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Pleomorphic myxoid liposarcoma, first described and defined by Alaggio et al1 ,2 as a neoplasm showing mixed distinctive histological features of conventional myxoid liposarcoma and pleomorphic liposarcoma and usually occurring in young patients, is a very rare and still poorly explored and under-recognised tumour entity. Boland et al3 reported an additional series of these lesions in the mediastinum of young patients with morphological features of a pleomorphic liposarcoma with prominent myxoid changes composed of areas resembling myxoid liposarcoma juxtaposed to myxoid areas containing bizarre pleomorphic lipoblasts. At present, it is still unclear whether this sarcoma represents a high-grade variant of a myxoid liposarcoma, a distinct morphologic myxoid variant of a conventional pleomorphic liposarcoma or an unique form of liposarcoma.
We report the case of a 21-year-old male who presented with a large mass with a maximal diameter of 16 cm arising in the neck. Histology of the resection revealed a myxoid, infiltrative growing tumour consisting of a homogeneous myxoid matrix containing ‘lymphangioma-like’ myxoid pools, a delicate plexiform capillary network and scattered lipoblasts (figure 1 …