soft tissue sarcoma (STS)
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Epidemiology
Pathology
histologic grade (number of mitoses, degree of cellularity & nuclear atypia, presence of necrosis) reflects metastatic potential more accurately than specific cell type
Microscopic pathology
(classification):
- alveolar soft part sarcoma
- desmoplastic small round cell tumor
- epithelioid sarcoma
- clear cell sarcoma
- chondrosarcoma, extraskeletal
- osteosarcoma, extraskeletal
- gastrointestinal stromal tumor
- Ewing's sarcoma/primitive neuroectodermal tumor
- fibrosarcoma
- leiomyosarcoma
- liposarcoma
- malignant fibrous histiocytoma
- malignant hemangiopericytoma
- malignant peripheral nerve sheath tumor
- rhabdomyosarcoma
- synovial sarcoma
- sarcoma NOS (not otherwise specified)
Genetics
- amplification of MDM2
Radiology
Staging
TNM classification/grading
- primary tumor (T)
- TX: primary tumor cannot be assessed
- T0: no evidence of primary tumor
- T1: tumor ~ 5cm in greatest dimension
- T1a: superficial tumor
- T1b: deep tumor
- T2: tumor > 5cm in greatest dimension
- T2a: superficial tumor
- T2b: deep tumor
- regional lymph nodes (N)
- NX: regional lymph nodes cannot be assessed
- N0: no regional lymph node metastases
- N1: regional lymph node metastasis
* regional lymph nodes metastasis is rare and cases NX can be considered N0
- distant metastases (M)
- MX: distant metastasis cannot be assessed
- M0: no distant metastasis
- M1: distant metastasis
histopathologic grading (G)
GX: grade cannot be assessed
G1: well differentiated
G2: moderately differentiated
G3: poorly differentiated
G4: poorly differentiated or undifferentiated
| stage | T | N | M | G | G | Risk |
|---|---|---|---|---|---|---|
| stage I | T1a,1b,2a,2b | N0 | M0 | G1-2 | G1 | low |
| stage II | T1a,1b,2a | N0 | M0 | G3-4 | G2-3 | high |
| stage III | T2b | N0 | M0 | G3-4 | G2-3 | high |
| stage IV | T_ | N1 | M0 | G_ | G_ | low or high |
| T_ | N0 | M1 | G_ | G_ | low or high |
Management
- surgery:
- goal: functional limb preservation[3]
- amputation may be necessary in some patients
- re-resection is preferred for patients with positive surgical margins
- external-beam RT (EBRT) with intensity-modulated RT (IMRT) &/or proton therapy
- adjunct to surgical resection
- not a substitute for suboptimal surgical resection[3]
- preoperative RT: 50 Gy in 1.8 to 2.0 Gy per fraction
- EBRT boost doses of 16-18 Gy for microscopic residual disease & 20-26 Gy for macroscopic residual disease.[3]
- brachytherapy boosts delivered several days after surgery, through catheters placed at surgery
- doses of 14-26 Gy, based on surgical margin status
- chemotherapy: anthracyclines 1st line[4]
- doxorubicin + ifosfamide
- doxorubicin + olaratumab is an option
- imatinib in patients with dermatofibrosarcoma protuberans
- trabectedin (2nd line)
- pazopanib is an option in non-adipogenic STS.
- eribulin is an option in patients with liposarcomas & leiomyosarcomas
- dacarbazine/gemcitabine or docetaxel/gemcitabine is an option in doxorubicin-pretreated patients
- regorafenib is an option in doxorubicin-pretreated advanced, non-adipogenic STS patients[4]
More general terms
More specific terms
- alveolar soft part sarcoma
- clear cell sarcoma
- desmoplastic small round cell tumor (DSRCT)
- Ewing's sarcoma
- gastrointestinal stromal tumor (GIST)
- hemangiosarcoma; angiosarcoma
- Kaposi's sarcoma
- leiomyosarcoma
- liposarcoma
- lymphangiosarcoma
- malignant fibroblastic/myofibroblastic neoplasm
- malignant fibrous histiocytoma (MFH, angiomatoid fibrous histiocytoma)
- malignant peripheral nerve sheath tumor (MPNST)
- rhabdomyosarcoma (Sarcoma Botryoides)
- synovial sarcoma
References
- ↑ WHO Classification Tumours of Soft Tissue and Bone Fletcher, Unni & Mertens Eds. IARC Press 2002
- ↑ AJCC Cancer Staging Manual 6th ed. Springer 2002
- ↑ 3.0 3.1 3.2 3.3 3.4 Von Mehren M, Randall RL, Benjamin RS, et al. Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2018 May;16(5):536-563. <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/29752328 <Internet> http://www.jnccn.org/content/16/5/536.long
- ↑ 4.0 4.1 4.2 Casali PG, Abecassis N, Bauer S, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. May 28, 2018. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29846498 https://academic.oup.com/annonc/advance-article/doi/10.1093/annonc/mdy096/5004451
- ↑ National Cancer Institure Soft Tissue Sarcoma - Health Professional version https://www.cancer.gov/types/soft-tissue-sarcoma/hp