Sarcoma

Rare Cancer

Category: 軟部組織ICD-10: C49

Overview

Sarcomas are malignant tumors arising from mesenchymal tissues including bone, muscle, fat, blood vessels, and nerves. Over 50 histological subtypes exist, accounting for about 1% of all adult cancers. They most commonly occur in the extremities, retroperitoneum, and trunk.

Symptoms

A painless lump or swelling in the extremities or trunk is the most common initial presentation. As the tumor enlarges, pain and functional impairment from compression of adjacent structures may develop.

Diagnosis

MRI evaluates the mass characteristics and extent, followed by core needle or incisional biopsy for histological diagnosis. Molecular testing for specific fusion genes (e.g., SYT-SSX) aids in subtype classification.

Treatments

Wide surgical excision with adequate margins is the cornerstone of treatment. High-grade tumors may receive neoadjuvant or adjuvant chemotherapy (doxorubicin plus ifosfamide). Radiation therapy is used pre- or post-operatively for local control.

Latest Research & Approaches

Trabectedin and eribulin have shown efficacy in liposarcoma and leiomyosarcoma. Tazemetostat (EZH2 inhibitor) is approved for INI1-negative epithelioid sarcoma.

Sources & References

NCI - Soft Tissue Sarcoma Treatment
Sarcoma Foundation of America
NCCN Guidelines: Soft Tissue Sarcoma