A case of lung metastasis in myxoinflammatory fibroblastic sarcoma: analytical review of one hundred and thirty eight cases

Abstract

Purpose 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 lowgrade 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.

Myxoid cutaneous tumors: a review

“…MIFS is a low‐grade sarcoma with frequent local recurrence if not completely resected . Only very rare cases of lymph node and lung metastasis have been reported . Wide local excision is the treatment of choice if possible to prevent recurrence.…”

Establishment of a spontaneously transformed cell line (JU-PI) from a myxoinflammatory fibroblastic sarcoma

“…MIFS most frequently arises as a painless slow-growing tumor in the distal extremities of adults in their 40s with no obvious gender predominance. [1][2][3] The histology of MIFS may be pleomorphic, which makes initial diagnostics challenging. The tumor consists of spindle cells and epithelioid cells with abundant cytoplasm.…”

“…1,4 High-grade, metastasizing forms of MIFS are rare, reported to represent some 2% of the cases. 3 Cytogenetic studies on MIFS have revealed a typical reciprocal translocation between chromosomes 1 and 10 t (1:10) (p22; q24), leading to juxtapositioning of parts of the TGFBR3 and MGEEA5 genes. 7,8 No fusion product is formed, however, since the genes are transcribed in opposite directions.…”

Aggressive myxoinflammatory fibroblastic sarcoma with multiple site metastases

“…Sakaki et al observed metastasis to a regional lymph node 3 months after resection in one patient and Hassanein et al reported two cases with distant metastases, one to the lung and the other to the skull at 3 months and 5 years, respectively 9 11. Lombardi et al in 2013 reviewed 133 cases from the literature and found four cases presenting with lung metastasis 14. Our case was characterised by an unusual fast progression of tumour presenting as local extension with a soft tissue mass involving the right foot extending to ankle joint viz the tibiotalar and tibiocalcaneal joints as well as metastases to distant bony sites including the vertebrae, rib and lung.…”