Contributed by Henning Leske1, Elisabeth Rushing1, Rene Bernays2
1 Department of Neuropathology University hospital of Zurich, Switzerland
2 Department of Neurosurgery Hirslanden, Switzerland
A 40 year old female presented to the neurosurgery department with a history of new onset, severe, pressure-like headaches. MRI (Fig. 1) revealed multiple mass lesions in the spinal cord, at C4-6 and T3 with infiltration of the neuroforamina. An epidural component was associated with spinal cord compression. In addition, there were lesions in the hip and shoulder, liver and small intestine, as well as right parietal and frontal, dural- based lesions, 3x3x4 and 1.5x1x1cm. Subsequent surgical removal of the dural tumors was complete. The postoperative course was uneventful and the patient described relief from the headaches. The intracranial tumors recurred at 1 and 2 years after the initial surgery.
Histologic examination of the biopsy revealed a pleomorphic, spindle cell neoplasm with a fascicular growth pattern (Fig 2). Occasional whorl-like structures were seen. The closely packed, eosinophilic tumor cells harbored dark, elongated nuclei with few nuclear pseudoinclusions (Fig. 2 insert). In 10 high power fields (hpf)/40x, up to 13 mitotic figures were observed (Fig. 3, arrowheads). The tumor cells were focally positive for the immunostain CD34 (Fig. 4) but negative for EMA (Fig. 5 ) and STAT6 (not shown). In addition, there was strong, diffuse immunolabeling with CD117 (Fig. 6) and Dog1 (Fig. 7). The proliferation marker Mib1 labeled about 25% of the tumor cells (Fig. 8). What is your diagnosis?