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Rom J Morphol Embryol ; 58 2 : The tumor was biopsied and surgically removed in order to increase the patient's comfort, due to its increased size and aggressive clinical behavior.

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Computed tomography CT revealed metastases in the pleura and mediastinal lymph nodes, so limb-sparing tumor excision followed by palliative care was the best practice for the patient until the fatal outcome. The histological assessment revealed a tumor composed partly of solid sheets of spindled and pleomorphic cells, partly of areas with prominent myxoid matrix and numerous elongated capillaries.

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Mitotic figures are frequent, often atypical, followed by numerous giant cells with abundant eosinophilic elongated cytoplasm, resembling myoid cells often cancer highly aggressive.

Due to the clinical findings combined with the histological profile, the fatal prognosis was expected, though the time period was shorter than predicted, confirming the aggressive nature of the tumor.

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Even if traditionally was considered MFS as a non-metastatic lesion, recent case reports and studies, including our case revealed that this tumors in fact have the potential to be fatal due to metastatic disease.