Well-differentiated intraosseous osteosarcoma (WDIOS) is a rare tumor and h
istologically similar to parosteal osteosarcoma. This study reports the cli
nicopathological features of five patients with WDIOS tumor seen at the Ins
tituto Nacional de Cancerologia in Mexico City. The patients' ages ranged f
rom 29 to 42 years with a median age of 32 years. Four of the five patients
presented with pain and a mass that had been present for more than 3 years
prior to the diagnosis. Radiologically, four of the lesions were present i
n the long bones and showed a predominantly trabeculated appearance with so
me small iii-defined lyric areas. The bony sclerosis was more prominent wit
hin the medullary cavity than in the soft tissue extension. The majority of
the tumors were large, averaging 21 cm in size. Histologically, the tumors
were characterized by spindle cells with areas of cytologic atypia and pre
sence of mature bone formation. In two patients a high-grade sarcoma compon
ent was found in addition to WDIOS component: in both patients the high-gra
de sarcoma component was identified in the amputation specimens, and in one
of these it was also recognized in the pulmonary metastasis. This study de
monstrated that WDIOS is a slow-growing malignant neoplasm that may show a
histologic progression to dedifferentiation (dedifferentiated osteosarcoma)
and an aggressive behavior with distant metastasis.