A case of lung metastasis in myxoinflammatory fibroblastic sarcoma: analytical review of one hundred and thirty eight cases
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- Lombardi, R., Jovine, E., Zanini, N. et al. International Orthopaedics (SICOT) (2013) 37: 2429. doi:10.1007/s00264-013-2048-5
Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare soft tissue tumour first identified at the end of the 1990s. This study presents our experience and literature reviews focusing on risk of recurrence.
Rizzoli Orthopaedic Institute database and literature were searched for patients with MIFS observed from 1997 to 2012. Data were analysed in a new database.
Five patients underwent surgery at our institute, and 133 cases were retrieved from the literature. Not all clinicopathological data were available: 76/138 were men (55 %), median age was 45 [interquartile range (IQR) 34–56] years, median tumour size was three (IQR two to five) centimetres. Common sites of occurrence were hand (24 %), fingers (23 %) and foot (20 %). Pain was present at diagnosis in 14/82 patients (17 %), with a median duration of seven (IQR three to 12) months. Surgery was performed for a suspected benign tumour in 88 patients (74 %). Resection was incomplete in 45/71 cases (63 %); re-excision was performed in 32/45 (71 %). At a median follow-up of 26 months, 26/118 patients (22 %) developed recurrent disease; median time to recurrence was 15 months (IQR seven to 26). Actuarial relapse-free survival (RFS) at one, three and five years was 93 %, 72 % and 67 %, respectively. At univariate analysis, only symptom duration of six months or less was significantly associated with a worse RFS (p = 0.046). Metastatic disease to lymph nodes and/or lungs was observed in four patients (3 %).
Clinicopathological findings confirm the low-grade nature of MIFS. However, local recurrence occurs, and patients may be affected by aggressive forms with a potential for distant metastases. Follow-up is strongly advised.