1/8/2012 Case 2: Atypical lipomatous tumor

Presented by: Dr. Leroy Sharer - New Jersey Medical School

Clinical History:

28-year-old woman with an intradural extra-axial mass from T4 to T7.  She had a prior resection in 1999 at age 17 at RWJ, when she presented with a fall and progressive lower extremity weakness and sensory changes, with bowel and bladder symptoms.  There was a second fall 2 months later at which time a posterior intradural lesion was seen on CT scan, found to be attached to the pia at surgery.  The present imaging showed a hypodense elongated sausage-shaped lesion.  The lamina were absent due to prior surgery.  Two surgeries were performed, 3 days apart.  At the second surgery, the tumor invaded fat and peripheral nerve roots.

Diagnostic Notes:

Sections showed a paucicellular cystic foamy cell lesion.  Nuclei were large and atypical with prominent nucleoli.  There were intranuclear vacuoles. Work-up included immunostaining for MDM2 which was positive in the tumor nuclei.

The diagnosis was ATYPICAL LIPOMATOUS TUMOR aka “WELL DIFFERNTIATED LIPOSARCOMA, RECURRENT”.  Dr. Rosenblum suggested that CDK4 might have been helpful, and cytogenetics may be useful as well.