Nasopharynx

Peak incidence in 4th & 5th Decades of life [1, 2] 2X more common in males over females [1] In US 7/1,000,000,000 people will get NPC with 2600 cases in 2010 [2] || i) poor ventilation, ii) occupational exposures to smoke or dusts, iii) diet high in salt cured meats iv) Smoking may contribute – more research needed || ENDOSCOPIC: examinations include nasopharyngoscopy and biopsies of the nasopharynx and adjacent suspicious areas. A Panendoscopy may or may not be done as well.[3] IMAGING: Radiographic studies of the head and neck are used to assess the locoregional extent. MRI is the study of choice because of it's superior sensitivity. CT with contrast is an acceptable substitute. A Chest x-ray is done. A Bone Scan may be ordered if the patient complains of pain or tenderness. Radiographs of the bones may be ordered if the bone scan is abnormal. A Liver scan is only indicated by right upper quadrant pain, enlarged liver by palpation or elevated liver chemistries. If there is advanced locoregional disease then a PET/CT can be ordered as well. LABS: Blood counts, blood chemistry profile, and liver function tests are ordered.[4] || Remaining 10% of types include lymphomas, plasmacytoma, melanoma, rhabdomyosarcoma, chordoma, and minor salivary gland origin [5,3] || Nodes involved include the Cervical, Subdigastric (Jugulodigastric), Supraclavicular, Retropharyngeal, Upper Jugular, and Spinal Accessory (Level V)   || Lymphatic spread of nasopharynx carcinoma occurs in many cases. According to Lu, “Lymphatic spread to the ipsilateral neck occurs in 85-90 % of cases and to bilateral neck in 50 % of the cases.” The lateral and medial retropharyngeal lymph nodes are the first level of nodes that are invaded by NPC. Other nodal invasion includes the Jugulodigastric and superior/posterior cervical nodes. The most common distant metastasis sites are the bone, lung, liver, and distant lymph nodes. According to Lu, “Distant metastasis occurs in 3% of the cases at presentation and may occur in a much higher percentage (ranging from 18% to above 50%) of the cases in the disease course.” Distant metastases are usually the results of highly advanced lymphatic spread. [6] || Stage II: An invasive cancer (T2) that has not spread to lymph nodes (N0), or to distant parts of the body (M0). 
 * Epidemiology: || Uncommon in most countries (Highest incidences in China, SE Asia & Eskimos) [1]
 * Etiology: || # ** Epstein Barr Virus ** – almost all NPC patients have evidence of exposure to the virus – connection not completely understood[1,2]
 * 1) ** Genetic Risk ** -if a family member has NPC increase risk for relatives (possible that shared diet or environmental exposure may cause this)[1]
 * 2) ** Environmental exposure ** [1,2]
 * Signs & Symptoms: || Nasopharyngeal cancer is most often seen in people over the age of 50. Symptoms from nasopharyngeal cancer are normally seen later in the course of disease. The symptoms associated with nasopharynx tumors include:
 * Nasal stuffiness, discharge, or epistaxis which occurs from tumor growth in the posterior nasal fossa.
 * Pain and a decrease in hearing may develop due to tumor growth of the orifice of the Eustachian tube.
 * The nasopharynx lesions cause unilateral serous otitis media or nasal obstruction or epistaxis.
 * Headache, pain in the occipital or temporal area.
 * Development of a neck mass (only seen in 18%-66% of the cases).
 * Cervical lymph node involvement (seen in physical examination 60%-87% of the time).
 * Proptosis may occur when the tumor extends into the orbit.
 * Sore throat from lesions that involve the oropharynx.
 * Possibility of cranial nerve involvement upon presentation.
 * Involvement of cranial nerves III through VI. [3] ||
 * Diagnostic Procedures: || GENERAL: An intake of the patient's thorough medical history must be performed as well as a physical examination.The physical exam is done to assess the primary tumor extent, palpate the neck node(s),test the cranial nerve(s) for assessment of vision, and inspect the tympanic membranes for hearing functions. While palpating the neck node(s) the following must be recorded: the size, laterality and lowest extent of enlarged node.
 * Histology: || Squamous cell or it's variants (Epidermoid or undifferentiated carcinomas) most common type, accounting for about 90%;
 * Lymph Node Drainage: || Bilateral drainage;
 * Metastatic Spread: || Nasopharyngeal carcinoma (NPC) metastases occur most frequently compared to other head and neck tumors. Organs surrounding the nasopharynx are greatly in danger of invasion. For example it is very common for the nasal cavity to become invaded through the choanae. Paranasal sinuses are also at risk of invasion, but it is less common. The tumor can also invade the parapharyngeal space, cranial nerves, cervical sympathetic nerves, internal carotid artery, pterygoid muscles, and other near by areas.
 * Grading: || Primary tumor described by its grade, by histological examination. Histological grade describes how closely the cancer cells resemble normal tissue under a microscope. Normal tissue contains many different types of cells grouped together, which is called differentiated.Tissue from a tumor usually has cells that look more alike each other (called poorly differentiated).Generally, the more differentiated the tissue, the better the prognosis. A tumor's grade is described using the letter "G" and a number. GX: Indicates the grade cannot be evaluated. G1: Indicates the cells look more like normal tissue (well differentiated). G2: The cells are only moderately differentiated. G3: The cells don’t resemble normal tissue (poorly differentiated). Recurrent: Recurrent cancer is cancer that comes back after treatment.[7] ||
 * Staging: || The American Joint Committee on Cancer (AJCC) TNM system. Tumor Describes the size and location of the tumor by assigning “T” plus a number (0-4) Tx Primary tumor cannot be evaluated T0 No evidence of primary tumor present T1 Confined to nasopharynx, or tumor extends to oropharynx and/or nasal cavity without parapharyngeal extension T2 Tumor has parapharyngeal extension (posterolateral infiltration of tumor, i.e. beyond the pharyngobasilar fascia) T3 Tumor grown to involve bony structures and/or paranasal sinusesT4 Tumor grown into the intracranial extension and/or involvement of cranial nerves, <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none; text-indent: 0.25in;">infratemporal fossa, hypopharynx, orbit, or masticator space.[8] <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none;">Nodes For head and neck cancer the “Regional lymph nodes” <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; margin-left: 0.25in; text-align: left; text-decoration: none;">Nx Lymph nodes cannot be evaluatedN0 No evidence of cancer in the regional lymph nodes <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none; text-indent: 0.25in;">N1 Unilateral lymph node involvement, 6 cm or less, above the supraclavicular fossa, and/or retropharyngeal lymph nodes 6 cm or less (unilateral or bilateral) <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none; text-indent: 0.25in;">N2 Bilateral lymph nodes involved, 6 cm or less, above the supraclavicular fossa <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; margin-left: 0.25in; text-align: left; text-decoration: none;">N3a Lymph node greater than 6 cm <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none; text-indent: 0.25in;">N3b Extension of lymph node involvement into the supraclavicular fossa (defined as the triangular region described by Ho, <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none; text-indent: 0.25in;">bounded by the superior margin sternal head of the clavicle, the superior margin of the lateral end of the clavicle, and the point where the neck meets the <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none; text-indent: 0.25in;">shoulder. This includes some of level IV as well as V.) <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none;">M - Indicates if the cancer has extended beyond primary site and spread to another anatomical part of the body. <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; text-align: left; text-decoration: none;">Mx Distant metastasis cannot be evaluated or determine <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; text-align: left; text-decoration: none;">M0 No evidence that cancer has spread to another anatomical site <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; text-align: left; text-decoration: none;">M1 Evidence that cancer has spread to another anatomical site in the body <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none;">Stage 0: Very early cancer (Tis) with no spread to lymph nodes (N0) or distant metastasis (M0).[7] <span style="background-color: transparent; color: #000000; display: block; font-family: 'Times New Roman'; font-size: 16px; text-align: left; text-decoration: none;">[[image:nasopharynx_Stage_0.jpg]] <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none;">Stage I: A noninvasive cancer (T1) with no spread to lymph nodes (N0) and no distant metastasis (M0). [[image:nasopharynx_Stage_1.jpg]]

<span style="background-color: transparent; color: #000000; display: block; font-family: 'Times New Roman'; font-size: 16px; text-align: left; text-decoration: none;"><span style="background-color: transparent; color: #17365d; font-family: 'Times New Roman','serif'; font-size: 16px; text-align: left; text-decoration: none;">Stage III: An invasive cancer (T3) with no spread to regional lymph nodes (N0) or metastasis (M0), as well as invasive cancers (T1, T2, T3) that have spread regional lymph nodes (N1), but have no sign of metastasis (M0). <span style="background-color: transparent; color: #17365d; font-family: 'Times New Roman','serif'; font-size: 16px; line-height: 0px; overflow: hidden; text-align: left; text-decoration: none;"> <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none;">Stage IVA: An invasive cancer (T4a) with either no lymph node involvement (N0) or spread to only a single same-sided lymph node (N1), but no metastasis (M0). It is also used for any cancer (T) with more significant nodal involvement (N2), but no metastasis (M0). <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none;">Stage IVB: An invasive cancer (any T) that has spread to lymph nodes (any N) but has no metastasis (M0). It is also used for any cancer (any T) that is found in lymph nodes and is larger than 6 cm (N3), but no metastasis (MO). <span style="background-color: transparent; color: #000000; display: block; font-family: 'Times New Roman'; font-size: 16px; text-align: left; text-decoration: none;"> <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: normal; text-align: left; text-decoration: none;">Stage IVC: Any tumor (any T, any N) when there is evidence of distant spread (M1). <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: 115%; text-align: left; text-decoration: none;">Illustrations from cancer.net[9,10] || Late: soft tissue fibrosis, trismus, xerostomia, hearing loss, osteoradionecrosis, hypothyroidism, and hypopituitarism (if included) [4] Cranial and cervical sympathetic nerve palsy 0.3-6.0% Brainstem or cervical spine myelopathy 1% Ophthalmologic side effects after 60Gy-opacities in the lens that develop several years later (radiation cataracts) 70Gy-retinopathy 24-108 months after treatment Deafness 1-7%, 8% significant hearing loss, 3% bilateral deafness Osteonecrosis of mandible or maxilla 1% Dental decay occurs frequently Xerostomia in approximately 75% of patients- IMRT can reduce this at 4% per Gy exponentially Trismus 5-10% Fibrosis of subcutaneous tissues of neck with doses of 50Gy [12] || <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Radiation therapy treatment planning: <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Dose: Full field dose: 45 Gy, Posterior neck lymph nodes are treated to 50-60 Gy with 9MeV. Nasopharynx receives 65-75 Gy, the last 20-25 Gy fractions may utilize a higher photon energy such as 18 MV to decrease the dose to the mandible and TMJ. The lower neck and supraclavicular fossa receive 50Gy. Hyperfractionation radiation therapy: 74.4 Gy in 62 twice daily fractions is also used to decrease the radiation damage to the optic nerve. [3,13] <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Special consideration should be given to the temporal lobe in order to prevent necrosis which is attributed to the fractionation schedule. Target volume is defined by the nasopharynx lesion with a 2-3 cm margin, retropharyngeal and cervical lymph nodes on both sides of the patient’s neck, and the base of skull. [13] <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Intensity modulated radiation therapy is used most often when planning nasopharyngeal lesions.[13] <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Conventional radiation therapy: A series of shrinking fields is the technique used when delivering conventional radiation therapy to the nasopharyngeal tumor which allows the dosimetrist to give a high dose to the tumor volume and decrease the dose to the normal structures. A boost is given after 60Gy to the tumor volume and retropharyngeal lymph nodes. The treatment is delivered with energy of 6 megavoltage. The typical field arrangement includes opposed lateral fields with a 5 degree posterior angle to hinder the ipsilateral dose to the middle and external ear and contra lateral lens. The treatment volumes include: nasopharynx, adjacent paraphayngeal tissues, cervical lymphatics, posterior ethmoid cells, sphenoid sinus, and basophenoid, base of skull, posterior pharyngeal wall to the lower pole of the tonsil, upper cervical, mastoid, and posterior cervical lymph nodes along with retropharyngeal lymph nodes. Anterior field is designed so that it is 2 centimeters posterior of the nasal cavity, posterior one third of the maxillary sinus, posterior of ethmoid sinuses, and posterior one fourth of orbit. Posterior field border is behind the spinous processes. The superior border is the entire sphenoid sinus, cavernous sinus and base of skull. The hyoid bone or thyroid notch is used to determine where to place the inferior margin of the upper lateral fields in order to decrease radiation delivered to the larynx. [13,3] <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Brachytherapy: Intraluminal appliances are used to boost the nasopharynx at a dose of 10Gy at a depth of 1 centimeter. [13] <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Nasopharynx Boost: For T1 and T2 lesions the nasopharynx tumor dose totals a dose of 65 Gy and for T3 and T4 tumors the dose goes to 70 to 75 Gy. Both lesions use reduced upper lateral fields for the boost. [3] ||
 * Radiation Side Effects: || Acute: mucositis, dermatiis, xerostomia
 * Prognosis: || <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: 115%; text-align: left; text-decoration: none;">Prognotic factor include cervical lymph node involvement, extent of local infiltration and histology. Some studies have shown a worse prognosis with keratinizing histology over non-keratinizing, however there are also studies that has shown the histology to have no prognotic differrence. The prognosis depends on which part of the nasopharynx is involved and the grade. Survival and local control decreases with advancing T and survival and distal failure are associated with advancing N. Most nasopharyngeal cancers are diagnosed at stage 3 or 4. There have been reports that indicate a better prognosis for patients under the age of 50 and in females.[9] For all people diagnosed with nasopharyngeal cancer, about 5 out of 10 (50%) people, live for at least 5 years. <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: 115%; margin-bottom: 12pt; text-align: left; text-decoration: none;">Below are some statistics for the different stages of nasopharyngeal cancer. <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: 115%; text-align: left; text-decoration: none;">Stage 1 70% 5 year survival <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: 115%; text-align: left; text-decoration: none;">Stage 2 &3 60% 5 year survival <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: 115%; text-align: left; text-decoration: none;">Stage 4 40% 5 year survival <span style="background-color: transparent; color: #17365d; display: block; font-family: 'Times New Roman',serif; font-size: 16px; line-height: 115%; margin-bottom: 12pt; text-align: left; text-decoration: none;">Overall survival rates for people with undifferentiated nasopharyngeal cancer are better than the keratinizing type.[11] ||
 * Treatments: || <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Radiation therapy and chemotherapy are the recommended treatment options for nasopharynx tumors. Surgical resection with an acceptable margin is not achievable due to the anatomy of the nasopharynx. Neck node metastasis may be treated with the combination of cheomradiotherapy and radical neck dissection. [3]
 * TD5/5: || The 5 % risk of complication within 5 years (TD5/5) are listed below for volumes within the irradiated field. [14] ||  ||   ||   ||   ||   ||


 * ** Organ ** || ** 1/3 Volume ** || ** 2/3 Volume ** || ** 3/3 Volume ** || ** Complications ** ||
 * Brain Stem || 6000 cGy || 5300 cGy || 5000 cGy || Necrosis Infarction ||
 * Ear mid/external || 3000 cGy || 3000 cGy || 3000 cGy || acute serous otitis ||
 * Ear mid/external || 5500 cGy || 5500 cGy || 5500 cGy || chronic serous otitis ||
 * Parotid ||  || 3200 cGy || 3200 cGy || xerostomia ||
 * Optic Chiasma & Optic Nerve || No Partial Volume || No Partial Volume || 5000 cGy || Blindness ||



<span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 140%;">__**GREEN GROUP**__ <span style="color: #ff00a5; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Lisa - Signs and Symptoms and Treatment <span style="color: #632423; font-family: 'Times New Roman',Times,serif; font-size: 15px;">Eric - Etiology and Epidemiology <span style="color: #ad14ad; font-family: 'Times New Roman',Times,serif; font-size: 16px; line-height: 23px;">Kim A - Diagnostic procedures <span style="color: #0a3771; font-family: 'Times New Roman',Times,serif; font-size: 16px; line-height: 23px;">LaDonna - Staging, Grading and Prognosis <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 120%;">Curtis - Histology and Lymphatic Drainage <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Shun - TD5/5 and Metastatic Spread

<span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[1] [|http://www.cancer.org/Cancer/NasopharyngealCancer/DetailedGuide/nasopharyngeal-cancer-risk-factors] <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">accessed 5/26/11 Last Medical Review: 04/21/2011 Last Revised: 04/21/2011 <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[2] [] <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">Accessed 5/26/11 <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[3] Chaos KS, Perez CA, Brady KW. //Radiation Oncology-Management Decisions.// 2nd edition. Philiadelphia: Lippincott, Williams & Wilkins. 2002 <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[4] Hansen E, Roach M. //Handbook of Evidence-Based Radiation Oncology.// New York: Springer 2007 <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[5] Washington, Charles, and Dennis Leaver. Principles and Practices of Radiation Therapy.St. Louis,Missouri: Mosby Elsevier, 2010. Print <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[6 ]Lu,JJ, Cooper, JS, Lee, AWM, Brady LW, Heilmann, HP, Molls M, Nieder C. //Nasopharyngeal Cancer: Multidisciplinary Management.//1st edition. Springer, 2009: 45-46. <span style="font-family: 'Times New Roman',serif; font-size: 16px; line-height: 20px;">[7] Cancer Research UK. //Cancerhelp//. Available at:__ [] __Accessed May 19, 2011. <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[8] American Cancer Society. //Nasal cavity and paranasal sinuses cancer//. Available at:__ [] __Accessed May 18, 2011. <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[9] Cancer.net (n.d). Nasopharangeal Cancer. Available at [] Accessed May 19, 2011. <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[10] MedlinePlus. //Trusted health information for you. Nasal cancer.// Available at:__ [] __Accessed on May 19, 2011. <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[11] Cancer.net. (n.d.). //Nasopharangeal Cancer//. Available at: http://www.cancer.net/patient/Cancer+Types/Nasopharyngeal+Cancer?sectionTitle=Patient Information Resources Accessed May 19, 2011. <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[12] Halperin E, Perez CA, Brady LW. //Perez and Brady's Principles and Practice of Radiation Oncology//. 5th edition. Philadelphia:Lippincott, Williams & Wilkins. 2008 <span style="font-family: 'Times New Roman',serif; font-size: 12pt;">[13] Khan FM. //Treatment Planning in Radiation Oncology//. 2nd ed. Philadelphia, PA; Lippincott Williams & Wilkins; 2007.

<span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">[14] Emami B, Lyman J, Brown A, et al. Tolerance of normal tissue to therapeutic irradiation. //Int J Radiat Oncol Biol Phys//.1991;21(1):109-122.

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