Tumores malignos primarios del esqueleto.

Malignant primary tumors of the skeletonPrimary malignant tumors of the skeleton are rare neoplasms whose diagnosis at advanced stages hasimportant implications for treatment. The objective of the present study is to describe their defining clinical,pathologic and radiological features, and the therapeutic options for their management, from the only primaryreference center in the city of Valencia, Carabobo State, over an eight-year period. We reviewed medicalrecords from the hospital files, to assess clinical, radiological, diagnostic and therapeutic aspects. Weidentified 28 cases with an average age of 22 years, and over 60% male. More than 50% were classified asstage III at the time of diagnosis. Tumor or increase of volume, pain and functional limitations were the mostfrequent clinical manifestations, with a maximum of 14 months evolution. 67.85% of the tumors were locatedin the appendicular skeleton with the lower extremities as the preferential site, particularly around the knee(distal femur and proximal tibia). Imaging studies of all the cases identified metastatic disease as the natureof the injury. 89.28% of primary malignant tumors of the skeleton were osteosarcomas. Of the total cases,46.42% initiated treatment with preoperative chemotherapy and 42.84% with surgery, 64.27% of whichcontinued with an irregular adjuvant therapy. 72.14% of surgical procedures for therapeutic purposes wereradical. Over 50% of radical surgeries meant sacrificing the affected limb; in the case of salvage surgery, aTikhoff-Lindberg and scapulectomy was performed. Conclusion: Prolongued development of skeletal tumorsmeans locally-advanced stages, which leads to extensive resection or to an aggressive surgery that mayinvolve sacrificing a limb, which in turn limits the use of more conservative techniques and reconstructionprocedures. It is necessary to inform the public about the defining characteristics of primary malignanttumors of the skeleton, and to educate people to seek early assistance for a timely diagnosis.