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Introduction & History.
Introduction & History.
• Nasopharyngeal carcinoma is a rare
tumor arising from the epithelium of
the nasopharynx.
• It accounts for approximately 1% of all
childhood malignancies.
Relevant Anatomy
Relevant Anatomy
• The nasopharynx represents the most
superior portion of the pharynx
• bounded superiorly by the skull base and
inferiorly by the soft palate.
• The nasopharynx connects the nasal cavity
to the oropharynx and contains the
Eustachian tube openings and adenoids
Relevant Anatomy
•
Relevant Physiology
Relevant Physiology
• Plays a role in purifying and conditioning
the inhaled air before letting it move further
down the respiratory tract.
Etiology
Etiology
• Idiopathic
• Congenital/ Genetic
• Nutritional Deficiency/excess
• Traumatic
• Infections /Infestation
• Autoimmune
• Neoplastic (Benign/Malignant)
• Degenerative
• Iatrogenic
Etiology
• Genetic-higher rates of disease
– within specific ethnic groups,
– first-degree relatives with the disease,
– patients with A2 HLA haplotypes, and
cytogenetic abnormalities identified within
tumor samples.
• Environmental-
– geographical distribution of the disease
– bimodal age distribution,
– consume a large amount of preserved foods
and/or salted fish
Pathophysiology
Pathophysiology
• Epstein-Barr virus (EBV) can infect
epithelial cells and is associated with their
malignant transformation.
• Copies of the EBV genome have been
found in cells of preinvasive lesions,
suggesting that it is directly related to the
process of transformation.
Pathology
Pathology
• Almost all adult nasopharyngeal
cancers are carcinomas only 35-50% of
nasopharyngeal malignancies are
carcinomas in children.
• In the pediatric population, additional
nasopharyngeal malignancies
include rhabdomyosarcomas or
lymphomas.
Classification
Classification
• 3 WHO categories-
1. WHO-1 is defined as well–to–moderately
differentiated squamous or transitional cell
carcinoma with keratin production.
2. WHO-2 is nonkeratinizing carcinoma.
3. WHO-3 is undifferentiated carcinoma,
The vast majority of children are found to
have WHO-3 disease. The vast majority of
children are found to have WHO-3 disease.
TNM Staging
•
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ
T1
Tumor confined to the nasopharynx or extends to
oropharynx and/or nasal cavity without parapharyngeal
extension
T2 Tumor with parapharyngeal extension
T3
Tumor involves bony structures of skull base and/or
paranasal sinuses
T4
Tumor with intracranial extension and/or involvement of
cranial nerves, hypopharynx, orbit, or with extension to
the infratemporal fossa/masticator space
TNM Staginng
•
NX
Regional lymph nodes cannot be
assessed
N0 No regional lymph node metastasis
N1
Unilateral metastasis in cervical lymph
node(s), less than or equal to 6 cm in
greatest dimension, above the
supraclavicular fossa, and/or unilateral or
bilateral retropharyngeal lymph nodes,
less than or equal to 6 cm in greatest
dimension
N2
Bilateral metastasis in a cervical lymph
node (s), less than or equal to 6 cm in
greatest dimension, above the
supraclavicular fossa
N3
Metastasis in a lymph node(s) greater
than 6 cm and/or to supraclavicular fossa
N3a Greater than 6 cm in dimension
N3b Extension to supraclavicular fossa
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1
Unilateral metastasis in cervical lymph node(s), less than or equal
to 6 cm in greatest dimension, above the supraclavicular fossa,
and/or unilateral or bilateral retropharyngeal lymph nodes, less than
or equal to 6 cm in greatest dimension
N2
Bilateral metastasis in a cervical lymph node (s), less than or equal
to 6 cm in greatest dimension, above the supraclavicular fossa
N3
Metastasis in a lymph node(s) greater than 6 cm and/or to
supraclavicular fossa
N3a Greater than 6 cm in dimension
N3b Extension to supraclavicular fossa
TNM Staginng
•
M0 No distant metastasis
M1 Distant metastasis
TNM Staginng
•
Stage T N M
0 Tis No M0
I T1 N0 M0
II
T1 N1 M0
T2 N0 M0
T2 N1 M0
III
T1 N2 M0
T2 N2 M0
T3 N0 M0
T3 N1 M0
T3 N2 M0
IVA
T4 N0 M0
T4 N1 M0
T4 N2 M0
IVB Any T N3 M0
IVC Any T Any N MI
Clinical Features
Clinical Features
• Demography
• Symptoms
• Signs
• Prognosis
• Complications
Demography
Demography
• 1 in every 100,000 children are diagnosed
annually in North America.
• Southeast Asian and Northern African
descent, with an incidence of 8-25 in every
100,000 children annually.
• Asian, Middle Eastern, and Northern
African descent are also more commonly
affected.
• male-to-female ratio is approximately 2:1.
Demography:Age
• bimodal age distribution.
• A small peak is observed in late childhood,
and a second peak occurs in people aged
50-60 years.
• Childhood nasopharyngeal carcinoma is
usually a disease of adolescence.
Symptoms
Symptoms
• rarely comes to medical attention before it
has spread to regional lymph nodes.
• Nasal symptoms: including bleeding,
obstruction, and discharge
• Ear symptoms: otalgia, serous otitis with
effusion, deafness, and tinnitus
• Headaches
• Trismus
• Neck swelling
• Cranial nerve palsies
Signs
Signs
• Examination of the nasopharynx was historically
performed with a mirror and has greatly been
improved with the use of a rigid or flexible
nasopharyngoscope .
Signs
• neck mass consisting of painless firm lymph node
enlargement often bilateral..
• jugulodigastric, and upper and middle jugular
nodes
• Cranial nerve palsy at initial presentation is
observed in 25% of patients.
• On nasopharyngoscopy, a mass arising in the
nasopharynx is often visible. The most frequent
site is the fossa of Rosenmüller.
• A paraneoplastic osteoarthropathy has been
described in patients with widespread metastatic
or recurrent disease.
Prognosis
Prognosis
• When radiotherapy is used alone, survival
rates range from 40-50%. Use of
combination radiation therapy and
chemotherapy allows long-term survival
rates of 55-80%.
Investigations
Investigations
• Laboratory Studies
– Routine
– Special
• Imaging Studies
• Tissue diagnosis
– Cytology
• FNAC
– Histology
Investigations
• Laboratory Studies
– Epstein-Barr virus (EBV) titers, including
immunoglobulin A (IgA) and immunoglobulin
G (IgG) antibodies.
Diagnostic Studies
Diagnostic Studies
Imaging Studies
• X-Ray
• USG
• CT
• Angiography
• MRI
• Endoscopy
• Nuclear scan
Diagnostic Studies
• CT scanning of the head and neck is
used to determine tumor extent, base of
skull erosion, and cervical
lymphadenopathy.
• CT scanning of the chest is used to
search for distant metastases.
• When intracranial extension is
suspected, MRI of the head and skull
base may better reveal the extent of the
tumor.
Diagnostic Studies
• Bone scans are used to search for
distant bony metastatic disease.
• Positron emission tomography (PET)
imaging has been used to assess
questionable neck nodes and evaluate
for other sites of distant disease.
Other Tests
• A baseline audiogram is helpful prior
to platinum-based chemotherapy and
radiotherapy.
• Creatinine clearance rates (24-hour
collection or nuclear GFR testing)
should be obtained at baseline and
during treatment for those patients
being treated with platinum-based
chemotherapy
Differential Diagnosis
Differential Diagnosis
• Pediatric Nasal Polyp
• Pediatric Non-Hodgkin Lymphoma
• Pediatric Rhabdomyosarcoma
Management
Management
• Surgical therapy for these patients is often
limited to a biopsy for tissue diagnosis.
Nearly all tumors are unresectable at
diagnosis because of their location.
• Radiation therapy is the mainstay of
treatment, with chemotherapy used in
advanced cases.
Management
• Sequential chemoradiotherapy with
gemcitabine and cisplatin has been shown
to improve survival in locoregionally
advanced nasopharyngeal carcinoma.
• Concurrent cisplatin, 5-fluorouracil, and
radiotherapy have been shown to improve
survival.
• Nivolumab- a monoclonal antibody.
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Nasopharyngeal carcinoma.pptx

  • 1. Tips on using my ppt. 1. You can freely download, edit, modify and put your name etc. 2. Don’t be concerned about number of slides. Half the slides are blanks except for the title. 3. First show the blank slides (eg. Aetiology ) > Ask students what they already know about ethology of today's topic. > Then show next slide which enumerates aetiologies. 4. At the end rerun the show – show blank> ask questions > show next slide. 5. This will be an ACTIVE LEARNING SESSION x three revisions. 6. Good for self study also. 7. See notes for bibliography.
  • 3. Introduction & History. • Nasopharyngeal carcinoma is a rare tumor arising from the epithelium of the nasopharynx. • It accounts for approximately 1% of all childhood malignancies.
  • 5. Relevant Anatomy • The nasopharynx represents the most superior portion of the pharynx • bounded superiorly by the skull base and inferiorly by the soft palate. • The nasopharynx connects the nasal cavity to the oropharynx and contains the Eustachian tube openings and adenoids
  • 8. Relevant Physiology • Plays a role in purifying and conditioning the inhaled air before letting it move further down the respiratory tract.
  • 10. Etiology • Idiopathic • Congenital/ Genetic • Nutritional Deficiency/excess • Traumatic • Infections /Infestation • Autoimmune • Neoplastic (Benign/Malignant) • Degenerative • Iatrogenic
  • 11. Etiology • Genetic-higher rates of disease – within specific ethnic groups, – first-degree relatives with the disease, – patients with A2 HLA haplotypes, and cytogenetic abnormalities identified within tumor samples. • Environmental- – geographical distribution of the disease – bimodal age distribution, – consume a large amount of preserved foods and/or salted fish
  • 13. Pathophysiology • Epstein-Barr virus (EBV) can infect epithelial cells and is associated with their malignant transformation. • Copies of the EBV genome have been found in cells of preinvasive lesions, suggesting that it is directly related to the process of transformation.
  • 15. Pathology • Almost all adult nasopharyngeal cancers are carcinomas only 35-50% of nasopharyngeal malignancies are carcinomas in children. • In the pediatric population, additional nasopharyngeal malignancies include rhabdomyosarcomas or lymphomas.
  • 17. Classification • 3 WHO categories- 1. WHO-1 is defined as well–to–moderately differentiated squamous or transitional cell carcinoma with keratin production. 2. WHO-2 is nonkeratinizing carcinoma. 3. WHO-3 is undifferentiated carcinoma, The vast majority of children are found to have WHO-3 disease. The vast majority of children are found to have WHO-3 disease.
  • 18. TNM Staging • TX Primary tumor cannot be assessed T0 No evidence of primary tumor Tis Carcinoma in situ T1 Tumor confined to the nasopharynx or extends to oropharynx and/or nasal cavity without parapharyngeal extension T2 Tumor with parapharyngeal extension T3 Tumor involves bony structures of skull base and/or paranasal sinuses T4 Tumor with intracranial extension and/or involvement of cranial nerves, hypopharynx, orbit, or with extension to the infratemporal fossa/masticator space
  • 19. TNM Staginng • NX Regional lymph nodes cannot be assessed N0 No regional lymph node metastasis N1 Unilateral metastasis in cervical lymph node(s), less than or equal to 6 cm in greatest dimension, above the supraclavicular fossa, and/or unilateral or bilateral retropharyngeal lymph nodes, less than or equal to 6 cm in greatest dimension N2 Bilateral metastasis in a cervical lymph node (s), less than or equal to 6 cm in greatest dimension, above the supraclavicular fossa N3 Metastasis in a lymph node(s) greater than 6 cm and/or to supraclavicular fossa N3a Greater than 6 cm in dimension N3b Extension to supraclavicular fossa NX Regional lymph nodes cannot be assessed N0 No regional lymph node metastasis N1 Unilateral metastasis in cervical lymph node(s), less than or equal to 6 cm in greatest dimension, above the supraclavicular fossa, and/or unilateral or bilateral retropharyngeal lymph nodes, less than or equal to 6 cm in greatest dimension N2 Bilateral metastasis in a cervical lymph node (s), less than or equal to 6 cm in greatest dimension, above the supraclavicular fossa N3 Metastasis in a lymph node(s) greater than 6 cm and/or to supraclavicular fossa N3a Greater than 6 cm in dimension N3b Extension to supraclavicular fossa
  • 20. TNM Staginng • M0 No distant metastasis M1 Distant metastasis
  • 21. TNM Staginng • Stage T N M 0 Tis No M0 I T1 N0 M0 II T1 N1 M0 T2 N0 M0 T2 N1 M0 III T1 N2 M0 T2 N2 M0 T3 N0 M0 T3 N1 M0 T3 N2 M0 IVA T4 N0 M0 T4 N1 M0 T4 N2 M0 IVB Any T N3 M0 IVC Any T Any N MI
  • 23. Clinical Features • Demography • Symptoms • Signs • Prognosis • Complications
  • 25. Demography • 1 in every 100,000 children are diagnosed annually in North America. • Southeast Asian and Northern African descent, with an incidence of 8-25 in every 100,000 children annually. • Asian, Middle Eastern, and Northern African descent are also more commonly affected. • male-to-female ratio is approximately 2:1.
  • 26. Demography:Age • bimodal age distribution. • A small peak is observed in late childhood, and a second peak occurs in people aged 50-60 years. • Childhood nasopharyngeal carcinoma is usually a disease of adolescence.
  • 28. Symptoms • rarely comes to medical attention before it has spread to regional lymph nodes. • Nasal symptoms: including bleeding, obstruction, and discharge • Ear symptoms: otalgia, serous otitis with effusion, deafness, and tinnitus • Headaches • Trismus • Neck swelling • Cranial nerve palsies
  • 29. Signs
  • 30. Signs • Examination of the nasopharynx was historically performed with a mirror and has greatly been improved with the use of a rigid or flexible nasopharyngoscope .
  • 31. Signs • neck mass consisting of painless firm lymph node enlargement often bilateral.. • jugulodigastric, and upper and middle jugular nodes • Cranial nerve palsy at initial presentation is observed in 25% of patients. • On nasopharyngoscopy, a mass arising in the nasopharynx is often visible. The most frequent site is the fossa of Rosenmüller. • A paraneoplastic osteoarthropathy has been described in patients with widespread metastatic or recurrent disease.
  • 33. Prognosis • When radiotherapy is used alone, survival rates range from 40-50%. Use of combination radiation therapy and chemotherapy allows long-term survival rates of 55-80%.
  • 35. Investigations • Laboratory Studies – Routine – Special • Imaging Studies • Tissue diagnosis – Cytology • FNAC – Histology
  • 36. Investigations • Laboratory Studies – Epstein-Barr virus (EBV) titers, including immunoglobulin A (IgA) and immunoglobulin G (IgG) antibodies.
  • 38. Diagnostic Studies Imaging Studies • X-Ray • USG • CT • Angiography • MRI • Endoscopy • Nuclear scan
  • 39. Diagnostic Studies • CT scanning of the head and neck is used to determine tumor extent, base of skull erosion, and cervical lymphadenopathy. • CT scanning of the chest is used to search for distant metastases. • When intracranial extension is suspected, MRI of the head and skull base may better reveal the extent of the tumor.
  • 40. Diagnostic Studies • Bone scans are used to search for distant bony metastatic disease. • Positron emission tomography (PET) imaging has been used to assess questionable neck nodes and evaluate for other sites of distant disease.
  • 41. Other Tests • A baseline audiogram is helpful prior to platinum-based chemotherapy and radiotherapy. • Creatinine clearance rates (24-hour collection or nuclear GFR testing) should be obtained at baseline and during treatment for those patients being treated with platinum-based chemotherapy
  • 43. Differential Diagnosis • Pediatric Nasal Polyp • Pediatric Non-Hodgkin Lymphoma • Pediatric Rhabdomyosarcoma
  • 45. Management • Surgical therapy for these patients is often limited to a biopsy for tissue diagnosis. Nearly all tumors are unresectable at diagnosis because of their location. • Radiation therapy is the mainstay of treatment, with chemotherapy used in advanced cases.
  • 46. Management • Sequential chemoradiotherapy with gemcitabine and cisplatin has been shown to improve survival in locoregionally advanced nasopharyngeal carcinoma. • Concurrent cisplatin, 5-fluorouracil, and radiotherapy have been shown to improve survival. • Nivolumab- a monoclonal antibody.
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