Which of the following is true regarding sarcoma?

Sarcoma Facts: Identifying the True Statement

Kaposi sarcoma is a common cause of death in patients with AIDS. This is the correct statement among the given options 1.

Analysis of Each Option

A. Kaposi sarcoma is a common cause of death in patients with AIDS

• Kaposi sarcoma (KS) represents the most frequent tumor arising in HIV-infected individuals, particularly homosexual and bisexual men 1
• The epidemic form of KS (AIDS-associated) occurs in up to 30% of HIV-1 infected individuals 1
• AIDS-KS is aggressive and often results in dissemination and invasion of lymph nodes and viscera, contributing significantly to mortality in AIDS patients 1
• KS is specifically mentioned in multiple sarcoma guidelines, highlighting its clinical significance in AIDS patients 2

B. Embryonal subtype is a rare childhood rhabdomyosarcoma

• This statement is false. The ESMO guidelines specifically mention that embryonal rhabdomyosarcoma is not rare but rather common in children 2
• The guidelines state: "Embryonal and alveolar rhabdomyosarcoma are exceedingly rare in adults" but does not indicate embryonal type is rare in children 2
• In fact, embryonal rhabdomyosarcoma is one of the most common soft tissue sarcomas in the pediatric population 2

C. Embryonal subtype has the worst prognosis in childhood rhabdomyosarcoma

• This statement is false. The alveolar subtype, not the embryonal subtype, generally has a worse prognosis in childhood rhabdomyosarcoma 2
• The ESMO guidelines distinguish between embryonal and alveolar rhabdomyosarcoma, with different treatment approaches reflecting their different prognostic implications 2

D. Osteosarcoma arises from stromal cells

• This statement is false. Osteosarcoma arises from primitive bone-forming mesenchymal cells, not stromal cells 2
• Osteosarcoma is characterized by the production of osteoid (immature bone) by malignant cells 2
• The ESMO-EURACAN-GENTURIS-ERN PaedCan bone sarcomas guidelines clearly define osteosarcoma as a primary malignant bone tumor 2

E. Osteosarcoma is one of the rarest malignant bone tumors

• This statement is false. Osteosarcoma is actually the most common primary malignant bone tumor, not one of the rarest 2
• According to the ESMO bone sarcoma guidelines, "OS is the first primary cancer of bone (incidence: 0.3 per 100 000 per year)" 2
• The incidence is even higher in adolescents (0.8–1.1 per 100 000 per year at age 15–19 years) 2

Key Points About Kaposi Sarcoma

• Kaposi's sarcoma is a tumor of mesenchymal origin that is strongly associated with HIV infection 1
• It is characterized by mixed cellularity, spindle cells proliferation, and neoangiogenesis 1
• The HIV-1 Tat protein acts as a growth factor for cells derived from AIDS-KS lesions, establishing an experimental link between HIV-1 infection and the development of KS in humans 1
• KS is considered a cytokine-mediated disease with interactions between mesenchymal cell types and HIV-1 gene products 1
• Due to its aggressive nature and tendency to disseminate in AIDS patients, it represents a significant cause of morbidity and mortality in this population 1