Nasopharyngeal carcinoma


A squamous cell cancer originating in the fossa of Rosenmuller, with a high prevalence in southern Chinese.

An  epithelial tissue derived  malignant neoplasm.

Most common cancer of the head and neck in southeastern part of China, Taiwan, Hong Kong and Singapore.

It is a rare form of head and neck cancer in the West, with an annual incidence of less than 1 per 100,000 individuals in the US, while it is much more prevalent in southern China and Southeast Asia, with an annual incidence of about 9/100,000.

Related to the Epstein-barr virus.

Latent infection with EBV, is crucial for the development of NPC, with environmental factors, such as tobacco and alcohol consumption, playing lesser roles.

Screening for either EBV specific antibodies or  ENV DNA can increase the percentage of patients in whom nasopharyngeal carcinoma is diagnosed it an early stage from approximately 20% to more than 70%.

Associated with abundant lymphocytic infiltration, high programmed death ligand numeral 1 (PD – L1) expression, and the presence of several immune targets CD 40, CD 70, CD, 80, and CD 86 suggest the strong biological rationale for immunotherapy in the treatment of nasopharyngealcarcinoma.

95% of cases in endemic regions are of differentiated or undifferentiated nonkeratinizing type, which is very closely associated with infection with Epstein-Barr virus.

Worldwide,approximately 129,000 new cases of NPC and 73,000 deaths attributed to this disease or reported annually.

Accounted for 0.6% of all cancer is diagnosed worldwide in 2012.

Highest prevalence at 20-50 cases per 100,000 in south China.

Highest rates occur in South China, Southeast Asia, and North Africa.

Lowest prevalence in Europe and US.

Annual incidence 0.6 per 100,000 population in the U.S.

Rates are 2-3 times higher in men than women.

Has one of the highest propensities for metastases among head and neck cancers.

The incidents of synchronous metastatic disease is about 5% in newly diagnosed patients.

About 20% of patients with nonmetastatic NPC develop distant failure after definitive treatments, which remains a main cause of death in these patients.

Most patients present with local regional advanced disease with a proclivity for metastatic recurrence to bones, liver, and lungs.

Approximately 1/4 of patients  with NPC eventually experience metastatic diseases that require systemic therapy.

More than 70% of patients have local regional advanced disease at presentation, and this is associated with an unfavorable prognosis.

Intra-tumoral bacterial load is associated with poor survival and patients with nasopharyngeal carcinoma and is negatively associated with T-lymphocyte infiltration.

Regional recurrences are uncommon, and occur only in 10-19% of patients.

Stage I-5-year survival 70% and 50% for Stage II.

Its location is contiguous with critical neural structures at the base of the skull.

External radiation is the primary treatment.

30% of patients have distant metastases or large cervical lymph nodes on presentation.

Approximately 70% of patients present with stage III or IV disease at the time of diagnosis (Lee AWM et al).

Locoregional failure is the main cause of death.

19-29% of patients will have distant metastases with controlled local regional disease.

35% die of local disease or local recurrence despite radiotherapy.

Distant metastases is the major cause of mortality.

Induction chemotherapy with radiation therapy is the standard of care although most studies do not show survival advantage for combination treatment.

Induction chemotherapy followed by radiation is noninferior to combination chemoradiotherapy upfront.

Platinum based chemotherapy is the frontline treatment for patients with metastatic NPC.

Median progression free survival after first line treatment ranges from 5 to 7 months.

Concurrent chemoradiation therapy with the platinum-based agent is the primary treatment, with the chemotherapy sensitizing the tumor to the toxic effects of radiotherapy.

Among patients with low risk NPC, treatment with radio therapy alone results in a three-year failure free survival that is not inferior to concurrent chemoradio therapy; stage II.

The addition of induction therapy with gemcitabine plus cis-platinum to a back bone of chemoradiotherapy with cisplatin, administered every three weeks improved recurrence free survival among patients with high risk local regional advance nasal pharyngeal cancer (Zhang Y).

Active chemotherapy agents including cisplatinum, fluorouracil, gemcitabine, paclitaxel, and capecitabine.

Distant metastases predominate as a pattern of relapse and accounts for cancer specific mortality among approximately 70% of patients.

Gemcitabine-cisplatinum chemotherapy is the current standard first line treatment for patients with recurrent or metastatic NPC.

((Toripalimab)) is a monoclonal antibody that blocks programmed cell death protein and approved  for recurrent/nasopharyngeal Cancer .

Capecitabine maintenance therapy may prolong progression free survival.

Capecitabine is  an effective adjuvant chemotherapy.

The addition of toripalimab (a monoclonal PDL-1 inhibitor) to chemotherapy as first line treatment of recurrent/metastatic NPC provided significant clinically meaning full progression free, survival and overall survival benefits compared with chemotherapy alone: supports the use of immune checkpoint, inhibitors plus gemcitabine+cisplatinum as new standard of care.



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