Nasopharyngeal Darcinoma

Nasopharyngeal Darcinoma

Summarize

Nasopharyngeal carcinoma, which develops on the inner walls of the nasal cavity and pharynx, accounts for about one-third of all cases of upper respiratory tract cancer in children. This type of cancer is very rare in children under 10 years old, but it affects 8 out of every 10 million people aged 10 to 14 annually; and 13 out of every 10 million people aged 15 to 19 annually.

Epidemiological

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Etiology & Risk Factors

Risk factors for disease

        Nasopharyngeal carcinoma (NPC) is closely linked to EB virus (EBV) infection. Over 98% of patients can be detected with the EBV virus in their serum. NPC cells contain EBV DNA, and the tumor cells exhibit EBV antigens on their surface. The levels of EBV DNA in the patient's circulation and the serological records of EBV infection may aid in diagnosis. Specific HLA subtypes, such as the HLA A2Bsin2 haplotype, are associated with a higher risk of NPC.

Classification & Staging

 histology

        The World Health Organization (WHO) has classified nasopharyngeal carcinoma into three histological subtypes:

50. Type I is keratotic squamous cell carcinoma. ​
50. Type II is non-keratinizing squamous cell carcinoma. Type II is classified as IIA or IIB depending on the presence of lymphoid infiltration.
50. Type III is undifferentiated carcinoma. Type III is classified as type IIIA or Type IIIB depending on the presence of lymphoid infiltration.
50. Children with nasopharyngeal carcinoma are more likely to have WHO type II or III disease.

Clinical Manifestations

 clinlcal symptom

        Symptoms of nasopharyngeal cancer include:

50. Cervical lymphadenopathy ​
50.  nose bleeding
50. Nasal congestion and obstruction
50.  headache
50.  otalgia
50.  tympanitis

        Due to the rich lymphatic drainage in the nasopharynx, enlarged bilateral cervical lymph nodes are often the first sign of the disease. If the tumor spreads locally to the oropharyngeal region, it may extend to the base of the skull, causing cranial nerve paralysis or jaw movement disorders (such as inability to open the mouth or restricted opening). The potential sites for distant metastasis of nasopharyngeal cancer include bones, lungs, and liver.

Clinical Department

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Examination & Diagnosis

Diagnosis and staging assessment

        The purpose of the diagnostic examination is to determine the status of the primary tumor and whether there is metastasis. The otolaryngologist can observe the nasopharynx of the patient through nasal endoscopy and head and neck magnetic resonance imaging to confirm the status of the primary tumor.

        Doctors can diagnose nasopharyngeal cancer based on the results of a biopsy from the primary tumor or enlarged cervical lymph nodes. To differentiate nasopharyngeal cancer from other cancers that may cause lymph node enlargement or other types of head and neck cancers, it is important to consider conditions such as thyroid cancer, rhabdomyosarcoma, non-Hodgkin's lymphoma (including Burkitt's lymphoma), Hodgkin's lymphoma, and benign conditions like nasal angiofibroma (common in adolescent males, characterized by nosebleeds, infectious lymphadenitis, and giant lymph node sinus histiocytosis).

        Chest and abdominal areas may be evaluated by computed tomography (CT) and bone scans to determine if there is cancer metastasis. Fluorine F-18-fluorodeoxyglucose positron emission tomography (PET)-CT may also help assess potential metastatic lesions.

Clinical Management

Treatment of newly diagnosed children with nasopharyngeal carcinoma

        The treatment of nasopharyngeal carcinoma is a combination of various treatment methods, including the following:

50. Chemotherapy and radiotherapy: High-dose radiotherapy alone is effective in the treatment of nasopharyngeal carcinoma, however, studies in children and adults have shown that combination chemotherapy and radiotherapy is the most effective treatment for nasopharyngeal carcinoma.
50. Surgery: Surgery has limited effectiveness in treating nasopharyngeal carcinoma due to its widespread local spread, which is generally considered inoperable. A combination of cisplatin-based chemotherapy and high-dose radiotherapy is used in the nasopharynx and neck, but this approach is associated with a range of side effects, including hearing loss, hypothyroidism, and panhypopituitarism, as well as issues such as bruxism, dry mouth, dental problems, and chronic sinus or otitis media.

Treatment of refractory pediatric nasopharyngeal carcinoma

        In view of the unique pathogenesis of nasopharyngeal carcinoma, trials of immunotherapy in patients with refractory disease have been initiated, some examples are as follows:

50. Two phase II clinical trials of anti-procedural death ligand 1 (PD-L1) monoclonal antibodies in adults with refractory nasopharyngeal carcinoma have shown a response rate of 20.5-25.90% (33% in PD-L1-positive tumors) and evidence of long-term remission.

Prognosis

Over the past 40 years, the overall survival of children and adolescents with nasopharyngeal cancer has improved; with the use of advanced multimodal treatments, the 5-year survival rate has surpassed 80%. In the same staging, children with nasopharyngeal cancer have a significantly better prognosis than adults. However, the frequent use of chemotherapy and radiotherapy can also lead to severe acute or long-term complications, including secondary tumors.

Follow-up & Review

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Daily Care

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Cutting-edge therapeutic and clinical Trials

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References

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Audit specialists

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