Soft Tissue Sarcoma Reference Guide

Patients, families, and their caregivers can use this reference guide to better understand their diagnosis, find a sarcoma center, find related clinical trials, and connect to patient advocacy and support organizations. Over 70 soft tissue subtypes are defined and organized within 10 chapters.

IN THE GUIDE

Sources

WHO Classification of Tumors Editorial Board. Soft tissue and bone tumours [internet], Lyon (France): International Agency for Research on Cancer; 2020 [cited December 29, 2024]. (WHO classification of tumors series, 5th ed.; vol. 3). Available from: https://tumorclassification.iarc.who.int/chapters/33.

Antonescu CR, Bridge JA, Cunha IW, et al. Soft Tissue Tumours. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumours [internet], Lyon (France): International Agency for Research on Cancer; 2020 [cited June 30, 2025]. (WHO classification of tumors series, 5th ed.; vol. 1). Available from: https://tumorclassification.iarc.who.int/chapters/33.

Bridge JA. Undifferentiated Small Round Cell Sarcomas of Bone and Soft Tissue. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumours [internet], Lyon (France): International Agency for Research on Cancer; 2020 [cited June 30, 2025]. (WHO classification of tumors series, 5th ed.; vol. 2). Available from: https://tumorclassification.iarc.who.int/chapters/33.

* 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.

Chapter 1

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

Chapter 2

Desmoid and Other Fibrous tumors

  • Desmoid Tumor
  • Desmoplastic Fibroblastoma
  • Adult Fibrosarcoma
  • Desmoplastic Small Round Cell Tumor (DSRCT)
  • Dermatofibrosarcoma Protuberans (DFSP)
  • Solitary Fibrous Tumor (SFT)
  • 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

Chapter 3

PVNS and Tenosynovial Giant Cell Tumor

Chapter 4

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

Chapter 5

Leiomyosarcoma and Smooth Muscle Sarcomas

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

Chapter 6

Rhabdomyosarcoma and Skeletal Muscle Sarcomas

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

Chapter 7

Gastrointestinal Stromal Tumors (GIST)

Chapter 8

Extraskeletal Osteosarcoma

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

Chapter 9

Malignant Peripheral Nerve Sheath Tumors (MPNST)

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

Chapter 10

Aggressive Soft Tissue SarcomaS of Uncertain Differentiation

  • Epithelioid Sarcoma (ES)
  • Undifferentiated Pleomorphic Sarcoma (UPS)
  • Clear Cell Sarcoma (CCS)
  • Alveolar Soft Part Sarcoma (ASPS)
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