Moez Hamdani / Oussama Belkacem
Librería Samer Atenea
Kálamo Books
Librería Elías (Asturias)
Librería Kolima (Madrid)
Librería Proteo (Málaga)
Maffucci syndrome is characterized by the combination of multiple enchondromatosis and cutaneous hemangiomatosis, with a high risk of malignant sarcomatous transformation, the most common and most serious of which remains chondrosarcoma. We report the case of a 31-year-old female patient with a history of Maffucci syndrome who presented with a massive mass on her right leg; imaging revealed an osteolytic tumor causing complete destruction of the knee. The diagnosis of chondrosarcoma arising from multiple enchondromatosis was established. A mid-thigh amputation of the right lower limb was decided upon. On pathological examination, the tumor measured 40 cm along its longest axis; it extensively infiltrated the bones of the leg and the lower end of the femur, causing complete destruction of the knee and extending into the adjacent soft tissues.The risk of sarcomatous degeneration of bone lesions in Maffucci syndrome ranges from 15 to 40 percent, depending on the study series. Histological confirmation via biopsy can be difficult, which may delay treatment.
