Nasopharyngeal carcinoma in situ (NPCIS)—pathologic and clinical perspectives

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Abstract
Background Dysplasia or carcinoma in situ lesions (NPCIS) of the nasopharynx have rarely been reported. The prevalence, biologic behavior, and the transformation period of the pure preinvasive lesions have not been fully explained. Methods All cases of NPCIS were retrospectively reviewed during the period between 1990 and 2000. The clinical features of all cases were studied. The biopsy samples were examined using light microscopy and in situ hybridization (ISH) for EBV-encoded RNA (EBER). The sera taken before and after the transformation were analyzed for anti-viral capsid antigen (VCA) EBV titers and circulating cell-free EBV DNA concentration. Results Three cases of NPCIS were identified. Two of the three cases subsequently developed into invasive NPC after initial presentation. The interval of transformation varied from 40 to 48 months. In all three cases, the specimens showed abnormal findings on light microscopy and positive staining for EBER. Elevated anti-VCA titers were present in two of the preinvasive lesions. No cell-free EBV DNA was detected in the sera of these patients during the preinvasive phase of the disease. Conclusions Preinvasive NPC is a rare but distinct entity. Its transformation period can be as long as 4 to 5 years. Elevated anti-VCA titers, in the presence of abnormal cells on light microscopy, should alert the pathologist to perform ISH EBER studies to diagnose this rare condition.