Lipoblastomatosis of the retropharyngeal space: pathogenesis, presentation, and management, with a focus on head-neck lipoblastoma(toses)

Lipoblastomatosis of the retropharyngeal space: pathogenesis, presentation, and management, with a focus on head-neck lipoblastoma(toses). Objective: Lipoblastoma(toses) are benign neoplasms of embryonic white fat in a state of arrested maturation, and are seldom encountered in the head-neck region. We discuss the clinical details of lipoblastomatosis in the retropharyngeal space of a 9-year-old boy, with an emphasis on the head-neck area, the histopathological maturity of the tumor, and the practical roles of imaging, cytogenetics, and immunostaining in the diagnosis. Methods: Clinical record analysis; literature review. Results: A malnourished child presented with worsening respiratory distress and feeding difficulties. Imaging suggested a large, heterogeneous fibrofatty lesion in the retropharyngeal space. Surgery revealed an otherwise encapsulated mass that densely adhered to the pre-vertebral soft tissue. The findings were consistent with a diagnosis of maturing lipoblastomatosis on histopathology, which was supported by additional immunohistochemistry panel analysis. Conclusion: This is the first report of lipoblastomatosis with the retropharyngeal space as the epicenter of involvement/ origin. Although rare and seldom diagnosed before surgery, lipoblastoma(tosis) may be more common in the head-neck area than thought previously and should be an important differential diagnosis for pediatric fibrolipomatous neoplasms in this anatomic subsite.