Soft-tissue sarcoma is a group of rare solid tumours of connective tissue. More than 50 different histological 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 pathological 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.
Sarcoma is a group of rare solid tumours originating in mesenchymal (connective tissue) cells and accounting for 1% of all adult malignancies. They can be divided into 2 broad categories: sarcoma of soft tissues (including fat, muscle, nerve and nerve sheath, blood vessels, and other connective tissue) and sarcoma of bone. More than 50 different histological subtypes of sarcoma of soft tissues are known.
History and exam
Key diagnostic factors
- upper/lower gastrointestinal bleed
- purplish macular-papular lesions
- dysfunctional uterine bleeding
- increased abdominal girth
- history of HIV infection
- features of acute abdomen
- neuropathic pain
Other diagnostic factors
- weight loss
- abdominal bloating, discomfort, pain
- unilateral extremity swelling
- genetically inherited syndromes
- human herpesvirus-8 (HHV-8) infection
- congenital disorders
- history of exposure to chemical carcinogens
1st investigations to order
- CT scan of primary tumour
- MRI of primary tumour
- CT scan chest
- HIV test
Investigations to consider
- ultrasound of primary tumour
- chest x-ray
- positron emission tomography (PET) scan
- biopsy for histology
- full blood count (FBC)
- liver function tests (LFTs)
- echocardiogram or multi-gated acquisition (MUGA) scan
- gene rearrangement testing
extremity: stage I
extremity: stage II-III, resectable
extremity: stage II-III, unresectable
extremity: stage IV, single organ plus limited tumour bulk or regional nodes
extremity: stage IV, disseminated metastases
retroperitoneal/intra-abdominal: unresectable or stage IV
gastrointestinal stromal tumours: localised or potentially resectable
gastrointestinal stromal tumours: unresectable or metastatic
James S. Hu, MD, FACP
Associate Professor of Clinical Medicine
USC Norris Comprehensive Cancer Center
JSH declares that he has no competing interests.
Swati Sikaria, MD
USC Norris Comprehensive Cancer Center
SS declares that she has no competing interests.
Sant P. Chawla, MD, FRACP
Sarcoma Oncology Center
SPC declares that he has no competing interests.
Dr James S. Hu, Dr Swati Sikaria, and Dr Sant P. Chawla would like to gratefully acknowledge Dr Jonathan C. Trent, Dr Saira Hassan, and Dr David Thomas, previous contributors to this topic.
JCT and SH each declare that they have no competing interests. DT has received research support from Pfizer, Amgen, and Novartis.
Alfio Ferlito, MD, DLO, DPath, FRCSEd
Department of Surgical Sciences
Professor and Chairman
University of Udine
AF declares that he has no competing interests.
Adrienne Flanagan, MB BCh FRCPath PhD
Professor of Musculoskeletal Pathology
Institute of Orthopaedics and Musculoskeletal Science
University College London
AF declares that she has no competing interests.
Robert Maki, MB, PhD
Memorial Sloan-Kettering Cancer Center
RM declares that he has no competing interests.
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