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.


CONCLUSIONS
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.
Dutta
D
M
Kundu
K
S
Roy
R
S
S Mukhopadhya
D002648:Child; D006258:Head and Neck Neoplasms / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D062689:Lipoblastoma / Q000175:diagnosis* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D010610:Pharyngeal Neoplasms / Q000175:diagnosis* / Q000601:surgery; D014057:Tomography, X-Ray Computed