Nasopharyngeal carcinoma

ABU SUFIAN HASSAN AHMED EL HAJ(E.N.T. Consultant)

Associate Professor Department of Surgery

Faculty of Medicine, University of Gezira

ANATOMY

ANATOMY

Introduction

The Nasopharynx has a cuboidal shape, the lateral walls are formed by the Eustachian tube and the fosse of Rosenmuller.The roof, sloping downwards from the anterior to posterior, is boarder by pharyngeal Hypopharyx, pharyngeal tonsil, and pharyngeal bursa with the base of

skull above .

Anteriorly, the Nasopharynx abutted the choanae and nasal cavity ,

posterior boundary is formed by the muscle of posterior pharyngeal wall .

Inferiorly, the Nasopharynx ends at an imaginary horizontal line formed by the upper surface of the soft palate and the posterior pharyngeal wall.

Pathology of the Nose and Nasopharynx

1 .Congenital malformations.

2 .Inflammation.

3 .Infection and Granulomatus Diseases

4 .Trauma.

5 .neoplasm

1 -Congenital malformations

1.Choanal stenosis or atresia.

2.Involvmemt in cleft palate.

3.Saddle nose in hypertelorism.

1.Choanal stenosis or atresia.

2 .Inflammations & Infections.

A. Acute Rhinitis or Rhino sinusitis

B. Chronic Rhinitis or Rhino sinusitis

- Nonspecific,

- Specific

Cont.

A. Acute Rhinitis or Rhino sinusitis Inflammation of the nasal mucosa with or with out Inflammation of the PNS mucosa due to:

( i )Allergy

( ii )Viral infection

( iii )Bacterial infection

Cont.

B. Chronic Rhinitis or Rhino- sinusitis:

i- Chronic Non specific infection as, Bacterial infection

ii- Chronic specific infection as,

1- Tuberculosis

2- Leprosy

3 - Scleroma

4- Fungal infections as, * Aspergillosis

*Rhinosporidiosis *Candidiasis

5 -Leishmaniasis

Nasal polyposis

a. Allergic

i- Allergic rhinitis

ii- Vasomotor rhinitis

* bilaterally

* ethimoids

* associated ;Asthma, penicillin&

aspirin allergy .

Cont. nasal polps

Non –allergic including antro-choanal polps

4 .Trauma.

5.Neoplasm

Benign tumors

Malignant tumors

Benign tumors

1-Epithelial

i- Papilloma : HPV , EBV

a- Squamous ,fungiform : wart , in vestibule & septum.

b- Inverted Papilloma )Tansitional( lateral wall , high recurrence , 10% malignant transformation )intermediate tumour(

Benign tumors 1-Epithelial Cont .

c- Keratoacanthoma. )exposed skin to sun ___scc(

d- Adenoma ) mucous glands(.

Malignant tumors Epithelial

Carcinoma

a. Scc

b. Tansitional – type

c. Adenocarcinama

d. Anaplastic

Malignant Melanoma

2 -Vascular

Benign tumors a. Capillary haemangioma

b. Juvenile angiofibroma c. Haemangiopericytoma )intermediate

tumour( Malignant tumors

Haemangiopendothelioma)Angiosarcom(

3 -Lymphoid tissue

i- Lymphoma

II- Myeloma

4 -Neurogenic

i.Neurofibroma

II. Nasal glioma )ectopic glial tissue(

iii. Olfactory neuroblastom

iv. Neurilemmoma )schwannoma( –nerve sheath-

5 -Bone and connective tissues

Benign tumors a. Osteoma

b. Chondroma c. Ossifying fibroma

Malignant tumors a. Fibrosarcoma

b.Chondrosarcoma c. Osteogenic sarcoma

Tumours of the Nasopharynx

Benign tumors

a. Tansitional – type papilloma

b. Adenoma

c. Cavernus haemangioma

d. Juvenile angiofibroma

Malignant tumors 1-Carcinomas

a. Anaplastic carcinoma including ( lymphoepitheomalymphoepitheoma)

b. Squamous cell carcinoma c. Tansitional – type carcinoma

d. Adenocarcinama .

Malignant tumors NPx

2-Lymphoma and Myeloma

3-Sarcomas

a.Rhabdomyosarcoma

b. Fibrosarcoma

c. Chondrosarcoma

4 -Chondroma arising from the base of skull

Nasopharyngeal carcinoma )NPC( is epidemiologically and histologically different from other head and neck cancers5

.It is an, Epstein-Barr Virus )EBV(–associated carcinoma. It has been demonstrated that EBV is harbored in almost every NPC tumor, regardless of the degree of differentiation and geographic distribution.2, 3, 4,5

•NPC highest incidence in the World is in Southeast China, Hong Kong and Mediterranean basin.

North Africa and Mediterranean basin. It commonly has poorly differentiated or undifferentiated pathology with a high incidence of cervical lymph node metastasis and great radiosensitivity and chemosensitivity1

•Age distribution ranged from 11 to 82 years with mean age 44.25 years and median of 46 years. The male to female

ratio was 2:1 .

Table (2)Table (2): : Types of malignancies encountered at INMOTypes of malignancies encountered at INMO..

No.No.TypeTypeFrequencyFrequencyPercentagePercentage

11HematologyHematology63363323.823.8

22BreastBreast50150118.818.8

33GITGIT36736713.813.8

44Head and neckHead and neck31431411.811.8

55GynecologyGynecology31031011.611.6

66UrologyUrology22222208.308.3

77Childhood tumorsChildhood tumors09309303.503.5

88Soft tissue sarcomaSoft tissue sarcoma08108103.003.0

99Bone tumorsBone tumors06606602.502.5

1010SkinSkin04704701.801.8

1111Others brain ,lung)Others brain ,lung)282801.101.1

TotalTotal26622662100%100%

m a le

f e m a le

s e x

m a le

f e m a le

s e x

Graph 1: Sex distributionGraph 1: Sex distribution

33.3%33.3%

FemaleFemale

66.7%66.7%

MALEMALE

On the other hand it has low incidence in Europe, Japan and North America.)JCO, Abdelrahhim(. Most cases presents with local disease and cervical lymphadenopathy.

Nasopharyngeal cancer )NPC( is a common cancer in Sudanese and affects

men more than women .

Sudanese usually presents late with

cervical lymphadenopathy .

The commonest histological types were

WHO type II and III.

CLINICAL PRESENTATIONS

NPC characterize by non-specific presentation. Most cases presents with local disease and/or cervical lymphadenopathy, approximately 60-90% of patients present with cervical nodal metastasis3, 11, 12, 13.

Patients with nodal metastasis have ,

higher rates of treatment failure

Symptoms related to primary tumor include ear pain, nasal tone speech, hearing loss, trismus and symptoms and signs of other cranial nerves involvement 14.

Larger tumors may cause nasal block and bleeding. In Sudan NPC is the leading cancer in men15.

CLINICAL PRESENTATIONS

The patients had different clinical presentationsThe most common clinical presentation in the order of frequency was

.1 Cervical lymphadenopathy)73.2%(..2Nasal block)33.9%(.

.3Hearing impairment)27.7%(..4Epistaxis)22.3%( .

.5Ear pain)18.8%( ..6Palatal paralysis)14.3%(.

1 -NECK MASS

Cervical lymph nodes

- More than 75%

- unilateral or bilateral

- Jugulodigastic L N

2 .NASAL.

> 40% Of patients may presented with

Nasal symptoms, in the forms of:

- Nasal bleeding

- Nasal mass.

- Nasal discharge.

- .Nasal deformity

- Nasal obstruction.

3 -EAR

> 30% Of patients may presented with

otological symptoms, Include

- Ear pain ,

- Hearing loss ,

- Ear discharge

( Secrotory otitis media ) Eustachian tube obstruction

CRINIAL NERVES

NoNoNerveNerveNo. of patientsNo. of patientsPercent %Percent %

11OpticOptic 01010.890.89

22OcculomotorOcculomotor07076.256.25

33TrigeminentTrigeminent09098.038.03

44AbducentAbducent11119.829.82

55FacialFacial09098.038.03

66GlossopharngealGlossopharngeal161614.2914.29

77VagusVagus03032.672.67

88HypoglossalHypoglossal09098.038.03

Total = ( 112 )Total = ( 112 )656558.0358.03

Table (1) : Cranial nerve injuryTable (1) : Cranial nerve injury

DISTAL METASTASIS

96%96%

22

lung

bone

liver

Missing96%96%

2%2%

1%1%

1%1%

96%96%

INVESTIGATIONS

1 -RADIOLOGY

- X-ray

soft tissue lateral view of the neck.

- CT scan

nasophynx

- MRI

CRINIAL NERVES

2. ENDOSCOY

3 .GENERAL

4 .BODY SCAN

World Health Organization )WHO( classifies

NPC into 3 types according to histology .

Keratinizing squamous cell carcinoma is WHO type 1. Non keratinizing carcinoma is WHO type 2. Undifferentiated carcinomas and lymphoepithelioma are WHO type 3.

Risk factors in endemic areas

EBV-association is reported to be strongly associated with types 2 and 33, 4.

Risk factors in endemic areas include:

i.EBV,ii salty diet, iii. volatile nitro,iii. HLA antigen haplotype9.

However, in non-endemic regions disease

is associated with alcohol and tobacco use9

Stage:

Locally advanced and metastatic disease

was observed in 85.4% of patients.

Locally advanced disease includes all cases with any cranial nerve palsy,

cervical lymphadenopathy or T4 lesions. Common sites for metastasis were bone, lung and Liver.

SUMMARY

NPC is common in Sudanese population and tends to affect younger age group with male predominance. Most cases present with nodal involvement )CLN(or locally advanced disease. Patients had similar features of histology seen in endemic regions with predominance of WHO-3 histology type

References:1

Cathryn E. Tune, Per-Gunnar Liavaag, Jeremy L. Freeman, et al. Nasopharyngeal Brush Biopsies and Detection of Nasopharyngeal Cancer in a High-Risk Population. J Natl Cancer Inst; 1999; 91: 796–800.

Collins SL. Squamous cell carcinoma of the oral cavity and oropharynx. In: Ballenger JJ, Snow JB Jr., eds. Otorhinolaryngology: head and neck surgery.15th ed. Media, Pa.: Williams & Wilkins, 1996:249-368.Vasef MA, Ferlito A, Weiss LM. Nasopharyngeal carcinoma, with emphasis on its relationship to Epstein-Barr virus. Ann Otol Rhinol Laryngol; 1997;106: 348-56.Cheng Her. Nasopharayngeal cancer and the Southeast Asian patient. Am Fam Physician; 2001; 63 )9(: 1776-1782.Levine PH, Pocinki AG, Madigan P, Bale S. Familial nasopharyngeal carcinoma in patients who are not Chinese. Cancer; 1992; 70:1024–9.

Armstrong RW, Imrey PB, Lye MS, Armstrong MJ, Yu MC, Sani S. Nasopharyngeal carcinoma in Malaysian Chinese: salted fish and other dietary exposures. Int J Cancer. 1998; 77:228-35.Vokes EE, Weichselbaum RR, and Lippman SM, Hong WK. Head and neck cancer. New Eng J Med; 1993; 328:184-194.

Hirayama, T. Descriptive and analytical epidemiology of nasopharyngeal cancer. In: Nasopharyngeal Carcinoma: Etiology and Control. Eds. G. de The and Y, Ito. IARC Scientific Pub 20: p167, 1978.Vokes EE, Liebowitz DN, Weichselbaum RR. Nasopharyngeal carcinoma. Lancet 1997;350: 1087–91.Farrell PJ, Cludts I, Stuhler A. Epstein-Barr virus genes and cancer cells. Biomed Pharmacology; 1997; 51:258–67.

Gasim J, Bachouchi M, Cvitkovic E, et al. Nasopharangeal carcinoma: medical oncology view point: the Gustave Roussy experience. Ann oncol; 1990; 1: 245-253. )Cross ref( )Medline(.Neel HB 3d. Nasopharyngeal carcinoma: diagnosis, staging, and management. Oncology )Huntingt(; 1992; 6:87–95.

Perez CA, Devineni VR, Marcial-Vega V, Marks JE, Simpson JR, Kucik N. Carcinoma of the nasopharynx: factors affecting prognosis. Int J Radiat Oncol Biol Phys; 1992; 23:271–80.

Altun M, Fandi A, Dupuis O, Cvitkovic E, Krajina Z, Eschwege F. Undifferentiated nasopharyngeal cancer )UCNT(: current diagnostic and therapeutic aspects. Int J Radiat Oncol Biol Phys 1995; 32:859-877.Hamad H. M. A. cancer initiative in Sudan. Annals of oncology; 2006; 17 )supplement 8(: viii33-viii38.

Hidayatalla A, Malik M.O.A, El Hadi A. E, Osman A. A, Hutt M. S. R. studies on nasopharyngeal carcinoma in the Sudan: Epidemiology and aetiology. Eur J cancer clin oncol; 1983; 16 )6(: 705-710.Bing-Jian Feng , Majida Jalbout , Wided Ben Ayoub , et al. Dietary risk factors for nasopharyngeal carcinoma in Maghrebian countries. Inter J cancer; 2007; 121 )7(: 1550-1555.Cancer incidence in Denmark 1996. Copenhagen: Danish National Board of Health, 1999.Ho, J. H. Epidemiology of nasopharyngeal carcinoma. In: Cancer in Asia. Ed. T. Hirayama. Baltimore University press; 1976: Page 49.

Kumar S, Zinyu R, Singh I.K.K, Medhi S. B, Baruah T, Das B, Dutta L P. Studies on naspharyngeal carcinoma with reference to the North East region of India. Ann Natl Acad Med Sci )India(; 1996; 32:199.Commoun M, Hoerner G. V, Mourali N. Tumor of nasopharynx in Tunisia: And anatomic and clinical study based on 143 cases. Cancer; 1974; 33:184.Umpati prasal, Lekhraj Rampal. Descriptive epidemiology of nasopharyngeal carcinoma in Peninsula Malaysia. Cancer causes and control; 1992; 3 )2(: 179-182.

Marks JE, Phillips JL, Menck HR. The national cancer data base report on the relationship of race and national origin to the histology of nasopharyngeal carcinoma. Cancer; 1998; 83: 582-88.Burt RD, Vaughan TL, Mcknight B. Descriptive epidemiology and survival analysis of nasopharyngeal carcinoma in the United States. Inetr J cancer; 1992; 52: 549-56.Ho, J. H. AN epidemiology and clinical study of nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys; 1978; 4:189.

References:2

Lin JC, Chen K Y, Wang CW, Jan JS, Liang WM, Tsai CS, Wei YH. Detection of Epstein - Barr virus DNA in the peripheral-Blood cells of patients with nasopharyngeal carcinoma: Relation to distant metastasis and survival. J of Clin Oncol; 2001; 19: 2607-2615.

Chang YS, Tyan YS, Liu ST, et al: Detection of Epstein-Barr virus DNA sequences in nasopharyngeal carcinoma cells by enzymatic DNA amplification. J Clin Microbiol; 1990; 28: 2398-2402.

Mann RB, Epstein JI, et al: Abundant expression of EBER1 small nuclear RNA in nasopharyngeal carcinoma: A morphologically distinctive target for detection of Epstein-Barr virus in formalin-fixed paraffin-embedded carcinoma specimens. Am J Pathol; 1991; 138: 1461-1469.Chen CL, Wen WN, Chen JY, et al: Detection of Epstein-Barr virus genome in nasopharyngeal carcinoma by in situ DNA hybridization. Intervirology; 1993; 36: 91-98.

Pathmanathan R, Prasad U, Chandrika G, et al: Undifferentiated, nonkeratinizing, and squamous cell carcinoma of the nasopharynx: Variants of Epstein-Barr virus-infected neoplasia. Am J Pathol; 1995; 146: 1355-1367 .

Jun Ma, Hai-Qiang Mai, Ming-Huang Hong, Hua-Qing Min, Zhi-Da Mao, Nian-Ji Cui, Tai-Xiang Lu, and Hao-Yuan Mo. Results of a prospective randomized trial comparing neoadjuvant chemotherapy plus radiotherapy with radiotherapy alone in patients with locoregionally advanced nasopharyngeal carcinoma. J of Clinl oncol; 2001; 19 )5(: 1350-57.Min HQ. Nasopharyngeal carcinoma, in Gun GU )ed(: Epidemiology: Beijing, China, People's medical press; 1996: 280-285.

Geara FB, Nasr E, Tucker S L, Birihi E, Zaytoun G, Hadi U, Salem Z, El Saghir N, Issa PH, Shamseddine A. Nasopharyngeal cancer in MiddleEast: Experience of the American University of Beirut medical centre. Int. J. Radiation oncology Biol. Phys; 2005; 65: 1408-15 .

Dodge J L, Mills P K, Yang R C. Nasopharyngeal cancer in California Hmong, 1988-2000. Oral Oncology; 2005; 41: 596-601.Burt RD, Vaughan TL, Mcknight B. Descriptive epidemiology and survival analysis of nasopharyngeal carcinoma in the United States. Inetr J cancer; 1992; 52: 549-56.

Lee A W N, Foo W, Mang O, Sze W.M, Chappell R, Lau W.H, Ko W.M. Changing epidemiology of nasopharyngeal carcinoma in Hong Kong over a 20 -year period ) 1980-1999(: An encouraging results in both incidence and mortality. Inter J cancer; 2002; 103; 5: 680-685.

Chua D. T. T., Jonathan S. T. Sham, Damon Choy, Virchan Lorvidhaya, Yupa Sumitsawan, Sumitra Thongprasert, Visoot Vootiprux, Arkom Cheirsilpa, Tahir Azhar, Ary H. Reksodiputro. Preliminary report of the asian-oceanian clinical oncology association randomized trial comparing cisplatin and epirubicin followed by radiotherapy versus radiotherapy alone in the treatment of patients with locoregionally advanced nasopharyngeal carcinoma. Cancer; 1998; 83: 2270-2283.

Marks JE, Phillips JL, Menck HR. The national cancer data base report on the relationship of race and national origin to the histology of nasopharyngeal carcinoma. Cancer; 1998; 83: 582-88.

Marks JE, Phillips JL, R. Menck HR. The national cancer data base report on the relationship of race and national origin to the histology of nasopharyngeal carcinoma. Cancer; 1998; 83 )3(: 582-588.Ayan I, Altun M. Nasopharyngeal carcinoma in children: retrospective review of 50 patients. Inter J Radiat Oncol Biol Phys; 1996; 35: 485-92.

Barnes L, Brandwein M, Som PM. Diseases of the nasal cavity, paranasal sinuses, and nasopharynx. In: Barnes L, ed. Barnes Surgical Pathology of the Head and Neck. 2nd ed. New York: Marcel Dekker, Inc; 2000.Mertens R, et al. Treatment of nasopharyngeal carcinoma in children and adolescents. Cancer; 2005; 104: 1083-1089.Catherine K, Wang CC. Prognostic value of Chine race in nasopharyngeal cancer. Int J radiation oncology Biol Phys; 2002; 54: 752-58.Buddy YK. Wong. Nasopharyngeal carcinoma in Hong Kong- clinical features and diagnosis. Journal of Hong Kong medical association; 1987; 242-244.

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