Sarcoma

Malignant bone tumors. Incidence is 1:100,000; 3000 new cases/y in USA, with bimodal age distribution (first peak during adolescence, second peak in older adulthood).

Soft tissue sarcomas: Incidence is 1:100,000 for ages <20 y and 7:100,000 for ages ≥20 y; 12,000 new cases/y in USA; mean age at diagnosis is 58 y.

Prevalence equal in both genders and all races except Ewing sarcoma (high Caucasian predominance).

Overall: 15% of cancers in children age <20 y.

Morbidity and mortality related to surgical procedure.

Metastatic vital organ involvement, especially lungs and liver.

Mass effect, direct compression of organs and vascular structures.

Adriamycin-induced cardiotoxicity (global LV hypokinesis)

Mitomycin-induced acute pulm toxicity, pulm fibrosis, ARDS with increased FiO ₂

Immunosuppression, hemorrhagic cystitis, renal failure induced by chemotherapeutic agents

Heterogeneous group of malignant tumors of connective tissue derived from the embryonic mesoderm.

Two most common sarcoma forms: Malignant bone tumors and soft tissue sarcomas.

Malignant bone tumors such as osteosarcomas and Ewing sarcomas can be found throughout bones and cartilage.

Soft tissue sarcomas often arise from muscles, joints, fat, nerves, deep skin tissues, and blood vessels.

Can spread aggressively by local invasion and early hematogenous spread, especially to lungs.

Genetic factors, high-dose radiation, carcinogens (dibenzanthracene, methylcholanthrene), and some viral infections (Moloney sarcoma virus, human herpes virus 8) may predispose pts to sarcoma.

von Recklinghausen’s disease: 10–12% develop neurofibrosarcomas.

Li-Fraumeni syndrome: Strong association with sarcomas.

Retinoblastoma survivors have much higher incidence of osteosarcomas.

Paget’s disease: 0.9% develop osteosarcoma.

Kaposi sarcoma strongly associated with human herpes virus 8 in immunocompromised pts.