Ewing's sarcoma natural history
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2];Assistant Editor(s)-In-Chief: Michael Maddaleni, B.S.
Overview
Common complications of Ewing sarcoma include pathologic fracture and metastasis. Pretreatment factors that influence outcome of the Ewing sarcoma are site of the tumor, size of the tumor, serum LDH levels, and site of metastasis. After administration of preoperative chemotherapy, patients with minimal or no residual viable tumor have a significantly better prognosis than do patients with larger amounts of viable tumor.
Complications
- The most frequent complications of Ewing sarcoma are pathologic fracture and the development of metastatic disease.
- Approximately 25% of patients with Ewing sarcoma have metastatic disease at the time of diagnosis.
Prognosis
- Out of all primary musculoskeletal tumors, Ewing's Sarcoma has maintained the form with the most unfavorable long term prognosis.[1] In fact, prior to multi-drug chemotherapy, the survival rate was less than 10%. Now that there have been many options developed, such as chemotherapy, surgery, and irradiation, long term survival has increased to greater than 50% in most clinical centers.[1]
- Staging attempts to distinguish patients with localized from those with metastatic disease. Most commonly, metastases occur in the chest, bone and/or bone marrow. Less common sites include the central nervous system and lymph nodes.
- Survival for localized disease is 65-70% when treated with chemotherapy. Long term survival for metastatic disease can be less than 10% but some sources state it is 25-30%.
The two major types of prognostic factors for patients with Ewing sarcoma are grouped as follows:[2]
- Pretreatment factors.
- Response to initial therapy factors.
Pretreatment factors
Site of tumor
- Patients with Ewing sarcoma in the distal extremities have the best prognosis.
- Patients with Ewing sarcoma in the proximal extremities have an intermediate prognosis, followed by patients with central or pelvic sites.
Tumor size or volume
- Tumor size or volume has been shown to be an important prognostic factor in most studies.
- Cutoffs of a volume of 100 mL or 200 mL and/or single dimension greater than 8 cm are used to define larger tumors.
- Larger tumors tend to occur in unfavorable sites.
Age
- Infants and younger patients have a better prognosis than do patients aged 15 years and older.
- Early death was more common in infants, but the overall survival (OS) did not differ significantly from that of older patients.
Gender
- Girls with Ewing sarcoma have a better prognosis than do boys with Ewing sarcoma.
Serum LDH
- Increased serum LDH levels before treatment are associated with poor prognosis.
- Increased LDH levels are also correlated with large primary tumors and metastatic disease.
Metastases
- Any metastatic disease defined by standard imaging techniques or bone marrow aspirate/biopsy by morphology is an adverse prognostic factor.
- The presence or absence of metastatic disease is the single most powerful predictor of outcome.
- Patients with metastatic disease confined to the lung have a better prognosis than do patients with extra pulmonary metastatic sites.
- The number of pulmonary lesions does not seem to correlate with outcome, but patients with unilateral lung involvement do better than patients with bilateral lung involvement.
- Patients with metastasis to only bone seem to have a better outcome than do patients with metastases to both bone and lung.
- Based on an analysis from the SEER database, regional lymph node involvement in patients is associated with an inferior overall outcome when compared with patients without regional lymph node involvement.
Previous treatment for cancer
- Patients with Ewing sarcoma as a second malignant neoplasm were older (secondary Ewing sarcoma, mean age of 47.8 years; primary Ewing sarcoma, mean age of 22.5 years), more likely to have a primary tumor in an axial or extraskeletal site, and had a worse prognosis (5-year OS for patients with secondary Ewing sarcoma, 43.5%; patients with primary Ewing sarcoma, 64.2%).
Detectable fusion transcripts in morphologically normal marrow
- Reverse transcriptase polymerase chain reaction can be used to detect fusion transcripts in bone marrow. Fusion transcript detection in marrow or peripheral blood was associated with an increased risk of relapse.
Other biological factors
- Over expression of the p53 protein, Ki67 protein, and loss of 16q may be adverse prognostic factors.
- High expression of microsomal glutathione S-transferase, an enzyme associated with resistance to doxorubicin, is associated with inferior outcome for Ewing sarcoma.
Response to initial therapy factors
- Multiple studies have shown that patients with minimal or no residual viable tumor after presurgical chemotherapy have a significantly better prognosis than do patients with larger amounts of viable tumor.
References
- ↑ 1.0 1.1 Iwamoto Y (2007). "Diagnosis and treatment of Ewing's sarcoma". Japanese Journal of Clinical Oncology. 37 (2): 79–89. doi:10.1093/jjco/hyl142. PMID 17272319. Retrieved 2011-12-09. Unknown parameter
|month=ignored (help) - ↑ Ewing's sarcoma. National cancer institute. http://www.cancer.gov/types/bone/hp/ewing-treatment-pdq#section/_1