Contributed by Diana Murro Lin, MD1, Aidnag Z. Diaz, MD2, Robert D. Aiken, MD3, Paolo Gattuso, MD1, Sukriti Nag, MD1
Departments of 1Pathology, 2Radiation Oncology, and 3Neuro-oncology, Rush University Medical Center, Chicago, IL, USA.
A 54-year old male presented with left vision loss, headache and left-sided facial pain. On physical examination medial and inferior displacement of the left eye was noted. He had a remote history of intracranial surgeries 17 and 5 years prior to this admission which included a left frontotemporal craniectomy, partial resection of the left anterior temporal lobe and sinonasal surgeries. Magnetic resonance post-contrast images showed enhancing lesions on the left side adjacent to the optic nerve, in the anterior temporal lobe, fronto-ethmoid sinus (Figure 1A), parasellar region, skull base, right dura, nasal cavities, and nasopharynx. A body positron emission tomography scan demonstrated evidence of tumor in the left deep cervical lymph nodes. Eight months later, intradural masses were noted in the cauda equina, thoracic paravertebral region and in the lumbar vertebral bodies. The nasopharyngeal mass was biopsied.
Multiple, irregular, tan, soft tissue fragments measuring 2.4 x 1.9 x 0.4 cm in aggregate were formalin-fixed and processed using standard techniques. Sections stained with H&E showed a highly cellular tumor with cells arranged in sheets and indistinct lobules separated by fibrovascular tissue (Figure 1B). Microvascular proliferation was present (Figure 1C). Tumor cells showed nuclear pleomorphism and mitoses (arrowheads, Figures 1B, 1C) which averaged 4/10 high power fields (HPFs). Few areas of necrosis were also present. Tumor cells did not express cytokeratin (CK8/18) (Figure 1D) but showed strong synaptophysin (Figure 1E), and p53 immunoreactivity (Figure 1F). The Ki-67 labeling index was high (Figure 1G) being 33%. What is your diagnosis?