Myxoid lipoblastoma:a clinicopathological study of four cases / 中华病理学杂志

ABSTRACT

Objective@#To study clinicopathological features,diagnosis and differential diagnosis of myxoid lipoblastoma.@*Methods@#Four cases of myxoid lipoblastoma, from 2010 to 2017 at Wuxi People′s Hospital of Nanjing Medical University, the Affiliated Hospital of Xuzhou Medical University and Binhai People′s Hospital, were studied by clinicopathological analysis, immunohistochemistry and in situ hybridization along with a literature review.@*Results@#The age of the patients ranged from 1 to 6 years. Histologically, all tumors had thin fibrous capsule and irregular lobules separated by fibrous septa. The individual lobules consisted of myxoid stroma,prominent plexiform capillary network and stellate or spindle mesenchymal cells. Lipoblasts (S-100 positive) and mature adipocytes varies among different lobules. FISH revealed PLAG1 disruption in all 4 cases. MDM2 or CHOP alterations were not detected. None of the patients had tumor recurrence upon follow up from 12 to 80 months.@*Conclusions@#Myxoid lipoblastoma is a very rare tumor, usually in the first 5 years of life. The clinical features of myxoid lipoblastoma and lipoblastoma are similar, while myxoid lipoblastoma has prominent myxoid change, a plexiform vascular pattern and rare mature fat cells. The patient age,S-100 positive lipoblasts and cytogenetic alteration are the key diagnostic features.