DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20163346

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

Pradosh Kumar Sarangi, Sanjay Kumar Nahak, Sasmita Parida, Jayashree Mohanty, Basanta Manjari Swain

Abstract


Multifocal soft tissue sarcoma (STS) is a rare and controversial entity, accounting for about 1% of patients with extremity STS and 4.5% of patients with liposarcoma. Multifocal presentation can occur synchronously or metachronously and is defined as the presence of tumor at two or more anatomically separate sites before the manifestation of disease in sites where sarcomas usually metastasize (e.g., lungs, liver, bone). Myxoid liposarcoma is the predominant histological type in multifocal presentation. This matter is debated as to whether  this entity represent an unusual pattern of metastasis or multiple separate primary tumors as the differentiation between second primary and metastatic lesions has major clinical consequences. Recent literatures based on molecular biologic analysis of tumor clonal heterogeneity suggest metastatic nature. Multifocal myxoid liposarcoma has an aggressive clinical course with frequent recurrences and poor prognosis. Surgery remains the mainstay of treatment with adjuvant chemo and radiotherapy. Herein we are reporting a case of metachronous multifocal myxoid liposarcoma with multiple tumor sites (bilateral breasts, anterior chest wall, anterior abdominal wall, right shoulder area, left thigh etc.) which developed after one year of lumpectomy of myxoid liposarcoma of left breast. A recent review of literature pertaining to its unusual metastatic character, imaging and pathologic features is made.


Keywords


Myxoid liposarcoma, Multifocal, Metastasis, TLS-CHOP fusion, Metachronous

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References


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