Lipoblastoma

Vellios et al. 1958

Benign, but with a relevant tendency to recur, single or multiple (lipoblastomatosis), up to 5cm large fat tissue tumor, which occurs subcutaneously in early childhood (especially in boys) as well as in the deep soft tissues of the body. The tumours correspond histomorphologically to embryonic fatty tissue.

Infants in the first 3 years of life

Lipoblastoma does not play a role in the dermatological routine, as it is usually a deep-seated, 5.0-15.0 cm soft tissue tumor with mediastinal, abdominal or deep muscular seat. Other localizations are buttocks, the extremities, buttocks and the inguinal scrotal/pulva region.

Lobular, encapsulated tumour consisting of lipoblasts, mature adipocytes and immature spindle or star-shaped cells. No increased mitotic activity. The stroma may have a myxoid or a prominent vascular component. In older tumours there is "maturation" of the tissue with dominance of mature adipocytes. Fusion mutations of the PLAG1 gene with the HAS2 gene and the COL1A2 gene have been demonstrated.

Excision; the tumours are characterised by a relevant tendency to recur

1. Ghosh P et al (2015) Lipoblastoma and lipoblastomatosis: A clinicopathological study of six cases. J Cancer Res Ther 11:1040.
2. Kalaivani Selvi S et al (2016) Lipoblastoma presenting as a rapidly growing paravertebral mass and masquerading as myxoid liposarcoma on fine needle aspiration cytology. Diagn Cytopathol 44:426-429.
3. Kerkeni Y et al (2014) Lipoblastoma in childhood: about 10 cases. Afr J Paediatr Surg 11:32-34.
4. Kumar A et al (2015) Rapidly-growing buccal mass in a 6-month-old infant. Br J Oral Maxillofac Surgery 53: 888-890.
5. Langhans L et al (2015) Lipomatous tumours of the face in infants: diagnosis and treatment. J Plast Surg Hand Surg 9:1-5.
6. Mirkovic J et al (2015) Lipoblastoma-like tumor of the vulva: further characterization in 8 new cases. On J Surg Pathol 39:1290-1295.
7. Val-Bernal JF et al (2016) Intradermal Lipoblastoma-Like Tumor of the Lip in an Adult Woman. Actas Dermosifiliogr 107: 88-90.
8. Vellios F et al (1958) Lipoblastomatosis: a tumor of fetal fat different from hibernoma. Am J Pathol 34: 1149-1159
9. Warren M et al (2016) Undifferentiated myxoid lipoblastoma with PLAG1-HAS2 fusion in an infant; morphologically mimicking primitive myxoid mesenchymal tumor of infancy (PMMTI)-diagnostic importance of cytogenetic and molecular testing and literature review. Cancer gene 209:21-29.
10. Yoshida H et al (2014) Identification of COL3A1 and RAB2A as novel translocation partner genes of PLAG1 in lipoblastoma. Genes Chromosomes Cancer 53:606-611.