neuroplex
New York Association of Neuropathologists
4/21/2009 Case 1: Lipoblastoma/lipoblastomatosis
Presented by: Dr. Rao - University Hospital SUNY HSCB
Clinical History:
An 18-month-old girl presented with clinical evidence of spinal cord compression from intraspinal extension of a large (6.6 x 6.8 x 9.1 cm) paraspinal mass. Intraspinal extension was said to be through widened intervertebral foramina at T12/L1 and possibly at T11/T12 and L1/L2. The spinal cord was noted to be deviated to the right side from T10 to L3. The tumor was biopsied but not resected. The question from the surgeon was whether this was a malignancy so that he could plan aggressive therapy, if indicated. The frozen section diagnosis was not of a malignancy.
Diagnostic Notes:
The final diagnosis and that of a consultant was the same: Lipoblastoma/lipoblastomatosis. Discussion was of the possibility of malignancy versus a variation of a lipoma. The answer is somewhat in the middle in that a lipoblastoma is an infantile lipoma of embryonic adipose tissue that can mature, and complete resection is curative. Incomplete resection allows continued growth, giving a recurrence rate of 9–22%. Only 5% of cases are seen after 7 years of age. Only 5 cases (and some on the Internet) have involved spinal cord. There is usually an aberration of chromosome 8. This child’s tumor was not completely resected and the extent of the tumor remains unknown except from imaging. Therefore, the second part of the final diagnosis is important due to the possibility of local tumor infiltration (lipoblastomatosis). This would be more difficult to resect completely.
