Soft Tissue Sarcoma Reference Guide

SARC has created the Soft Tissue Sarcoma Subtype Reference Guide to help sarcoma patients find a more efficient and timelier triage, diagnosis, and treatment process. Patients and their families can look up their sarcoma diagnosis and find detailed information and basic clinical information.

This information has been simplified to be more accessible for individuals without a medical background.

Source: “WHO Classification of Tumors Editorial Board: Soft Tissue and Bone Tumors,” Lyon (France): International Agency for Research on Cancer; 2020 [internet December 29, 2024]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumorclassification.iarc.who.int/chapters/33.

IN THE GUIDE

* Less common, benign, and low-grade variations are included because of the diagnostic overlap and challenges of diagnosing and differentiating specific malignant subtypes and benign from low grade malignancies.

Liposarcoma

  • Differentiated (Low-Grade) Liposarcoma (WDLPS) and Atypical Lipomatous Tumor (ALT)
  • High-Grade Dedifferentiated Liposarcoma (DDLPS)
  • Myxoid Liposarcoma (MLPS)
  • Myxoid Pleomorphic Liposarcoma
  • Pleomorphic Liposarcoma
  • Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts (PHAT)
  • PEComa
  • Benign and Low-Grade Myxomatous Soft Tissue Tumors
  • Lipomatous and Fatty Tumors

Desmoid and Other Fibrous tumors

  • Desmoid Tumor
  • Desmoplastic Fibroblastoma
  • Adult Fibrosarcoma
  • Desmoplastic Small Round Cell Tumor (DSRCT)
  • Dermatofibrosarcoma Protuberans (DFSP)
  • Inflammatory Myofibroblastic Tumor (IMT)
  • Low-Grade Myofibroblastic Sarcoma
  • Superficial CD34-Positive Fibroblastic Tumor
  • Myxoinflammatory Fibroblastic Sarcoma
  • Infantile FibroSarcoma
  • Myxofibrosarcoma
  • Low-Grade Fibromyxoid Sarcoma
  • Sclerosing Epithelioid Fibrosarcoma
  • Benign Fibrous Tumors

PVNS and Tenosynovial Giant Cell Tumor

Angiosarcoma and Vascular Sarcomas

  • Angiosarcoma
  • Epithelioid Hemangioendothelioma (EHE)
  • Kaposi Sarcoma (KS)
  • Intimal Sarcoma of the Pulmonary Artery
  • Hemangioendothelioma
  • Pseudomyogenic Hemangioendothelioma
  • Benign and Low-Grade Pericytic Tumors

Leiomyosarcoma and Smooth Muscle Sarcomas

  • Leiomyosarcoma (LMS)
  • Inflammatory Leiomyosarcoma
  • Benign and Low-Grade Smooth Muscle Tumors

Rhabdomyosarcoma and Skeletal Muscle Sarcomas

  • Alveolar Rhabdomyosarcoma (ARMS)
  • Embryonal Rhabdomyosarcoma (ERMS)
  • Pleomorphic Rhabdomyosarcoma
  • Spindle Cell
  • Extrarenal Malignant Rhaboid Tumor
  • Benign and Low-Grade Tumors

Gastrointestinal Stromal Tumors (GIST)

Extraskeletal Osteosarcoma

  • Extraskeletal Osteosarcoma
  • Extraskeletal Soft Tissue Ewing Sarcoma
  • Extraskeletal Myxoid ChondroSarcoma (EMC)
  • Benign and Low-Grade Tumors Soft Tissue Chondroma

Malignant Peripheral Nerve Sheath Tumors (MPNST)

  • Malignant Peripheral Nerve Sheath Tumor (MPNST)
  • Malignant Melanotic Nerve Sheath Tumor (MMNST)
  • Benign and Low-Grade PNST

Aggressive Soft Tissue SarcomaS of Uncertain Differentiation

  • Epithelioid Sarcoma (ES)
  • Undifferentiated Pleomorphic Sarcoma (UPS)
  • Clear Cell Sarcoma of Soft Tissue (CCS)
  • Alveolar Soft Part Sarcoma (ASPS)
Back to top