Nasopharyngeal Cancer Treatment & Management

  • Author: Arnold C Paulino, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Apr 26, 2012
 

Medical Care

Radiation therapy is the mainstay of treatment, with chemotherapy used in advanced cases. Concurrent cisplatin, 5-fluorouracil, and radiotherapy have been shown to improve survival.[15, 16, 17] Many pediatric studies have used neoadjuvant chemotherapy followed by radiation therapy with improvement in local control or progression-free survival rates over radiotherapy alone.[18, 19, 20]

Radiotherapy is administered to the gross tumor volume (GTV) or disease in the primary site and neck adenopathy. The initial clinical target volume (CTV) includes the GTV and all sites of potential subclinical disease to a dose of 45-50.4 Gy at 1.8-2 Gy/fraction. It is anticipated that in most patients, levels I to V neck nodes are included in the initial CTV. For the boost CTV, the GTV with a margin is treated to a dose of 15-25 Gy. The total dose to the GTV usually ranges from 65-70 Gy. A planning target volume (PTV) takes into account the CTV and daily set-up variation; in most cases with an immobilization mask for the head and neck, a 0.5-cm margin is added to the CTV to create the PTV. The PTV receives the same prescription dose as the CTV.

In the past, the initial radiotherapy fields were treated with 2 parallel opposed lateral fields that encompassed the nasopharynx and upper cervical nodes. The lower cervical nodes were treated with an anterior field, which abuts the upper lateral fields superiorly. These patients are now commonly treated with intensity-modulated radiation therapy (IMRT). IMRT may be used to spare neighboring critical structures next to the nasopharynx, such as the brain, pituitary gland, optic chiasm, optic nerve, and spinal cord.

A multi-institutional study showed that doses of at least 66 Gy to gross disease are needed for optimal local control.[21] Others have used a radiotherapy dose adaptation strategy with which children responding well to chemotherapy receive less radiotherapy dose. In the Italian Rare Tumors in Pediatric age (TREP) Project, 3 courses of cisplatin and 5-fluorouracil followed by radiotherapy, with doses ranging from 60-65 Gy to gross disease, resulted in a 5-year progression-free survival rate of 79.3%.[22] In another study, cervical nodal irradiation was reduced to less than 50 Gy, with good response to chemotherapy (>90% shrinkage of original tumor) with a 5-year, event-free survival rate of 75%.[23]

Data using IMRT reveal equivalent or better locoregional control compared with conventional radiotherapy and sparing of the parotid glands from high doses of radiation therapy.[24, 25]

During the course of radiotherapy, several immediate effects may occur, usually after the first 2 weeks of treatment. Confluent mucositis usually occurs, especially in children receiving both radiotherapy and cisplatin. Dry mouth and thick saliva are also likely secondary to irradiation of the salivary glands. Because of this oropharyngeal mucositis, consideration of placement of a gastrostomy tube prior to initiation of radiotherapy. If a gastrostomy tube is not placed, poor nutrition and dehydration are quite common, and intravenous fluids may need to be administered. Redness, itching, and peeling of the treated skin can occur towards the end of radiotherapy and may need to be treated with topical antibiotics and Silvadene. A study of children treated with IMRT showed less acute toxicity (skin, mucous membrane, pharynx) compared with conventional radiotherapy.[26]

Some centers use amifostine, a radioprotective agent, to help reduce radiation-related xerostomia. Possible adverse effects of amifostine such as flulike symptoms, nausea, low calcium levels, and hypotension have limited its widespread use in the oncologic community.

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Surgical Care

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.

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Consultations

Consultation with an otolaryngologist is often required in the initial management to obtain tissue diagnosis and in follow-up endoscopic examinations to rule out recurrence. An otolaryngologist may also be involved if the child develops sensorineural hearing loss from cisplatin and radiotherapy.

Consultation with an endocrinologist may be required in the future if the child shows signs of growth retardation or hypothyroidism secondary to radiotherapy.[24] Occasionally, children develop panhypopituitarism.

Consultation with a dentist familiar with radiation effects should be performed prior to initiation of radiotherapy to minimize risk of osteoradionecrosis. Patients also need to be followed after treatment as xerostomia and change in salivary consistency may predispose the patient to dental caries.

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Diet

Many patients experience severe mucositis during radiotherapy. Certain foods may irritate irradiated mucosa, causing pain or difficulty swallowing or chewing. Soft foods such as milkshakes, mashed potatoes, and pureed meats are advisable during the course of radiotherapy. Citrus fruits, spicy foods, salty foods, and coarse foods can make the irritated mucosa worse. Gastrostomy tube placement allows adequate hydration and calorie intake during radiotherapy.

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Activity

Activity depends on the child's condition. During periods of chemotherapy-induced thrombocytopenia, some limitation of strenuous activity and avoidance of contact sports is necessary. Infectious contacts should be avoided where possible, especially during periods of neutropenia.

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Contributor Information and Disclosures
Author

Arnold C Paulino, MD  Professor of Radiation Oncology, Methodist Hospital and Weill-Cornell Medical College; Associate Professor of Pediatrics, Baylor College of Medicine

Arnold C Paulino, MD is a member of the following medical societies: American Medical Association, American Radium Society, American Society for Therapeutic Radiology and Oncology, American Society of Clinical Oncology, Children's Oncology Group, International Society of Paediatric Oncology, and Radiological Society of North America

Disclosure: Nothing to disclose.

Coauthor(s)

Chrystal U Louis, MD, MPH  Assistant Professor, Texas Children's Cancer Center and Hematology Service, Center for Cell and Gene Therapy, Baylor College of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD  Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology

Disclosure: Nothing to disclose.

Helen SI Chan, MBBS, FRCP(C), FAAP  Associate Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Canada

Helen SI Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

References

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MRI of the head and neck in a patient with nasopharyngeal carcinoma showing the primary tumor and cervical lymph node metastases
Intensity modulated radiotherapy images for a patient with nasopharyngeal carcinoma
Table 1. AJCC Staging for Nasopharyngeal Cancer
StageTNM
0TisNoM0
IT1N0M0
IIT1N1M0
T2N0M0
T2N1M0
IIIT1N2M0
T2N2M0
T3N0M0
T3N1M0
T3N2M0
IVAT4N0M0
T4N1M0
T4N2M0
IVBAny TN3M0
IVCAny TAny NMI
Table 2. Tumor (T) Staging
TXPrimary tumor cannot be assessed
T0No evidence of primary tumor
TisCarcinoma in situ
T1Tumor confined to the nasopharynx or extends to oropharynx and/or nasal cavity without parapharyngeal extension
T2Tumor with parapharyngeal extension
T3Tumor involves bony structures of skull base and/or paranasal sinuses
T4Tumor with intracranial extension and/or involvement of cranial nerves, hypopharynx, orbit, or with extension to the infratemporal fossa/masticator space
Table 3. Nodal (N) Staging
NXRegional lymph nodes cannot be assessed
N0No regional lymph node metastasis
N1Unilateral 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
N2Bilateral metastasis in a cervical lymph node (s), less than or equal to 6 cm in greatest dimension, above the supraclavicular fossa
N3Metastasis in a lymph node(s) greater than 6 cm and/or to supraclavicular fossa
N3aGreater than 6 cm in dimension
N3bExtension to supraclavicular fossa
Table 4. Metastasis (M) Staging
M0No distant metastasis
M1Distant metastasis
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