Contributed by Pier Luigi Di Patre*, Ildiko Szalay*, and Jacqueline Delavelle+
Departments of Pathology*, and Radiology+, University Hospitals, Geneva, Switzerland
Published on line in February 2004
CLINICAL HISTORY AND NEUROIMAGING:
A previously healthy man in his 40s came to clinical attention with a 2-year history of epistaxis. Upon rhinoscopic inspection, an exophytic, red nodule was found in the nasopharynx, centered on the midline. The tumor was covered by intact mucosa and appeared pulsating. Sagittal T1-weighted images (Figure 1) with fat suppression showed a midline mass with strong contrast enhancement, involving the nasopharyngeal space (arrow) and eroding the clivus (arrowhead). Following an incisional biopsy, surgical excision of the tumor was carried out by transfacial approach.
The surgical specimen consisted of multiple fragments of red-brown tissue with a nodular appearance and a friable texture, measuring in aggregate 4.5 cm. On H&E (Figure 2A) and reticulin (Figure 2B) stains, the tumor showed an organoid architecture resulting from nests of tumor cells separated by thin trabeculae. A rich capillary network was evident, as shown by CD34 immunohistochemistry (Figure 2C). Tumor cells had abundant slightly granular cytoplasm and uniform, vesicular nuclei. No mitosis or necrosis was found. Immunohistochemical studies demonstrated that neoplastic cells expressed synaptophysin (Figure 2D), neuron specific enolase, and chromogranin, but were negative for pan-keratin, leukocyte common antigen, HMB-45, CD31, CD34, CD 68 and vasoactive intestinal polypeptide (VIP). Scattered cells within the neoplasm were immunoreactive for S-100.