Lipoblastoma

Key points

Benign fat tissue tumours, arising from embryonic white fatty cells

Patients usually <3 years

Lipoblastoma (LB) is typically focal, well-circumscribed, and often mimics a lipoma

Lipoblastomatosis (LBS) is diffuse, infiltrative, and has a higher recurrence rate (14-25%)

PLAG1 gene rearrangement is found in most cases

Associated Conditions:

Beckwith-Wiedemann Syndrome (BWS) and Familial Adenomatous Polyposis (FAP)

Sacrococcygeal LB may be associated with conditions like Dravet's syndrome and can mimic sacrococcygeal teratoma

CT/MRI can often underestimate the size or nature of the tumour, leading to misdiagnosis.

Histology:

LB exhibits a spectrum of adipose tissue differentiation, with features ranging from immature to mature fat cells

Operation:

Radical surgery may not always be necessary for LB due to its benign nature

Local recurrences however are relatively common

Indications for surgery include uncertainty about the diagnosis, rapid growth, local symptoms, or cosmetic concerns

LBS poses surgical challenges due to poorly defined boundaries and sometimes proximity to vital structures, requiring careful planning to avoid mutilating surgery

References

Spătaru RI, Cîrstoveanu C, Iozsa DA, Enculescu A, Tomescu LF, Șerban D. Lipoblastoma: Diagnosis and surgical considerations. Exp Ther Med. 2021 Aug;22(2):903. doi: 10.3892/etm.2021.10335. Epub 2021 Jun 24. PMID: 34257716; PMCID: PMC8243331.

Gisselsson D, Hibbard MK, Dal Cin P, Sciot R, Hsi BL, Kozakewich HP, Fletcher JA. PLAG1 alterations in lipoblastoma: involvement in varied mesenchymal cell types and evidence for alternative oncogenic mechanisms. Am J Pathol. 2001 Sep;159(3):955-62. doi: 10.1016/S0002-9440(10)61771-3. PMID: 11549588; PMCID: PMC1850475.