Background

Soft tissue multifocal sarcoma is a rare clinical entity occurring in 1% of patients with limb soft tissue sarcoma and in 4.5% of patients with liposarcoma. Multimodality is defined as the presence of sarcoma at two or more anatomically separated sites prior to disease manifestation in sites where Soft tissue multifocal sarcoma is most commonly characterized by metastases such as lungs.

The first reported case of multifocal sarcoma dates back to 1934, when Siegmund described a case with multiple malignant lipomatous tumors in a 65-year-old patient in whom the first tumor was noted in the soft tissues of the thigh. Since then, many case studies have been described and the difficulty always lies in the existence of controversy as to whether this entity represents several separate primary tumors or is simply an unusual pattern of metastasis.

Our patient presented multiple synchronous locations and she had as a circumstance of discovery a breast nodule. The relevance of our case is in the clinical presentation of the disease, the particular type of liposarcoma in its multifocal form and the richness of the iconography presented.

1 Observation

Patient KH.H, 34 years old, nulliparous nulliparous, consults for left breast nodules discovered by self-examination with mastodynia.

In mammography, we note the presence of two breasts of comparable volume, fibro-lipomatous density type 2 of Birads. Four dense round masses of regular contours have been found in the left breast (Figure 1).

At the echomammography, several masses of the left breast were found in the form of well circumscribed, hypoechoic masses of great axis parallel to the skin, having a posterior reinforcement, without other detectable anomalies.

These lesions appeared to presumably benign masses of the left breast adenofibromas and the balance was rated ACR 3 on the left and ACR1 on the right.

The patient complained of persistent mastodynia, with a sensation of increased size of nodules palpated a month after echomammography.

In view of the persistence of mastodynia, a mammary micro-biopsy of the masses was performed and concluded a morphological appearance and immunohistochemical profile of a myxoid liposarcoma.

The patient reported subcutaneous masses in the upper and lower limbs, of soft and non-painful consistency that were neglected. An ultrasound of the soft parts was made and concluded to lipomatous masses of well-circumscribed soft parts, without signs of muscular or bone invasion.

Despite the reassuring ultrasound appearance of these masses, an excisional biopsy was made of one of the masses at the level of the forearm and concluded that a malignant tumor of high grade of probably sarcomatous nature recall the appearance of a liposarcoma myxoid.

Thoraco-abdominopelvic CT was performed as part of the myxoid liposarcoma extension assessment and concluded that multiple tissue masses were present in the left breast (Figure 2), left subcutaneous axillary (Figure 3), thoracic parietal (Figure 4), left gluteal, lumbar and intra and retro peritoneal (Figure 5); They were of the same finely heterogeneous nature before and after injection of contrast product and exerting a mass effect on neighboring structures without evidence of obvious invasion.

The patient was lost sight of despite repeated phone calls. She consulted after 5 months for multiple masses of lower and upper limbs with increased volume of the left breast.

On examination: appearance of multiple masses of firm, subcutaneous and painless consistency in the lower and upper limbs, the largest of which sits at the level of the posterior surface of the right thigh and at the level of the internal surface of the left thigh (Figure 6).

An MRI of both thighs cuts was requested axial and coronal; we note the presence of a bulky tumor mass developing at the level of the posterior compartment of the lower end of the right thigh, It is in isosignal T1 with respect to the muscle, in hyper signal T2 with intense enhancement and finely heterogeneous after gadolinium injection, this mass pushes back the superficial femoral vascular axis which remains permeable with no sign of bone infiltration (Figure 7).

The patient was placed on Doxorubicin (adriamycin) monotherapy which is the reference treatment. There was a good clinical response to 5 courses of chemotherapy with decreased size of all clinical targets, we did not opt for intensive chemotherapy. The course of action was to undertake maintenance chemotherapy with (navelbine) monotherapy. We had a good clinical response with decreased size of the left breast nodule with disappearance of the left limb mass.

After 2 months and during this course of chemotherapy, the patient presented an increase in the size of the right forearm mass with pain of the right upper limb radiating to the neck; it was put under moscontin® and a CT control was requested which showed the appearance of other abdominopelvic and parietal lesions (gluteal and right lumbar masses) (Figure 8) and also the appearance of a basal posteral pleural nodule that measures 12 mm with a stable appearance of other lesions (Figure 9).

The progression was marked by the aggravation of pain and the appearance of other tumor lesions in the upper and lower limbs. The patient died in an acute respiratory distress chart most likely related to pulmonary embolism.

2 Discussion

Our patient presented multiple synchronous locations and she had as a circumstance of discovery a breast nodule. The relevance of our case is in the clinical presentation of the disease and in the particular type of liposarcoma in its multifocal form.

Soft Tissue Multifocal Liposarcoma is a rare entity in pathology and accounts for only 1 percent of adult malignancies. Liposarcoma is characterized as multifocal when it appears simultaneously in two or more locations, before extension to areas where it is more frequently metastasized, such as lung, liver and bone.

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; Fernández 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; Fernández 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; Fernández 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; Fernández 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; Fernández 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; Fernández 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.

In addition these localizations are associated with other usual locations of solitary liposarcoma as shown by our case: retro peritoneum, lower extremities, pectoral muscles, at the pelvic level and even at the level of the spermatic cord (Enzinger et al., 1994; Barrena et al., 2001).

The definition of multifocal liposarcoma involves its location in two different sites before pulmonary involvement, which is considered to be metastasis (Blair et al., 1998).

3 Conclusions

Multifocal liposarcoma is a rare disease and has a very poor prognosis compared to solitary liposarcoma. It has polymorphic radiological presentations that can sometimes be confused with a benign tumor in the case of well differentiated liposarcoma.

The recognition of this pathology is necessary not to confuse it with a metastatic disease.

Authors’ contributions

R.H: Editing and supervision, read and approved the final manuscript; D.A: participated in the drafting of the observation, read and approved the final manuscript; B.N: participated in the drafting of the discussion, read and approved the final manuscript; A.A: checking references. All authors read and approved the final manuscript.

Acknowledgments

We thank the radiology department of Ibn El Jazzar Hospital, Kairouan.

Conflict of interest: none.

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