Metachronous multifocal myxoid liposarcoma involving the gastrointestinal tract. Management and literature review

Abstract

Multifocal soft tissue sarcoma is a rare clinical entity occurring in 1% of patients with extremity soft tissue sarcoma and in 4.5% of patients with liposarcoma. Multifocal disease may arise either synchronously or metachronously and has been associated with poor prognosis. Herein, we have described a rare case of metachronous multifocal myxoid liposarcoma involving the gastrointestinal tract that developed 14 months after the resection of a myxoid buttock liposarcoma. Diagnostic evaluation and management of the patient are discussed along with a review of the relevant literature. We conclude that multifocal myxoid liposarcoma is a rare clinical entity that usually represents metastatic disease with poor prognosis. A thorough imaging and careful physical examination are essential in the preoperative evaluation and postoperative follow-up of patients with myxoid extremity liposarcomas, as these tumors are known to have a tendency to spread toward extrapulmonary sites, frequently without pulmonary metastases.

Multifocal myxoid liposarcoma: a rare and controversial entity-case report with literature review

“…A resectable second primary tumor would indicate an optimal surgical approach combined with (neo) adjuvant chemoradiotherapy with curative intent, whereas in metastatic disease, the choice of treatment, surgery, radiotherapy, or chemotherapy is made with a limited expectation of ultimate cure with guarded prognosis, predicting other metastases in the near future. 1,2 A clonal relationship between two tumors proves their common origin in case of metastases. Conversely, the absence of a clonal relationship would suggest a second primary sarcoma.…”

“…In few cases, a variant chromosomal translocation has been described, t(12;22), resulting in an EWS-CHOP fusion. [1][2][3]7,14 The TLS-CHOP protein is thought to function primarily as an aberrant transcriptional regulator that interferes with adipocyte differentiation. 3…”

“…They found 20 cases of liposarcomas with multifocal presentation from their study of 103 cases of liposarcomas. 1,17 The debate as to whether this entity represents separate primary tumors or is simply an unusual pattern of metastasis from one primary lesion is not fully resolved. This differentiation has important clinical implications.…”

“…Multifocal soft tissue sarcoma (STS) is a rare clinical entity occurring in 1% of patients with extremity soft tissue sarcoma and in 4.5% of patients with liposarcoma. 1,2 Multifocality in STS is defined as the presence of sarcoma at two or more anatomically separate sites before the manifestation of disease in sites where STSs most commonly metastasize such as the lungs. 2,3 The first reported case of multifocal sarcoma dates back to 1934, when Siegmund described a case of multiple primary malignant fatty tumors in a male patient of 65 years in whom the first tumor was noted in the soft tissue of the thigh.…”

“…Recent literatures are in favour of the monoclonal origin of multifocal myxoid liposarcoma, establishing the metastatic nature of distant soft tissue lesions. [1][2][3]13,14 A systemic review was done from searching relevant articles from electronic database of medline and google. The database was searched with the terms "multifocal liposarcoma" and "multifocal myxoid liposarcoma".…”