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.

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.

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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