Date: 28 Sep 2010
Myxofibrosarcoma: Prognostic Factors and Survival in a Series of Patients Treated at a Single Institution
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.Get Access
First described in 1977, myxofibrosarcoma is one of the most common sarcoma subtypes of the elderly. Until some years ago, myxofibrosarcoma was diagnosed as “myxoid malignant fibrous histiocytoma.” The aim of this retrospective case series analysis was to investigate prognostic factors and the clinical outcome of a cohort of patients with myxofibrosarcoma treated at a single institution.
We reviewed 158 patients with localized myxofibrosarcoma who underwent surgery at the Istituto Nazionale Tumori of Milan, Italy, over 15 years. Local recurrence, distant metastases, and survival were analyzed.
One hundred twenty patients had primary tumors, while 38 patients had locally recurrent tumors. Five-year overall survival was 77%. Tumor size, grade, and margins were statistically significant predictors of survival. Five-year local recurrence and distant metastases rate were 18% and 15%, respectively. Surgical margins were the only statistically significant prognosticator of local relapses. Patients treated with radiotherapy had the same prognosis as nontreated patients, but likely they had worse local presentations. The histological grade correlated with distant recurrences but not with local relapses. The value of adjuvant chemotherapy could not be determined.
Patients with myxofibrosarcoma have a better disease-specific survival than other sarcoma subtypes, but also a higher local relapse rate. This is likely related to the peculiar local growth pattern of these tumors. Adequate surgery should be pursued, while the role of adjuvant therapies need to be investigated.
Angervall L, Kindblom LG, Merck C, et al. Myxofibrosarcoma. A study of 30 cases. Acta Pathol Microbiol Scand. 1977;85A:127–40.
Fletcher CDM, Unni KK, Mertens F, eds. World Health Organization Classification of Tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon, France: IARC Press; 2002. p. 102–3.
Merck C, Angervall L, Kindblom LG, et al. Myxofibrosarcoma. A malignant soft tissue tumor of fibroblastichistiocytic origin. A clinicopathologic and prognostic study of 110 cases using multivariate analysis. Acta Pathol Microbiol Immunol Scand Suppl. 1983;282:1–40.PubMed
Fletcher CDM, Gustafson P, Rydholm A, et al. Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol. 2001;19:3045–50.PubMed
Marubini E, Valsecchi MG. Analysing survival data for clinical trials and observational studies. Chichester, UK: Wiley; 1995.
Frustaci S, Gherlinzoni F, De Paoli A, et al. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol. 2001;19:1238–47.PubMed
- Myxofibrosarcoma: Prognostic Factors and Survival in a Series of Patients Treated at a Single Institution
Annals of Surgical Oncology
Volume 18, Issue 3 , pp 720-725
- Cover Date
- Print ISSN
- Online ISSN
- Additional Links
- Industry Sectors
- Author Affiliations
- 1. Department of Cancer Medicine, Istituto Nazionale Tumori, Milan, Italy
- 2. Unit of Clinical Epidemiology and Trial Organization, Istituto Nazionale Tumori, Milan, Italy
- 6. General Hospital, Alessandria, Italy
- 3. Department of Surgery, Istituto Nazionale Tumori, Milan, Italy
- 4. Department of Pathology, Istituto Nazionale Tumori, Milan, Italy
- 5. Department of Radiotherapy, Istituto Nazionale Tumori, Milan, Italy