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Dr Harjitpal Singh Assistant Professor(ENT), Dr RKGMC, Hamirpur TUMORS OF NASOPHARYNX
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MALIGNANT TUMORS NASOPHARYNX Epithelial tumours: 1 Nasopharyngeal carcinoma 2 Adenocarcinoma 3 Papillary adenocarcinoma 4 Mucoepidermoid carcinoma 5 Adenoid cystic carcinoma 6 Polymorphous low-grade adenocarcinoma
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MALIGNANT TUMORS NASOPHARYNX II. Soft tissue tumours: 1 Fibrosarcoma 2 Rhabdomyosarcoma 3 Angiosarcoma 4 Kaposi’s sarcoma 5 Malignant haemangiopericytoma 6 Malignant nerve sheath tumour 7 Synovial sarcoma
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MALIGNANT TUMORS NASOPHARYNX III. Tumours of bone and cartilage IV. Malignant lymphomas 1 Non-Hodgkin lymphoma 2 Extramedullary plasmacytoma 3 Midline malignant reticulosis 4 Histocytic lymphoma 5 Hodgkin disease V. Secondary tumours VI. Unclassified tumours
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MALIGNANT TUMORS NASOPHARYNX VII. Miscellaneous tumours: 1 Malignant melanoma 2 Chordoma 3 Malignant germ cell tumours
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Ca NASOPHARYNX NPC is the third most common malignancy among men. In India, nasopharyngeal cancer constitutes only 0.5% of all body cancers. It is relatively common in North East states of India where people are predominantly of Mongoloid origin. The male to female ratio of the disease is 3 to 1. The peak age group of presentations of NPC in endemic areas is 50–55 years old, and it decreases with increasing age. More than 80 percent of the carcinomas are squamous cell carcinomas and grossly, the tumor may be of exophytic type, ulcerative or infiltrative variety.
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Ca NASOPHARYNX NPC is a non lymphomatous squamous cell carcinoma that occurs in the epithelial lining of the nasopharynx. This neoplasm shows varying degrees of differentiation and is frequently seen in the pharyngeal recess (Rosenmüller’s fossa), posteromedial to the medial crura of the Eustachian tube opening in the nasopharynx
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Ca NASOPHARYNX 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. Nasopharyngeal carcinoma (NPC) is epidemiologically and histologically different from other head and neck cancers.
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Ca NASOPHARYNX Aetilogy: The exact etiology is yet unknown. NPC is a multifactorial disease. The various factors include genetic susceptibility, environmental factors, dietary and personal habits. Genetic: Chinese, whether they live in china or elsewhere, have a higher genetic susceptibility to nasopharyngeal cancer. Abnormality in chromosome 1-6, 9, 11, 13, 14, 16, 17, 22 and x. Allelic losses on short arms of chromosomes 3 and 9 lead to inactivation of tumor suppressor genes p14,p15, and p16. EBV: Gains on chromosome 12 and allelic losses on 11q, 13q, and 16q causes invasive carcinoma.
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Ca NASOPHARYNX Aetilogy: Mutations in p53 and aberrant expressions of cadherins cause metastases. Specific haplotypes in human lymphocyte antigen (HLA) on short arm of chromosome 6 increases the risk of NPC. Viral: Epstein Barr virus (EBV) is usually associated with nasopharyngeal cancer. Specific viral markers are used to screen people in high-risk areas. Environmental: Air pollution, smoke from burning of incense and wood (polycyclic hydrocarbon). Smoking: Tobacco and opium.
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Ca NASOPHARYNX Aetilogy: EBV typically infects B-lymphocytes and produces the acute syndrome of infectious mononucleosis. The virus does not commonly infect normal nasopharynx epithelium. It is postulated that the combination of genetic susceptibility, environmental factors and EBV infection all contribute to development of NPC.
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Ca NASOPHARYNX Differential diagnosis: • Nasopharyngeal fibroma • Thornwaldt’s cyst or bursitis • Rhinosporidiosis • Inverted papilloma • Other malignant tumors like lymphomas, rhabdomyosarcomas, plasmacytomas, adenoid cystic carcinoma. • Olfactory neuroblastoma • Infections in the nasopharynx including viral infections or tuberculosis of the nasopharynx
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Difference in Nasopharyngeal Fibroma & Ca Nasopharynx
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Difference in Nasopharyngeal Fibroma & Ca Nasopharynx
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Ca NASOPHARYNX Risk Factors: Diets - in salt cured fish and meat Epstein Barr Virus Infection Genetic Factors Male Age 30- 55 yrs old Asian
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Ca NASOPHARYNX Signs and Symptoms: Early cancers of the nasopharynx produce minimal and trivial symptoms. The central location of the nasopharynx in the head means that cancer can spread in all directions, affecting many surrounding organs like the ears and eyes, producing symptoms that would not immediately relate to the nasopharynx
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Ca NASOPHARYNX Signs and Symptoms: Local symptoms and signs of NPC can be classified in to four categories: Nasal; Otological; Cervical; and Neurological.
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Ca NASOPHARYNX Signs and Symptoms: Nasal:- Around 50% of the patients have suffered from nasal symptoms on presentation. The most common symptoms are blood stained nasal discharge or post-nasal drip. The patient may present with nasal blockage. Patients may also report cacosmia or a smell of blood due to bleeding from the tumour.
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Ca NASOPHARYNX Signs and Symptoms: Otological:- 30% and 40% of NPC patients suffer from some form of ear symptom. Theses are due to eustachian tube dysfunction secondary to tumour bulk and/or invasion. Recent onset of ipsilateral hearing loss, muffled sound, tinnitus and sensation of ear blockage. Otoscopic examination will reveal middle ear effusion.
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Ca NASOPHARYNX Signs and Symptoms: Cervical:- Up to 70% of patients with NPC have enlarged neck lymph nodes on presentation. One third of the patients have bilateral enlarged neck lymph nodes. The most frequently involved nodes are level II (upper jugular) and upper level V (apex of posterior triangle). An isolated enlarged supraclavicular lymph node is almost never a lymph node metastasis from NPC. Occasionally, NPC patients can present with metastatic neck lymph nodes from an unknown primary.
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Ca NASOPHARYNX New imaging modalities like magnetic resonance imaging and positron emission tomography (PET) may reveal it. Additional investigations like serological testing for EBV and immunohistochemical staining of the cytological material for EBV can also point to a nasopharyngeal primary.
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Ca NASOPHARYNX Signs and Symptoms: Neurological:- Usually signals advanced disease. Headache usually localized to the vertex or occiput, is present in up to 20% of patients. It is caused by the invasion of the clivus bone by the tumour. Facial pain and midface numbness can also be a presenting symptom, caused by tumour invasion into the pterygopalatine fossa and the branches of V2
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Ca NASOPHARYNX Signs and Symptoms: Neurological:- Cranial nerve (CN)-V2, V3 and VI. palsies are caused by extension of the tumour into the skull base or intracranially. Involvement of CNIII and CNIV are indicative of cavernous sinus invasion by tumour. Rarely, patients present with ophthalmoplegia, decreased vision and proptosis caused by direct invasion of the tumour into the orbital apex Trismus is rare unless the tumour has directly invaded the pterygoid muscles
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Ca NASOPHARYNX
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Ca NASOPHARYNX
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Ca NASOPHARYNX Secondaries Lung– Cannon Ball Appearance
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Ca NASOPHARYNX- SPREAD
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Ca NASOPHARYNX Local Spread: Anteriorly into the nasal fossa, Posterolaterally beyond the pharyngobasilar fascia to involve the parapharyngeal and the carotid spaces Laterally to the pterygoid muscles, Posteriorly to the prevertebral muscles inferiorly to the oropharynx
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Ca NASOPHARYNX- SPREAD Local Spread: Superiorly, bony erosion of the skull base involving: The floor of the sphenoid sinus, Clivus Apex of petrous bone Basal foramina. Intracranially via foramen lacerum High frequency of perineural spread along the maxillary division (V2) and the mandibular division (V3) of the trigeminal nerve with subsequent intracranial extension through the foramen rotundum and foramen ovale
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Ca NASOPHARYNX Diagnosis: In most patients, a tumour mass will be found in the nasopharynx and a biopsy should be taken without delay. Most of the cancers are readily apparent under endoscopy, presenting as a mass with abnormal capillaries on the surface with areas of ulceration. Occasionally, the tumours are infiltrative and no mass may be seen. However, close inspection will reveal abnormal capillaries in the mucosa. Easy contact bleeding is a sign of NPC.
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NASOPHARYNX THROUGH 0O ENDOSCOPE The tumour can be seen obliterating the left fossa of Rosenmüller extending to the right across the midline.
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Biopsy of the Ca NASOPHARYNX 32
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Ca NASOPHARYNX Radiological evaluation: MRI is the imaging of choice for delineating the extent of disease in the skull base. CT scan is useful in detecting cortical bone erosion in the skull base but cannot often differentiate between normal structures and tumour invasion in the skull base. PET CT is a useful adjunct to delineate the extent of disease, nodal metastasis and distant metastasis. CXR, Chest CT, bone scans. Ultrasound scan is suited for investigating neck lymph node metastasis. Ultrasound combined with fine-needle aspiration cytology can achieve a very high sensitivity and specificity.
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Contrast CT Scan Nasopharyngeal Mass
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Ca NASOPHARYNX Radiological evaluation: Positron emission tomography:- A PET scan is a functional imaging study and is normally combined with a cross-sectional imaging modality like a CT scan for anatomical localization. For use in NPC, 18F uoro-deoxyglucose (18FDG) is the most frequently used isotope.
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Ca NASOPHARYNX Laboratory evaluation: Laboratory tests can aid the diagnosis or be useful for monitoring and screening the disease. Blood tests are especially useful for screening purposes and easier to deploy. EBV antibodies serology: 8 IgA antibodies against viral capsid antigen (VCA), early antigen (EA) and EBV nuclear antigen 1 (EBNA1) are commonly used as tumour markers to screen for NPC. EBV IgG titre signifies prior infection by the virus and elevated serum EBV IgM titre indicates recent infection. EBV serology is not an ideal tumour marker for NPC as the serology has weak correlation with the stage of the disease, unable to reflect the tumour response to therapy and does not show increasing titre in recurrent disease
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Ca NASOPHARYNX Laboratory evaluation: Plasma EBV DNA titre can be used as a tumour marker of NPC. A high level of EBV DNA before treatment correlates with the larger tumour load and more advanced stage of the cancer. Plasma EBV DNA levels rapidly decrease to undetectable or very low levels within 1–2 weeks after completion of treatment. An initial drop of plasma EBV DNA levels after treatment and a later progressive rise in the level may represent tumour recurrence. But it can be elevated in any disease associated with EBV, like infectious mononucleosis and EBV-associated lymphoid malignancy. Nasopharyngeal brushing for EBV DNA
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TNM CLASSIFICATION Primary tumor (T) TX Cannot be assessed T0 No tumor Tis Carcinoma in situ T1 Tumor is confined to nasopharynx T2 Tumor extends to soft tissues of oropharynx or nasal fossa T2a No parapharyngeal extension T2b With parapharyngeal extension T3 Tumor invades bones and/or paranasal sinuses T4 Intracranial extension, involvement of cranial nerves, infratemporal fossa, hypopharynx, orbit, or masticator space
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TNM CLASSIFICATION Regional lymph nodes Nx Cannot be assessed N0 No nodal metastases N1 Single ipsilateral node except supraclavicular fossa <= 6 cm N2 Bilateral or contralateral nodes except supraclavicular fossa <= 6 cm N3a Any node > 6 cm N3b Supraclavicular node of any size Distant metastasis (M) Mx Cannot be assessed M0 No distant metastasis M1 Distant metastas
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TNM CLASSIFICATION Stage grouping Stage 0 Tis N0 M0 Satge I T1 N0 M0 Stage IIA T2a N0 M0 Stage IIB T2b N0 M0 or T1-2b N1 M0 Stage III T3 N0 M0 or T1-3 N1-2 M0 Stage IVA T4 N0-2 M0 Stage IVB T1-4 N3 M0 Stage IVC T1-4 N0-3 M1
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WHO CLASSIFICATION OF NPC Type 1 Keratinizing squamous cell carcinoma Type 2 Nonkeratinizing carcinoma - Without lymphoid stroma - With lymphoid stroma Type 3 Undifferentiated carcinoma -Without lymphoid stroma -With lymphoid strom
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Ca NASOPHARYNX
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Ca NASOPHARYNX- Stage T1
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Ca NASOPHARYNX- Stage T2
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Ca NASOPHARYNX- Stage T3
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Ca NASOPHARYNX- Stage T4
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Ca NASOPHARYNX Prognosis: Five-year survival rate varies with stage of NPC. The treatment results of NPC have shown remarkable improvement in the last 40 years. The 5-year disease-specific survival for stage I --- 100%, stage II --- 90%, stage III --- 67%, stage IVA--- 67%, stage IVB --- 68% and stage IVC--- 18% .
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Ca NASOPHARYNX Radiotherapy: Radiotherapy is the mainstay of treatment for primary NPC. Surgical treatment is reserved for salvage of radiation failures. Early stage diseases,including stage I and low-risk stage II NPC, can be treated with radical radiotherapy alone. Stage II disease with higher tumour load and stage III, IV disease require combination chemotherapy and radiotherapy. The two major advances in NPC management are the introduction of intensitymodulated radiotherapy (IMRT) and the discovery that concurrent chemoradiation improves the efficacy of radiotherapy. External beam radiation of 6000–7000 cGy can be delivered by linear accelerator to the primary and both sides of neck IMRTallow higher dose delivery to the tumour with reduced damage to the adjacent normal structures such as spinal cord, brainstem and parotid glands
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Ca NASOPHARYNX Chemotherapy: Concurrent cisplatin during radiotherapy followed by adjuvant cisplatin and fluorouracil as the standard of care for advanced NPC. Goal of chemoradiotherapy in nasopharyngeal carcinoma is to improve local control of tumour and to treat distant metastases. Cisplatin is the usual agent used concurrently with radiotherapy. Weekly (30–40 mg / m2) or 3-weekly (100 mg / m2) cisplatin are acceptable standards of care. A total dose of 200 mg / m2 administered during radiotherapy is required to benefit survival. New chemotherapy agents such as taxanes and gemcitabine have been found to be useful in the treatment of recurrent or metastatic NPC.
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Ca NASOPHARYNX Recurrent Disease: First it should be evaluated by CT and MRI to see the size, location and regional extent or infiltration. More recently, PET-CT has been used to find any regional or systemic metastases. Recurrent or residual tumor may be managed with second course of radiation, cryosurgery, through a palatal fenestrationor skull base surgery in selected cases.
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Ca NASOPHARYNX Recurrent/Residual Neck Nodes: Re-irradiation treatment for salvaging nodal failure is not recommended They require radical neck dissection with removal of sternocleidomastoid muscle, CN XI and internal jugular vein. Bilateral neck disease may require bilateral neck dissection but with preservation of internal jugular vein to avoid cerebral and facial oedema
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Ca NASOPHARYNX Recurrent Disease: (iii) Nasopharyngectomy: It is only reserved for salvaging radiation failures. It can be done by various ways such as by (a) endoscopic approach, (b) lateral rhinotomy & medial maxillectomy, (c) maxillary swing or (d) Le Fort I approach. Before undertaking nasopharyngectomy exclude extension of growth intracranially, to parapharyngeal space, or around the internal carotid artery.
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Ca NASOPHARYNX Recurrent Disease: It can be treated by: (i) Second course of external radiation. IMRT has been used. Second course of radiation is more hazardous and causes injury to brainstem, eye, ear, pituitary gland and temporal lobe. (ii) Brachytherapy. It can deliver high dose to the tumour with less radiation to the surrounding structures. Gold grains (Gold 198) have been used.
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HO’S TRIANGLE(Supraclavicular fossa ) It is bounded by medial (A) and lateral (B) ends of clavicle and the point (C) where neck meets the shoulder. It includes caudal portions of levels IV and V Glands in this triangle may be because of NPC
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Ca NASOPHARYNX Trotter’s triad: NPC N: Neuralgia (CN-5); P: Palatalparalysis(CN-x); C: Conductive deafness Nasopharyngeal carcinoma symptoms: NOSE N: Neck nodes; O: Obstruction nose; S: Serous otitis media; E: Epistaxis. NPC may extend through foramen lacerum causing 6th nerve palsy, at the tip of petrous bone.
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COMPLICATIONS OF TREATMENT Mucositis of the oral cavity, oropharynx and hypopharynx occurs to a varying degree during treatment. The mucositis can be severely aggravated if concurrent chemotherapy is given. Salivary function will be decreased during radiotherapy and depending on the dose to the major salivary glands, the salivary function may or may not recover.
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COMPLICATIONS OF TREATMENT Otological complications: OME, acute otitis media and otitis externa are common complications. Late complications of radiotherapy to the ears include sensorineural hearing loss and osteoradionecrosis of the temporal bone. The treatment of OME should be conservative.
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COMPLICATIONS OF TREATMENT Olfactory dysfunction, nasal crusting, rhinosinusitis and intranasal adhesions are common nasal complications. Nasal crusting and symptoms of rhinosinusitis (thick nasal catarrh and foul smelling nasal discharge) are very common following radiotherapy. Minor nasal adhesions between septal spurs and the nasal turbinates are quite common and need no treatment. Occasionally, marked scarring at or close to the choana. These adhesions should be removed to facilitate future endoscopic assessment of the nasopharynx.
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COMPLICATIONS OF TREATMENT Swallowing problems in NPC patients treated with radiotherapy is very common and can occur early after treatment. Late dysphagia can affect up to 50% of the head and neck cancer patients treated with concurrent chemoradiation. Radiation-induced brosis is another late complication that can lead to neck stiffness and trismus.
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COMPLICATIONS OF TREATMENT Osteoradionecrosis of the skull base and radiation fibrosis can lead to lower cranial nerve palsies. The differential diagnosis of late lower cranial nerve palsies is local recurrence and can be difficult to differentiate from osteoradionecrosis or post- radiation fibrosis. Hypothalamic-pituitary dysfunction may result. The symptoms and signs of temporal lobe necrosis are subtle, including personality changes, memory loss and loss of cognitive function. Radiation-induced malignancies mostly develop beyond 5 years after radiotherapy
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OTHER MALIGNANT TUMOURS OF NASOPHARYNX They are rare and include: Lymphomas. Non-Hodgkin’s type is more common than Hodgkin’s type. Almost all are B-cell type. T-cell lymphomas are seen in Asian population. Rhabdomyosarcoma. Commonly seen in children. Embryonal rhabdomyosarcoma presents as a polypoid mass in the nasopharynx. Plasmacytoma. It may be solitary or part of generalized multiple myelomatosis. Chordoma (from remnant of notochord). Adenoid cystic carcinoma (from minor salivary glands). Melanoma (rare).
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HarjitpalSingh

About : Associate Professor (ENT) at Dr Radhakrishnan Govt Medical College, Hamirpur(H P)
Occupation : Medical Professional
Specialty : MD
Country : India
Website :

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