Sarcoma – Diagnosis & Treatment
Orthopedic, Pediatrics
Context
- Sarcoma is a broad group of malignancies (there are over 50 histological types) that generally arise de novo from mesenchymal originated tissues (i.e., soft tissue and bone). However in older patients they can arise secondarily in some instances.
- Sarcoma can be generally broken down into soft tissue and bone. The breakdown between these being roughly 80/20.
- Sarcoma is quite rare, and is therefore often missed. It makes up <1% of adult malignancies, but 11% of paediatric malignancies.
- Some of the soft tissue presentations appear similar to other common benign conditions such as lipomas.
- Most sarcomas arise from spontaneous mutations, but there is some association with:
- Neurofibromatosis Type 1 (NF1)
- Li-Fraumeni Syndrome
- Familial Adenomatous Polyposis (FAP)
- Radiation
- Secondary transformation of Paget’s disease of the bone in older patients
- Most common site of metastasis for soft tissue and bone sarcomas is the lungs.
- Clinical starting point is considering sarcoma in the differential based on history and physical findings.
- Clinical end point is contacting oncology if findings on history, physical exam and imaging are suspicious in order to organize diagnostic biopsy, staging and treatment promptly if appropriate.
Clinical Presentation
- Points in history that should raise suspicion:
- slowly growing painful or painless mass/lump
- distal neurovascular compromise
- edema
- size >5cm
- location deep to the muscle fascia
- previous radiation treatment
- The majority of soft tissue sarcomas present in all areas of the body. Rough distribution:
- 46% Thigh, gluteal and groin
- 18% torso
- 13% arms
- 13% retroperitoneum
- 9% head and neck
- Soft tissue sarcomas usually present as enlarging painless lumps/masses on the torso or extremities.
- Osteosarcoma:
- Usually present with a painful lump that may have been present for months.
- Generally not associated with B symptoms, such as malaise, fever, weight loss etc.
- most common location is a metaphyseal area of long bones (from most to least common: distal femur, proximal tibia, proximal humerus, middle and proximal humerus, other bones).
- Occur with highest frequency during times of growth spurt in adolescence.
- lab work generally normal, may have elevated ALP, LDH, ESR, though this is not the case the majority of the time.
Diagnostic Process
- Plain film imaging first diagnostic test if suspicion raised.
- Suspicious lesions on plain films:
- medullary and cortical bone destruction
- permeative/“moth-eaten” appearance
- Soft tissue mass
- aggressive periosteal reaction (indication of irritation)
- Lamellated (onion skin) reaction
- in osteosarcoma “skip lesions” within the same joint or bone may be present
- Up to 12% of patient to have pathologic fractures at diagnosis.
- If suspicious lesions noted on imaging screening imaging of lungs for metastases, as sarcoma mets have predilection for the lungs.
- If suspicious findings on imaging involve oncology immediately in order to arrange for appropriate CT/MRI to determine extent and size as well as diagnostic biopsy which will then guide treatment.
Recommended Treatment
- If suspicion on imaging involve oncology.
- Sarcoma treatments differ substantially depending on histological type.
- Sarcoma is rare. It must be treated by specialist that is familiar with current protocols.
- Treatment generally involves surgical resection and adjuvant therapy.
- Outcomes of osteosarcoma worse for adults >65 years of age.
Quality Of Evidence?
High
We are highly confident that the true effect lies close to that of the estimate of the effect. There is a wide range of studies included in the analyses with no major limitations, there is little variation between studies, and the summary estimate has a narrow confidence interval.
Moderate
We consider that the true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different. There are only a few studies and some have limitations but not major flaws, there are some variations between studies, or the confidence interval of the summary estimate is wide.
Low
When the true effect may be substantially different from the estimate of the effect. The studies have major flaws, there is important variations between studies, of the confidence interval of the summary estimate is very wide.
Justification
Though the information is corroborated by multiple sources the condition is rare. Therefore high volume of cases to base information on is not present.
Related Information
Reference List
Popovich, J., Kashyap, S., & Cassaro, S. (2021). Sarcoma. STATPearls.
Wang, L., Gebhardt, M., & Rainusso, N. (2021, July 14). Osteosarcoma: Epidemiology, pathogenesis, clinical presentation, diagnosis, and histology. Uptodate.
Ryan, C., & Meyer, J. (2021, June 4). Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma. Uptodate. https://www.uptodate.com/contents/clinical-presentation-histopathology-diagnostic-evaluation-and-staging-of-soft-tissue-sarcoma?search=sarcome&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H12227121
Skubitz KM, D’Adamo DR. Sarcoma. Mayo Clin Proc. 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362.
Place, R., Lagoc, A. M., Mayer, T., & Lawlor, C. (2011). Oncology and Hematology Emergencies in Children. In Tintinalli’s Emergency Medicine (7th ed., pp. 935–936). McGraw Hill.
RESOURCE AUTHOR(S)
DISCLAIMER
The purpose of this document is to provide health care professionals with key facts and recommendations for the diagnosis and treatment of patients in the emergency department. This summary was produced by Emergency Care BC (formerly the BC Emergency Medicine Network) and uses the best available knowledge at the time of publication. However, healthcare professionals should continue to use their own judgment and take into consideration context, resources and other relevant factors. Emergency Care BC is not liable for any damages, claims, liabilities, costs or obligations arising from the use of this document including loss or damages arising from any claims made by a third party. Emergency Care BC also assumes no responsibility or liability for changes made to this document without its consent.
Last Updated Jan 18, 2022
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