Myxoid and round cell liposarcoma: a spectrum of myxoid adipocytic neoplasia.

作者: Dei Tos Ap , Orvieto E , Laurino L , Furlanetto A

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摘要: Myxoid and round cell liposarcoma accounts for about 30% to 35% of all liposarcomas and, even if still classified by the World Health Organization (WHO) as 2 distinct subtypes, share both clinical morphologic features. Lesions combining patterns are frequent wide agreement exists in considering high grade counterpart myxoid liposarcoma. Furthermore, same characteristic chromosome change represented most frequently a reciprocal translocation t(12;16)(q13;p11) that fuses CHOP gene with TLS gene. Clinically, tend occur limbs peak incidence ranging between third fifth decade exhibit overall metastatic rate approximately 30%. A peculiar tendency metastasize soft tissue is observed should not be interpreted multicentricity. Microscopically, purely composed hypocellular spindle proliferation set background associated varying number monovacuolated lipoblasts. The helpful clue presence thin-walled capillary network organized plexiform pattern. important variation occurrence hypercellular areas may exhibits an undifferentiated morphology. On basis percentage hypercellularity/round formation, myxoid/round (more than 25% hypercellular/round areas) 75% somewhat arbitrarily recognized. Both recognition quantification represents crucial step evaluation this subtype because hypercellularity appears correlate outcome. In consideration intrinsic difficulty establishing accurately well application different cut off values, it safer consider any amount prognostically relevant. Careful extensive sampling mandatory permit detection smallest hypercellularity. differential diagnosis includes benign lesions, such lipoma, intramuscular myxoma lipoblastoma, malignant ones low myxofibrosarcoma, extraskeletal chondrosarcoma. great variability, immunohistochemistry genetics has proved sorting out more challenging cases.

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