Lipoblastoma
| Classification according to ICD-10 | |
|---|---|
| D17 | Benign new formation of adipose tissue |
| ICD-10 online (WHO version 2019) | |
| Classification according to ICD-O-3 | |
|---|---|
| 8881/0 | Lipoblastoma |
| 8881/0 | Lipoblastomatosis |
| ICD-O-3 first revision online | |
The Lipoblastom is a circumscribed, benign tumor of childhood, the morphological fetal adipose tissue is similar. It was first described in 1926 by the German pathologist Rudolf Jaffé . A distinction must be made between a diffusely spreading form of the tumor, which is known as lipoblastomatosis .
Epidemiology
Lipoblastomas usually appear in the first three years of life and can occasionally be present at birth. Male individuals are about three times more likely to be affected.
morphology
Macroscopically, lipoblastomas are usually small (usually 2–5 cm in size), well-circumscribed, lobed, adipose-like tumors with gelatinous areas of tissue. Histologically, they are composed of mature and immature forms of fat cells, with the latter representing lipoblasts of different developmental stages. Connective tissue strands create a lobular (lobed) tissue architecture.
Clinical symptoms
Lipoblastomas are often clinically noticeable as slowly growing nodules within the subcutaneous fat tissue of the extremities, but can in principle occur in many other locations ( mediastinum , retroperitoneum , trunk, head and neck, lungs, heart, etc.) and cause specific symptoms depending on the location .
diagnosis
Imaging diagnostic methods such as computed tomography and magnetic resonance tomography can contribute to diagnosis and treatment planning, but do not allow reliable differentiation between lipomas and liposarcomas , which is why a definitive diagnosis can only be made by histological examination of the tumor tissue.
therapy
The method of choice is complete surgical removal of the tumor.
forecast
Lipoblastomas are completely benign lesions that neither transform into malignant tumors nor metastasize , but can occasionally recur .
literature
- R. Sciot, N. Mandahl: Lipoblastoma / Lipoblastomatosis. In: Pathology and Genetics of Tumors of Soft Tissue and Bone. IARCPress, Lyon 2002.
