International Journal of Clinical Case Reports 2017, Vol.7, No.17, 73-80
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Figure 9 CT chest: appearance of a basal posteral pleural nodule
There is still controversy whether it is really a multifocal form due to a spontaneous malignant transformation of
cells in different localities or metastases of unknown origin (Blair et al., 1998; Antonescu et al., 2000; Sato et al.,
2004; Fern
á
ndez et al., 2008; Vargas Pierola et al., 2014).
The first case of soft tissue multifocal liposarcoma was described in 1934 (Siegmund, 1934) from the biopsy
results, for which there is little information on the clinical presentation of the disease (Siegmund, 1934; Enzinger
and Winslow, 1962; Blair et al., 1998; Fern
á
ndez et al., 2008; Vargas Pierola et al., 2014).
More recently data from several clinical cases have been published which made it possible to qualify some
controversial aspects of this pathology (Blair et al., 1998; Sato et al., 2004; Fern
á
ndez et al., 2008; Vargas Pierola
et al., 2014). Until the year 2000, less than 50 cases have been described in literature (Blair et al., 1998; Fern
á
ndez
et al., 2008; Vargas Pierola et al., 2014).
Liposarcoma is the most common histological type identified in multifocal soft tissue sarcomas, and of these, the
most common is myxoid liposarcoma (Reszel et al., 1966; Altho, 1992; Vargas Pierola et al., 2014).
Myxoid liposarcoma is also the most common type of liposarcoma and according to the different series, it
constitutes 50% of cases (Siegmund, 1934). There is a rather specific mutation of myxoid liposarcoma: the
translocation t(12,16) (Antonescu et al., 2000).
These tumors tend to relapse locally and develop distant metastases in 1/3 of the cases (Siegmund, 1934; Fern
á
ndez et al., 2008; Vargas Pierola et al., 2014). Among these types of tumors it is common to observe a progression
to a less differentiated histological form: round-cell sarcoma. This histological type is associated with a preserved
prognosis (Chang et al., 1989; Altho, 1992).
According to published data, soft tissue sarcomas have a pulmonary metastasis incidence of 21 percent and an
average survival at 5 years of 75 percent (Vezeridis et al., 1983; Karakousis et al., 1995; Blair et al., 1998; Fern
á
ndez et al., 2008; Vargas Pierola et al., 2014). Patients with diffuse soft tissue sarcoma have a pulmonary
metastasis incidence of 63 percent and a mean survival at 5 years of 36 percent (Blair et al., 1998). This proves
that patients with multifocal sarcoma have a much worse prognosis than those with solitary lesions.
The difficulty lies in identifying whether it was multifocal liposarcoma or metastatic disease: classically in favor
of the multifocal form the involvement of organs and regions that are not usually sites of metastases such as
adipose tissue.
