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CASE 21. 46 year old man with a papillary lesion of nasopharynx. CK 5/6. Ki-67. LAM. LAM. EBER. Diagnosis. Papillary nasopharyngeal carcinoma, non-keratinizing type undifferentiated, predominantly in-situ with areas of probable superficial invasion. Nasopharyngeal Carcinoma.
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CASE 21 46 year old man with a papillary lesion of nasopharynx.
Diagnosis Papillary nasopharyngeal carcinoma, non-keratinizing type undifferentiated, predominantly in-situ with areas of probable superficial invasion.
Nasopharyngeal Carcinoma “A carcinoma arising in the nasopharyngeal mucosa that shows light microscopic or ultrastructural evidence of squamous differentiation. It encompasses squamous cell carcinoma (differentiated or undifferentiated) and basaloid-squamous carcinoma. Adenocarcinoma and salivary gland carcinoma are excluded.”
Nasopharyngeal Carcinoma • Distinctive geographic and ethnic distribution • Relatively rare tumor worldwide • 65,000 new cases per year • 0.6% of all cancers
NPC - Etiology • Race • Genetics • HLA • Multiple chromosomal changes (loss of material short arm of C-3) • First degree relative with NPC
NPC – Etiology Increased Risk Decreased Risk HLA –A2 HLA –A11 HLA –B14 HLA –B13 HLA –B46 HLA –B22
NPC - Etiology 3. Environment A. Salted fish – nitrosamines B. Cigarette smoking C. Formaldehyde exposure D. Occupational exposure to wood dust E. Alcohol F. Crowded conditions
NPC - Etiology 4. Viruses A. EBV – nonkeratinizing B. ?HPV – keratinizing 5. Miscellaneous A. Chronic rhinosinusitis
NPC – Clinical Features • Peak incidence 30-50 yrs. • Some regions 2 peaks (10-20 and 40-60 yrs.) • M:F 2-3:1
NPC – Clinical Features • Symptoms: cervical mass, serous otitis, epistaxis, nasal obstruction • 10-20% cranial nerve dysfunction • 60-85% positive nodes, 35-45% bilateral • Less than 5% distant mets
NPC – Distribution of 437 Cases • 78% lateral wall – equally distributed between rt. and lt. sides • 12% central • 5% occult • 5% too large to determine Skinner DW, et al. Ann Otol Rhinol Laryngol 100:544, 1991
Nasopharyngeal Carcinoma*(N=209) 74.0% Exophytic 14.4% Infiltrative 6.7% Ulcerative 4.8% Unknown *Dickson, RI, Laryngoscope 91:33, 1981
WHO (1978) • Squamous cell carcinoma • Non-Keratinizing carcinoma • Undifferentiated carcinoma
WHO (1991) • Squamous cell carcinoma 2. Non-Keratinizing carcinoma A. Differentiated non-keratinizing carcinoma B. Undifferentiated carcinoma
WHO (2005) • Squamous cell carcinoma • Non-keratinizing carcinoma A. Differentiated non-keratinizing carcinoma B. Undifferentiated carcinoma 3. Basaloid squamous carcinoma
NPC – Squamous Cell Carcinoma 25% of all NPCs in U.S. Weak relationship to EBV Tends to remain localized Variable response to X-ray 20-40% 5-year survival
NPC – Non-Keratinizing Carcinomas 75% of all NPCs in U.S. Strong relationship with EBV Tend to disseminate Good response to x-ray 65% 5-year survival
Incidence of EBER-1 in 140 NPC* Type % WHO I (N=5) 80 WHO II (N=73) 97.3 WHO III (N=62) 96.8 Tsai S-T, et al, Cancer 77:231, 1996