrome. Sanger sequencing analysis concluded that exon 4 is the primary location of mutations in IDH1 and IDH2 genes are specifically responsible for hemangiomas. Maffucci syndrome carries a significantly higher risk of malignant transformations like chondrosarcomas but also much more aggressive tumors such as acute lymphocytic leukemia and gastrointestinal and ovarian malignancies.