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Published on: April 22, 2019
Lipoblastoma of the lip
Jochima Cota1, Richa R Borkar2, Jitisha J Savalkar1
1Department of Oral and Maxillofacial Pathology, Goa Dental College and Hospital, Bambolim, Goa, India.
Insights
Lipoblastomas, rare childhood tumors of embryonic fat cells, typically affect young children. This case highlights a lipoblastoma in a 10-year-old, emphasizing the need for careful evaluation of atypical presentations.
Area of Science:
- Pediatric Oncology
- Tumorigenesis
- Histopathology
Background:
- Lipoblastomas are benign tumors originating from embryonic white fat cells, predominantly affecting children under three years old.
- The neck is the most common head and neck site, with rare occurrences in older children.
- Genetic factors, including chromosome 8q11-13 abnormalities and PLAG1 gene involvement, are implicated in lipoblastoma development.
Purpose of the Study:
- To report a rare case of lipoblastoma occurring in the lip of a 10-year-old boy.
- To discuss the atypical immunohistochemical features observed in this case.
- To explore a potential novel pathogenetic mechanism involving CD34+ stem cells.
Main Methods:
- Clinical case presentation and detailed histopathological examination.
- Immunohistochemical analysis to characterize tumor cell markers.
- Review of genetic factors associated with lipoblastomas.
Main Results:
- A lipoblastoma was diagnosed in a 10-year-old male presenting with atypical features.
- Immunohistochemical findings suggested a novel pathway involving cluster of differentiation 34-positive (CD34+) stem cells.
- The case underscores the importance of comprehensive evaluation for atypical lipoblastoma presentations.
Conclusions:
- Lipoblastomas can occur in older children and present with atypical features requiring thorough investigation.
- A potential role for CD34+ stem cells in lipoblastoma pathogenesis is proposed.
- Clinical and histopathological correlation is crucial for accurate diagnosis and understanding of lipoblastoma.
Abstract:
Lipoblastomas are benign tumors arising from embryonic white fatty cells that continue to proliferate and develop during the postnatal period. It commonly affects children 3 years of age or younger, with the neck being the most frequent site in the head and neck region. Only 10% of cases are seen at 10 years or above. The etiopathogenesis of lipoblastomas is linked to polysomy and rearrangement of chromosome 8q11-13 with or without the involvement of the pleomorphic adenoma gene 1 (PLAG1) gene. Here, we report one of the first cases of lipoblastoma of lip in a 10-year-old boy with atypical immunohistochemical features and the need for thorough clinical and histopathological evaluation of cases with atypical findings. The novel pathogenetic mechanism of lipoblastoma from the cluster of differentiation 34-positive (CD34+) stem cells has also been discussed.
