In a 52-year-old female patient and a 50-year-old patient with a malig
n tumor of the right distal femur (stage IIIb) respectively the left d
istal tibia (stage Ia according to Enneking), the extremely rare prima
ry leiomyosarcoma of the bone was diagnosed. The mortality of the prim
ary leiomyosarcoma of the bone is 50%, the mean survival time after di
agnosis is 3.4 years. Diagnosis of a primary leiomyosarcoma of the bon
e is only possible after a secondary leiomyosarcoma has been excluded
as well as by histopathological including immunohistochemical examinat
ion. The operative procedure is the therapy of choice. A neoadjuvant a
nd/or adjuvant radio- or chemotherapy can be useful.