Soft-tissue sarcoma

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Evidence last reviewed: 16 Feb 2026

Topic last updated: 11 Nov 2025

Soft-tissue sarcoma (STS) is a group of rare solid tumors of connective tissue. More than 50 different histologic subtypes are known.

Most commonly it presents as a soft-tissue swelling, which may or may not be painful. Differential diagnoses that should be considered are lymphoma, metastatic carcinoma, and benign lesions such as lipoma and neuroma.

Some sarcomas are associated with specific gene mutations, in particular chromosomal translocations.

Diagnosis is made by biopsy, which should have a sufficient amount of tissue for accurate pathologic diagnosis.

Pathology should be reviewed by an expert pathologist who has experience in sarcoma histology. Diagnostic laboratory methods may include immunohistochemistry, cytogenetics, and molecular genetic testing.

Treatment is based on stage and histological subtype, with only some subtypes being chemotherapy-sensitive.

Definition

Sarcoma is a group of rare solid tumors originating in mesenchymal (connective tissue) cells and accounting for 1% of all adult malignancies. They can be divided into two broad categories: sarcoma of soft tissues (including fat, muscle, nerve and nerve sheath, blood vessels, and other connective tissue) and sarcoma of bone.[1][2][3][4][5][6][7][8] More than 50 different histologic subtypes of sarcoma of soft tissues are known.[9][10] This topic includes treatments for extremity STS, gastrointestinal stomal tumors (GIST), and retroperitoneal/intra abdominal STS.

For diagnosis and management of Kaposi sarcoma see Kaposi sarcoma.

History and exam

Key diagnostic factors

mass
upper/lower gastrointestinal bleed
neuropathic pain
increased abdominal girth
features of acute abdomen

Full details

Other diagnostic factors

unilateral extremity swelling
weight loss
fatigue
anorexia
abdominal bloating, discomfort, pain

Full details

Risk factors

genetically inherited syndromes
radiation
human herpesvirus-8 (HHV-8) infection
congenital disorders
male sex
lymphedema
history of exposure to chemical carcinogens

Full details

Diagnostic tests

1st tests to order

CT scan of primary tumor
MRI of primary tumor
CT scan chest
biopsy for histology

Full details

Tests to consider

abdominal/pelvic computed tomography
MRI of total spine
fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)
MRI of the central nervous system (CNS)
endoscopy
complete blood count (CBC)
coagulation studies
blood urea nitrogen (BUN)
creatinine
liver function tests (LFTs)
echocardiogram or multigated acquisition (MUGA) scan
ancillary techniques

Full details

Treatment algorithm

ACUTE

extremity: stage I

extremity: stage II resectable

extremity: stage III or select stage IV (any T, N1, M0), resectable

extremity: stage III or select stage IV (any T, N1, M0), unresectable

extremity: stage IV, oligometastases with limited tumor bulk or regional nodes

extremity: stage IV, disseminated metastases

extremity: recurrent

retroperitoneal/intra-abdominal: resectable (primary or recurrent)

retroperitoneal/intra-abdominal: unresectable (primary or recurrent)

retroperitoneal/intra-abdominal: metastatic disease, single organ and limited tumor bulk amenable to local therapy

retroperitoneal/intra-abdominal: disseminated metastases

gastrointestinal stromal tumors: resectable with minimal morbidity

gastrointestinal stromal tumors: resectable with significant morbidity

gastrointestinal stromal tumors: unresectable

gastrointestinal stromal tumors: progressive

gastrointestinal stromal tumors: recurrent or metastatic

Contributors

Authors

James S. Hu, MD, FACP

Associate Professor of Clinical Medicine

USC Norris Comprehensive Cancer Center

Los Angeles

გაფრთხილება:

JSH has been reimbursed for speaking on desmoid tumors and the drug Ogsiveo (nirogacestat).

Sant P. Chawla, MD, FRACP

Director

Sarcoma Oncology Center

Santa Monica

გაფრთხილება:

SPC owns stock in AADi, Cellestia Biotech, CounterPoint, and Immix BioPharma, and has received grants, consulting fees, payments of honoraria, and payment from speakers bureau for Amgen, Roche, GSK, Threshold Pharmaceuticals, CytRx Corporation, Ignyta, Immune Design, TRACON Pharma, Karyopharm Therapeutics, Sarcoma Alliance for Research through Collaboration (SARC), Janssen, Advenchen Laboratories, Bayer, Inhibrx, NKMax, and Thyme.

მადლიერება

Dr James S. Hu and Dr Sant P. Chawla would like to gratefully acknowledge Dr Swati Sikaria, Dr Jonathan C. Trent, Dr Saira Hassan, and Dr David Thomas, previous contributors to this topic.

გაფრთხილება:

SS, JCT and SH each declare that they have no competing interests. DT has received research support from Pfizer, Amgen, and Novartis.

რეცენზენტები

Richard F. Riedel, MD

Professor of Medicine

Duke University

Durham

გაფრთხილება:

RFR owns shares in Limbguard, has received institutional clinical research support from AADi, Adaptimmune, AROG, Ayala, BioAtla, Blueprint, Cogent, Daiichi-Sankyo, Deciphera, GlaxoSmithKline, InhibRx, NanoCarrier, Oncternal, PTC Therapeutics, SARC, SpringWorks, Tracon, and Trillium. He has been a consultant/advisor for AADi, Adaptimmune, Bayer, Blueprint, Boehringer Ingelheim, Daiichi-Sankyo, Deciphera, GlaxoSmithKline, NanoCarrier, SpringWorks and has received funding for travel from Deciphera.

რეცენზენტების განცხადებები

BMJ Best Practice-ის თემების განახლება სხვადასხვა პერიოდულობით ხდება მტკიცებულებებისა და რეკომენდაციების განვითარების შესაბამისად. ქვემოთ ჩამოთვლილმა რეცენზენტებმა თემის არსებობის მანძილზე კონტენტს ერთხელ მაინც გადახედეს.

გაფრთხილება

რეცენზენტების აფილიაციები და გაფრთხილებები მოცემულია გადახედვის მომენტისთვის.

წყაროები

ჩვენი მტკიცებულებებისა და სარედაქციო ჯგუფები თანამშრომლობენ საერთაშორისო ექსპერტებსა და რეცენზენტებთან, რათა უზრუნველვყოთ თქვენი წვდომა კლინიკურად ყველაზე მნიშვნელოვან ინფორმაციაზე.

ძირითადი სტატიები

World Health Organization. WHO classification of tumours: soft tissue and bone tumours. 5th ed (Vol 3). Lyon, France: IARC Press; 2020.

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: soft tissue sarcoma [internet publication].სრული ტექსტი

Gronchi A, Miah AB, Dei Tos AP, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical practice guidelines for diagnosis, treatment and follow-up*. Ann Oncol. 2021 Nov;32(11):1348-65.სრული ტექსტი აბსტრაქტი

Casali PG, Blay JY, Abecassis N, et al. Gastrointestinal stromal tumours: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2022 Jan;33(1):20-33. აბსტრაქტი

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: gastrointestinal stromal tumors (GIST) [internet publication].სრული ტექსტი

გამოყენებული სტატიები

ამ თემაში მოხსენიებული წყაროების სრული სია ხელმისაწვდომია მომხმარებლებისთვის, რომლებსაც აქვთ წვდომა BMJ Best Practice-ის ყველა ნაწილზე.

დიფერენციული დიაგნოზები
- Lipoma
- Gastrointestinal ulcer
- Lymphoma
მეტი დიფერენციული დიაგნოზები
გაიდლაინები
- NCCN clinical practice guidelines in oncology: gastrointestinal stromal tumors (GIST)
- NCCN clinical practice guidelines in oncology: soft tissue sarcoma
მეტი გაიდლაინები
შედით სისტემაში ან გამოიწერეთ BMJ Best Practice

ამ მასალის გამოყენება ექვემდებარება ჩვენს განცხადებას

Soft-tissue sarcoma

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to consider

Treatment algorithm

extremity: stage I

extremity: stage II resectable

extremity: stage III or select stage IV (any T, N1, M0), resectable

extremity: stage III or select stage IV (any T, N1, M0), unresectable

extremity: stage IV, oligometastases with limited tumor bulk or regional nodes

extremity: stage IV, disseminated metastases

extremity: recurrent

retroperitoneal/intra-abdominal: resectable (primary or recurrent)

retroperitoneal/intra-abdominal: unresectable (primary or recurrent)

retroperitoneal/intra-abdominal: metastatic disease, single organ and limited tumor bulk amenable to local therapy

retroperitoneal/intra-abdominal: disseminated metastases

gastrointestinal stromal tumors: resectable with minimal morbidity

gastrointestinal stromal tumors: resectable with significant morbidity

gastrointestinal stromal tumors: unresectable

gastrointestinal stromal tumors: progressive

gastrointestinal stromal tumors: recurrent or metastatic

Contributors

Authors

James S. Hu, MD, FACP

გაფრთხილება:

Sant P. Chawla, MD, FRACP

გაფრთხილება:

მადლიერება

გაფრთხილება:

რეცენზენტები

Richard F. Riedel, MD

გაფრთხილება:

რეცენზენტების განცხადებები

გაფრთხილება

წყაროები

ძირითადი სტატიები

გამოყენებული სტატიები

დიფერენციული დიაგნოზები

გაიდლაინები

შედით სისტემაში ან გამოიწერეთ BMJ Best Practice

შედით სისტემაში ან გამოიწერეთ BMJ Best Practice

შედით სისტემაში BMJ Best Practice-ის მასალებზე სრული წვდომისთვის