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      Nasopharyngeal carcinoma in Indonesia: epidemiology, incidence, signs, and symptoms at presentation

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          Among all head and neck (H&N) cancers, nasopharyngeal carcinoma (NPC) represents a distinct entity regarding epidemiology, clinical presentation, biological markers, carcinogenic risk factors, and prognostic factors. NPC is endemic in certain regions of the world, especially in Southeast Asia, and has a poor prognosis. In Indonesia, the recorded mean prevalence is 6.2/100 000, with 13 000 yearly new NPC cases, but otherwise little is documented on NPC in Indonesia. Here, we report on a group of 1121 NPC patients diagnosed and treated at Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia between 1996 and 2005. We studied NPC incidence among all H&N cancer cases ( n=6000) observed in that period, focusing on age and gender distribution, the ethnic background of patients, and the disease etiology. We also analyzed most prevalent signs and symptoms and staging of NPC patients at first presentation. In this study population, NPC was the most frequent H&N cancer (28.4%), with a male-to-female ratio of 2.4, and was endemic in the Javanese population. Interestingly, NPC appeared to affect patients at a relatively young age (20% juvenile cases) without a bimodal age distribution. Mostly, NPC initiated in the fossa of Rosenmuller and spreaded intracranially or locally as a mass in the head. Occasionally, NPC developed at the submucosal level spreading outside the anatomic limits of the nasopharynx. At presentation, NPC associated with hearing problems, serous otitis media, tinnitus, nasal obstruction, anosmia, bleeding, difficulty in swallowing and dysphonia, and even eye symptoms with diplopia and pain. The initial diagnosis is difficult to make because early signs and symptoms of NPC are not specific to the disease. Early-age Epstein-Barr virus (EBV) infection combined with frequent exposure to environmental carcinogenic co-factors is suggested to cause NPC development. Undifferentiated NPC is the most frequent histological type and is closely associated with EBV. Expression of the EBV-encoded latent membrane protein 1(LMP1) Oncogene in biopsy material was compared between NPC patients of < 30 years old and those of ≥ 30 years old, matched for sex and tumor stage. Higher LMP1 expression in patients of <30 years old was observed, which was related to more locoregional progressivity. Increased medical awareness of prevailing early stage signs and symptoms coupled to use of EBV-related diagnostic tumor markers may lead to down-staging and timely treatment to improve survival of patients with this aggressive disease.

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          Most cited references 50

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          Focus on nasopharyngeal carcinoma.

           Kwok Lo,  Ka To,  Dolly P Huang (2004)
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            Epidemiology of nasopharyngeal carcinoma.

            Nasopharyngeal carcinoma (NPC) is a rare malignancy in most parts of the world, with an incidence well under 1 per 100,000 person-years. Exceptions are the Chinese, especially the Cantonese living in the central region of Guangdong Province in Southern China. Other populations with elevated rates include the natives of Southeast Asia, the natives of the Artic region, and the Arabs of North Africa and parts of the Middle East. Intake of preserved foods at an early age has been linked to NPC risk in all population groups with increased NPC rates. Other recognized risk factors for NPC are cigarette smoking, and occupational exposure to formaldehyde and wood dust. Copyright 2002 Elsevier Science Ltd.
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              Epstein-Barr virus in the pathogenesis of NPC.

              Epstein-Barr virus (EBV) is consistently detected in nasopharyngeal carcinoma (NPC) from regions of high and low incidence. EBV DNA within the tumor is homogeneous with regard to the number of terminal repeats. The detection of a single form of viral DNA suggests that the tumors are clonal proliferations of a single cell that was initially infected with EBV. Specific EBV genes are consistently expressed within the NPC tumors and in early, dysplastic lesions. The viral proteins, latent membrane protein 1 and 2, have profound effects on cellular gene expression and cellular growth, resulting in the highly invasive, malignant growth of NPC tumors. In addition to potential genetic changes, the establishment of a latent, transforming infection in epithelial cells is likely to be a major contributing factor to the development of this tumor. Copyright 2002 Elsevier Science Ltd.

                Author and article information

                Chin J Cancer
                Chin J Cancer
                Chinese Journal of Cancer
                Sun Yat-sen University Cancer Center
                April 2012
                : 31
                : 4
                : 185-196
                [1 ]Department of Otorhinolaryngology,
                [2 ]Department of Anatomy Pathology,
                [3 ]Department of Radiotherapy, Faculty of Medicine, University of Indonesia, Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia;
                [4 ]Antoni van Leeuwenhoek Hospital, Netherlands Cancer Institute,
                [5 ]Department of Pathology, VU University Medical Center, Amsterdam, Netherlands.
                Author notes
                Corresponding Author: Jaap M. Middeldorp, Department of Pathology, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands. Tel: +31-204444052; Fax: +31-20-4442964; Email: j.middeldorp@ 123456vumc.nl .
                Chinese Journal of Cancer

                This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 Unported License, which allows readers to alter, transform, or build upon the article and then distribute the resulting work under the same or similar license to this one. The work must be attributed back to the original author and commercial use is not permitted without specific permission.

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