Nasopharyngeal carcinoma

Nasopharyngeal carcinoma 

Updated: 10/13/2020

© Jun Wang, MD, PhD

General features
  • Arising from the epithelium of the nasopharynx
  • Common in South Asia, North Africa, Middle East and the Arctic
  • More common in men
  • Rare in the states, but incident increasing in black teenagers
  • Bimodal age distribution, a small peak in late childhood, and second peak around 50-59
Risk factors
  • Associated with Epstein-Barr virus infection
  • Other risk factors: Consumption of salt-preserved fish containing carcinogenic nitrosamines, family history, specific HLA class I genotypes, tobacco smoking, chronic respiratory tract conditions and low consumption of fresh fruits and vegetables
Pathogenesis
  • EBV genome products (LMP1, LMP2, etc) associated telomerase dysregulation, bcl2 overexpression, etc.
  • Loss of function mutation of NF-kappa B inhibitors (CYLD, TRAF3, etc)
  • Multiple chromosomal abnormalities
  • p16 deletion
  • LTBR (lymphotoxin-beta receptor) amplification
Clinical presentations
  • Most common symptoms: Headache, diplopia, or facial numbness
  • Clinical triad: Neck mass, nasal obstruction with epistaxis, serous otitis media
  • Often bilateral neck involvement
  • Cranial nerve palsy
  • Nasopharynx mass by nasopharyngoscopy
Key morphological features
  • Three histologic subtypes:
Keratinizing squamous cell carcinoma (WHO type 1): Invasive carcinoma with obvious squamous differentiation
Nonkeratinizing-differentiated (WHO type 2): Cords or trabeculae, with little or no keratinization
Nonkeratinizing-undifferentiated (WHO type 3): Most common type, cohesive cells with indistinct cell margins or diffuse cellular infiltrate of non-cohesive cells resembling non-Hodgkin lymphoma
Marker
  • Positive for cytokeratin, EMA
  • Positive for EBV antigen, EBER (EBV encoded RNA)
  • Negative for lymphocytic markers: CD45 etc
Diagnosis
  • EBV studies: IgG against early EBV antigen; IgA against viral capsid antigen
  • Radiologic studies
  • Blind biopsies, particularly in fossa of Rosenmuller
Differential diagnosis:
  • Nasal Polyps: Microscopic morphology
  • Pediatric Non-Hodgkin Lymphoma: Positive for lymphocytic markers
  • Pediatric Rhabdomyosarcoma: Positive for desmin, negative for cytokeratin
Treatment
  • Radiation therapy: Mainstay of treatment
  • Chemotherapy for advanced cases
  • Usually unresectable by surgery
Poor prognostic factors
  • High staging
  • Keratinized type
  • Metastasis
  • Older age
  • High plasma/serum EBV DNA titers


Back to respiratory tract neoplasms
Back to contents

Comments

Post a Comment

Popular posts from this blog

Contents

Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4.  Benign blood cell disorders Anemia Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas...
Read more

Anemia

Anemia Updated: 07/13/2024 © Jun Wang, MD, PhD   General features Decrease in red cell mass Hemoglobin: Levels of hemoglobin in a specific volume of whole blood Hematocrit: Fraction of whole blood composed of red blood cells Red cell count: Quantity of red cell in a specific volume of whole blood Three major groups based on RBC sizes Microcytic Normocytic Macrocytic Three major causes Blood loss: acute or chronic Increased red cell destruction: hemolysis Reduced production: marrow failure due to genetic abnormality or nutritional deficiency Etiology Genetic abnormality Hemoglobinopathies RBC cytoskeletal abomorlities Enzyme abnormalities Fanconi anemia Nutritional deficiencies: iron, VB12, folate, general malnutrition Physical etiologies: trauma, burns, frostbite, prosthetic valves, etc Chronic diseases: chronic infections, neoplasia, autoimmune disorders, kidney or liver diseases, etc Infectious diseases: hepatitis, sepsis , malaria, e...
Read more

Lymphoid neoplasms

Lymphocytic neoplasms Updated: 05/17/2020 © Jun Wang, MD, PhD Terminology Lymphoma : Proliferations forming discrete tissue masses Leukemia : Widespread involvement of the bone marrow and usually the peripheral blood Any "lymphoma" can present with or evolve to a leukemic picture, and any "leukemia" can occasionally present with a mass lesion Other hematology terms Important risk factors Immunodeficiency (primary or secondary) Autoimmune disorders Viruses (HIV, KSHV/HHV8, HTLV-1) H. pylori infection Radiation Chemotherapy Diagnostic approaches Complete history, especially those with possible epidemic characters, such as Burkitt lymphoma CBC, Bone marrow Morphology/immunohistochemistry Immunofluorescence flow cytometry Cytogenetic studies Molecular studies Commonly used markers All lymphomas: CD45 B-cell neoplasms: CD5, CD10, CD19, CD20, CD79a, CD23, bcl2, bcl6, cyclin D1, light chai...
Read more