Soft-tissue sarcoma 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.
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 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 histologic subtypes of sarcoma of soft tissues are known.
History and exam
Key diagnostic factors
- upper/lower gastrointestinal bleed
- purplish macular-papular lesions
- abnormal 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 tumor
- MRI of primary tumor
- CT scan chest
- biopsy for histology
Investigations to consider
- HIV test
- ultrasound of primary tumor
- chest x-ray
- positron emission tomography (PET) scan
- complete blood count (CBC)
- blood urea nitrogen (BUN)
- liver function tests (LFTs)
- echocardiogram or multigated acquisition (MUGA) scan
- gene rearrangement testing
extremity: stage I
extremity: stage II-III, resectable
extremity: stage II-III, unresectable
extremity: stage IV, single organ + limited tumor bulk or regional nodes
extremity: stage IV, disseminated metastases
retroperitoneal/intra-abdominal: unresectable or stage IV
gastrointestinal stromal tumors: resectable with minimal morbidity
gastrointestinal stromal tumors: resectable with significant morbidity
gastrointestinal stromal tumors: unresectable, progressive, or metastatic with imatinib-sensitive mutation
gastrointestinal stromal tumors: unresectable, progressive, or metastatic with imatinib-insensitive mutation
- Gastrointestinal ulcer
- NCCN clinical practice guidelines in oncology: gastrointestinal stromal tumors (GIST)
- NCCN clinical practice guidelines in oncology: soft tissue sarcoma
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