title: A case of lung metastasis in myxoinflammatory fibroblastic sarcoma: analytical review of one hundred and thirty eight cases.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Rizzoli Orthopaedic Institute database and literature were searched for patients with MIFS observed from 1997 to 2012. Data were analysed in a new database.
  
  
  RESULTS
  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%).
  
  
  CONCLUSIONS
  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.
authors.affiliation: General and Thoracic Surgery, Istituto Ortopedico Rizzoli, Via Pupilli 1, 40136, Bologna, Italy.
authors.forename: Raffaele
authors.initials: R
authors.lastname: Lombardi
authors.forename: Elio
authors.initials: E
authors.lastname: Jovine
authors.forename: Nicola
authors.initials: N
authors.lastname: Zanini
authors.forename: Maria Cristina
authors.initials: MC
authors.lastname: Salone
authors.forename: Marco
authors.initials: M
authors.lastname: Gambarotti
authors.forename: Alberto
authors.initials: A
authors.lastname: Righi
authors.forename: Alba
authors.initials: A
authors.lastname: Balladelli
authors.forename: Marco
authors.initials: M
authors.lastname: Colangeli
authors.forename: Michele
authors.initials: M
authors.lastname: Rocca
mesh_terms: D000328:Adult; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008168:Lung / Q000000981:diagnostic imaging; D008175:Lung Neoplasms / Q000453:epidemiology* / Q000556:secondary*; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D012189:Retrospective Studies; D012307:Risk Factors; D012509:Sarcoma / Q000453:epidemiology* / Q000556:secondary*; D012983:Soft Tissue Neoplasms / Q000473:pathology*; D014057:Tomography, X-Ray Computed