Nasopharyngeal carcinoma
| Classification according to ICD-10 | |
|---|---|
| C11 | Malignant neoplasm of the nasopharynx |
| C11.0 | Upper wall of the nasopharynx, roof of the nasopharynx |
| C11.1 | Posterior wall of the nasopharynx, adenoids = pharynx tonsil |
| C11.2 | Side wall of the nasopharynx
|
| C11.3 | Anterior wall of the nasopharynx
|
| C11.8 | Nasopharynx, overlapping several areas |
| C11.9 | Nasopharynx, unspecified |
| ICD-10 online (WHO version 2019) | |
A nasopharyngeal carcinoma , also called epipharynx carcinoma or German nasopharynx cancer and often NPC for English. Nasopharyngeal Carcinoma, for short, is a cancer of the nasopharynx ( nasopharynx or epipharynx or pars nasalis pharyngis ). Nasopharyngeal cancer belongs to the group of head and neck tumors .
The nasopharynx is the upper part of the pharynx, which is divided into three areas (Greek pharynx ).
Epidemiology
Cancer of the nasopharynx is quite rare in Central Europe and North America . The incidence ranges from 0.5 to 1 in 100,000. This corresponds to a share of 0.2% of all tumor diseases. Nasopharyngeal cancer is endemic in some countries and areas, such as Taiwan , southern China , Southeast Asia, and parts of northern Africa , with an incidence of 30 per 100,000 . In Taiwan, nasopharyngeal cancer accounts for about 18% of all cancers. There, nasopharyngeal carcinoma is now the leading cause of death in young men. One reason seems to be the consumption of betel nuts .
While middle-aged people are mainly affected in Asia, children represent a high proportion of the newly infected in Africa. The causes of the increased risk of the disease in these countries are not yet fully understood.
Men are more often affected by the disease than women. The ratio is about 2: 1.
etiology
The causes or triggers of endemic nasopharyngeal cancer are the Epstein-Barr virus (EBV), various environmental factors, dietary habits and genetic characteristics of the patient.
Since positive IgA antibody titers against the EBV virus capsid antigen and elevated IgG antibodies against the EBV early antigen are found in 80 to 90% of patients suffering from nasopharyngeal carcinoma, there is an epidemiological close relationship between infection with the EBV virus and the Nasopharyngeal carcinoma. The Epstein-Barr virus (EBV) is also involved in the development of other forms of cancer, such as Hodgkin's disease , Burkitt's lymphoma, and HIV- associated lymphoma .
In terms of eating habits in Asia, the consumption of salted dried fish , which in some cases has a relatively high content of carcinogenic nitrosamines , was identified as a risk factor.
Well-differentiated nasopharyngeal carcinomas, the cells of which are histologically very similar to other squamous cell carcinomas of the head and neck area, can be assigned to the standard risk factors for cancer, such as nicotine abuse ( tobacco smoke ), especially in combination with high-proof alcoholic beverages.
Classification and histology
Nasopharyngeal cancer is a malignant neoplasm (cancer), around 90% of which is carcinoma. They are of epithelial origin ( squamous cell carcinoma ).
In histology, a distinction is made between three subtypes of nasopharyngeal cancer:
- a keratinizing, well-differentiated squamous cell carcinoma of WHO type 1 (approx. 20% of nasopharyngeal carcinomas)
- a non-keratinizing squamous cell carcinoma of WHO type 2 (approx. 30-40%)
- an undifferentiated form of WHO type 3 ( Schmincke-Regaud tumor or also called Schmincke tumor ), a lymphoepithelial carcinoma that typically contains non-malignant lymphocytes . This type can also occur on the tonsils and the hypopharynx . This undifferentiated form is most common (40–50%) and is directly related to a previous infection with the Epstein-Barr virus .
Malignant lymphomas , adenocarcinomas, or sarcomas are rarely found. This article is essentially limited to the undifferentiated carcinomas and squamous cell carcinomas.
Symptoms
Nasopharyngeal cancer occurs in all age groups. A clear accumulation can be observed in the age group in the range of 40 to 60 year olds. Because of their low symptoms, the tumors are usually only discovered very late in an advanced tumor stage. Epistaxis, difficulty breathing through the nose, and middle ear effusion or otitis media may be early symptoms . The disease often only becomes symptomatic through metastases in the cervical lymph nodes or paralysis of the cranial nerves III to VI through infiltration of the skull base . For this reason, in over 60% of patients, the main symptom at the time of diagnosis is enlarged cervical lymph nodes. Regional lymph node metastases are often found even with a very small primary tumor. Distant metastases are found in the bones , lungs, or liver more often than in other ENT tumors .
Nasopharyngeal carcinomas usually develop from the side walls and roof of the nasopharynx. From there, they spread early into the nasal cavity , the paranasal sinuses and the cranial nerves . In contrast, metastasis on the bloodstream is relatively rare. Even in the advanced stages of the disease, distant metastases are only diagnosed in 30% of patients.
diagnosis
The standard examination is endoscopy of the nasopharynx. Biopsies from the nasopharynx can be taken in this way.
Magnetic resonance imaging (MRI) is the imaging method of choice for assessing the extent of the primary tumor and possible regional lymph node metastases . If an infiltration of the skull base is suspected , computed tomography (CT) should be performed in a special bone window. In this case, CT is superior to MRI in assessing the extent of the tumor. Distant metastases in the bones, liver and lungs can be diagnosed using skeletal scintigraphy or upper abdominal sonography (ultrasound).
therapy
The nasopharynx is extremely difficult to access surgically . In addition, when the diagnosis is made, nasopharyngeal cancer is often so advanced that an operation can no longer be performed.
radiotherapy
The drug of choice for treating nasopharyngeal cancer is radiation therapy . The tumor is irradiated with locally very high doses of around 70 Gy in several sessions.
Radiotherapy responds very well to the non-keratinous and undifferentiated nasopharyngeal carcinoma subtype, while this is less the case with the keratinizing subtype.
chemotherapy
In a more recent study it could be shown that with locally advanced tumors an accompanying chemotherapy with the radiation therapy brought a clear advantage for the patients. As a chemotherapeutic agent is oxaliplatin at a dose of 70 mg / m 2 used per week.
surgery
Surgical removal of the primary tumor is usually not useful because of the often submucosal ("located below the mucous membrane") spread and difficult accessibility of the tumor. A complete resection is hardly possible with this form of cancer.
A removal of all cervical lymph nodes, a so-called neck dissection , only rarely has to be carried out.
forecast
As with all malignant neoplasms, the healing rates are strongly dependent on the tumor stage. In patients with stage I tumors, they are between 70 and 80%, in Schmincke-Regaud tumors - not least because of its high radiation sensitivity - well over 90% can be treated successfully if detected early. In stage IV, the rate drops to 20 to 40%.
The five-year survival rate of patients with non-keratinizing and undifferentiated nasopharyngeal cancer is about 65% with adequate therapy. Due to the high radiation sensitivity of the malignant tissue, even if the disease has already established itself in regional lymph nodes, there are very good chances of recovery. However, the prognosis is significantly worse for keratinizing nasopharyngeal cancer, as this form is considerably more resistant to radiation.
literature
- W. Arnold: Comparative Morphology of Lymphoepithelial Carcinoma Primary Tumor - Metastasis - Xenograft. In: European Archives of Oto-Rhino-Laryngology. 226/1980, ISSN 0937-4477 .
- C. Ilberg et al: The "Schmincke" carcinoma in the nasopharynx. In: European Archives of Oto-Rhino-Laryngology. 210/1975, pp. 296–298, ISSN 0937-4477 (print) ISSN 1434-4726 (online)
- I. Ganly et al .: Recurrent squamous-cell carcinoma of the head and neck: overview of current therapy and future prospects. In: Ann Oncol . 11/2000, pp. 11-16.
- RM Pryzant et al .: Re-treatment of nasopharyngeal carcinoma in 53 patients. In: Int J Radiat Oncol Biol Phys . 22/1992, pp. 941-947.
- A. Fandi et al.: Nasopharyngeal cancer: epidemiology, staging, and treatment. In: Semin Oncol . 21/1994, pp. 382-397.
- B. Brennan: Nasopharyngeal carcinoma. In: Orphanet J Rare Dis. Volume 1, Jun 26, 2006, p. 23. PMID 16800883 , PMC 1559589 (free full text)
Individual evidence
- ↑ a b c d e f g M. Dellian: Malignomas of the nasopharynx ( Memento from February 26, 2005 in the Internet Archive ). In: MANUAL head and neck malignancies. Munich Tumor Center, accessed on August 9, 2007.
- ^ C. Titcomb: High incidence of nasopharyngeal carcinoma in Asia. In: J Insur Med. 33/2001, pp. 235-238. PMID 11558403
- ↑ J. Jeng et al: Role of areca nut in betel quid-associated chemical carcinogenesis: current awareness and future perspectives. In: Oral Oncol . 37/2001, pp. 477-492. PMID 11435174
- ↑ MA Vasef et al.: Nasopharyngeal carcinoma, with emphasis on its relationship to Epstein-Barr virus. In: Ann Otol Rhinol Laryngol. 106/1007, pp. 348-356.
- ↑ EE Vokes et al.: Nasopharyngeal carcinoma. In: The Lancet . 350/1997, pp. 1087-1091.
- ^ R. Cote et al.: Modern Surgical Pathology. WB Saunders, London, ISBN 0-7216-7253-1 .
- ^ IF Tannock et al: The Basic Science of Oncology. 4th edition. McGraw Hill, 2005, ISBN 0-07-138774-9 .
- ↑ Page no longer available , search in web archives: Academic Teaching Hospital Munich Nymphenburg: What is throat cancer? Retrieved August 9, 2007.
- ↑ a b c Biowellmed.de: Nasopharyngeal carcinoma , accessed on August 9, 2007
- ^ AT Chan et al .: Concurrent chemotherapy-radiotherapy compared with radiotherapy alone in locoregionally advanced nasopharyngeal carcinoma: progression-free survival analysis of a phase III randomized trial. In: J Clin Oncol . 20/2002, pp. 2038-2044.
- ↑ EE Vokes et al .: Concomitant chemoradiotherapy as primary therapy for locoregionally advanced head and neck cancer. In: J Clin Oncol. 18/2000, pp. 1652-1661.
