Juvenile nasopharyngeal fibroma is a tumor of benign to malignant nature. Juvenile nasopharyngeal fibroma develops in the area of the roof of the throat. In the majority of cases, Juvenile Nasopharyngeal Fibroma affects boys after the age of ten. Juvenile nasopharyngeal fibroma belongs to the angiofibromas and thus represents a fibroma with numerous vessels.
What is juvenile nasopharyngeal fibroma?
Juvenile nasopharyngeal fibroma is also known by the synonymous names basal fibroid and juvenile angiofibroma. From a histologic perspective, juvenile nasopharyngeal fibroma is a benign tumor. However, due to its aggressive growth behavior, Juvenile Nasopharyngeal Fibroma is considered a malignant tumor from a clinical perspective. This is because Juvenile Nasopharyngeal Fibroma displaces and destroys structures in the paranasal sinuses, nose, orbit, and pterygopalatine fossa through its growth. Basically, Juvenile Nasopharyngeal Fibroma is located at the roof of the pharynx or in the lateral region of the nasopharynx, respectively. Juvenile nasopharyngeal fibroma occurs frequently in males during childhood. In this case, the malignant tumor is mainly seen in boys older than ten years.
Causes
The causes and processes of the pathogenesis of juvenile nasopharyngeal fibroma are not well understood at the present time. On the one hand, genetic factors may be considered for the development of juvenile nasopharyngeal fibroma. On the other hand, Juvenile Nasopharyngeal Fibroma possibly develops due to external influences on the affected individuals. Also, why juvenile nasopharyngeal fibroma occurs more frequently in male patients of a certain age group is not exactly known at present.
Symptoms, complaints, and signs
Juvenile nasopharyngeal fibroma is principally a benign tumor because it does not metastasize. However, its growth pattern destroys other structures in the nose and pharynx, so from a clinical point of view, juvenile nasopharyngeal fibroma is usually considered a malignant tumor. Juvenile nasopharyngeal fibroma has numerous vessels and forms from connective tissue. Usually, juvenile nasopharyngeal fibroma arises from fibrocartilago basilaris and sphenopalatine artery. In this case, the tumor is specifically located on the pharyngeal roof or the lateral area of the nasopharynx. Juvenile nasopharyngeal fibroma spreads primarily locally, exhibiting a relatively aggressive growth pattern. Here, Juvenile Nasopharyngeal Fibroma displaces other structures in the surrounding area and increasingly spreads toward the base of the skull, nose and paranasal sinuses, cavernous sinus, and pterygopalatine fossa. Juvenile nasopharyngeal fibroma causes numerous symptoms of varying severity in mostly child patients. Initially, juvenile nasopharyngeal fibroma impairs breathing through the nose. In addition, purulent rhinitis and rhinophonia clausea develop as a result of juvenile nasopharyngeal fibroma. Juvenile nasopharyngeal fibroma also disturbs the function of the tubes in most cases. Sometimes patients suffer from conductive hearing loss due to Juvenile Nasopharyngeal Fibroma. Other classic symptoms of the tumor consist of headaches and frequent nosebleeds. If infiltration of the skull base occurs, the first six nerves of the brain may fail. If Juvenile Nasopharyngeal Fibroma spreads unchecked, a bulge sometimes develops in the area of the facial skull. In Juvenile Nasopharyngeal Fibroma, patients are basically at risk of bleeding to death. The vessels of the tumor rupture quickly, causing nosebleeds and internal bleeding.
Diagnosis and course of the disease
Juvenile nasopharyngeal fibroma is diagnosed by an otolaryngologist, for example. During the initial history, the specialist discusses with the patient and, if necessary, with the patient’s guardians, the onset of symptoms, the onset of initial symptoms, and possible influencing factors that contributed to the development of juvenile nasopharyngeal fibroma. During the clinical examination, posterior rhinoscopy is used. Juvenile nasopharyngeal fibroma appears as a nodular tumor with a grayish-red color and a smooth surface structure. In addition, the numerous vessels on the surface of the juvenile nasopharyngeal fibroma as well as the extension into the nasopharynx and the choanae are visible.In addition, the specialist uses imaging methods such as an MRI examination to precisely determine the localization and extent of juvenile nasopharyngeal fibroma. A biopsy is not usually performed, as this increases the risk of bleeding from injury to the Juvenile Nasopharyngeal Fibroma. Sometimes the physician uses angiography to obtain more information about Juvenile Nasopharyngeal Fibroma. In the differential diagnosis of Juvenile Nasopharyngeal Fibroma, the most important thing to rule out is possible hyperplasia of the pharyngeal tonsil. A choanal polyp as well as a nasopharyngeal cyst should also be considered.
Complications
Nasopharyngeal fibroma can cause a variety of symptoms. These depend thereby strongly on the spread of the tumor. In most cases, however, this results in damage to the nose and throat. Most patients suffer from impaired breathing. Furthermore, the patient may experience shortness of breath, which is associated with a panic attack. It is also not uncommon for the breathing difficulties to lead to fatigue or exhaustion. Furthermore, hearing loss can develop, which can make the patient’s daily life considerably more difficult. It is not uncommon for those affected to also suffer from nosebleeds and severe headaches. These can lead to restrictions in concentration and coordination and thus have an overall negative effect on the patient’s quality of life. Treatment of nasopharyngeal fibroma does not lead to further discomfort or complications. It can be removed by surgery or radiation therapy. The patient’s life expectancy is usually not reduced after the removal. The formation of new tumors is also relatively unlikely. However, the affected person is still dependent on regular examinations.
When should one go to the doctor?
Difficulty breathing or interference with respiratory activity is cause for concern. If no colds are present, a doctor should be consulted if oxygen delivery through the nose is impaired. Otherwise, the body will not be supplied with enough oxygen and may cause further illness. If there is a feeling of tightness in the throat or at the back of the nose, it is advisable to have a check-up. If there are limitations in hearing or balance problems, a doctor should be consulted. If there is a feeling of pressure in the front of the head, headaches or earaches, a doctor should be consulted. If the affected person suffers from frequent and uncontrolled nosebleeds, discomfort of the jaw or teeth, a visit to the doctor is necessary to clarify the complaints. If the complaints persist for several weeks or months, a visit to the doctor must be made. If the complaints increase, immediate action is necessary. If a deformation of the face is noticed, the juvenile nasopharyngeal fibroma has already enlarged considerably. In these cases, a medical examination should be initiated as soon as possible, since a malignant course of the disease can lead to serious complications. If sensory disturbances, a feeling of numbness or hypersensitivity to touch occur, a visit to the doctor is advisable. If sleep disturbances, snoring, or a refusal to eat occur, a physician should be consulted.
Treatment and therapy
In some patients, spontaneous regression of juvenile nasopharyngeal fibroma occurs after the affected person has completed the pubertal phase. However, because of the discomfort, surgical removal of the tumor is usually used. Juvenile nasopharyngeal fibromas of smaller size are removed by the physician through the nose using endoscopy. Larger juvenile nasopharyngeal fibromas require complicated surgical intervention using transfacial extirpation and removal of the tumor through the oral vestibule. In some cases, surgical intervention is not possible, so radiation therapy is used to combat Juvenile Nasopharyngeal Fibroma. This aims to reduce the size of the Juvenile Nasopharyngeal Fibroma. In general, Juvenile Nasopharyngeal Fibromas often form recurrences even after successful removal. However, the likelihood of this decreases by the end of the 25th year of life.
Outlook and prognosis
The prognosis of juvenile nasopharyngeal fibroma depends largely on the benign or malignant nature of the tumor.Although it is a tumor disease that does not form metastases in the organism, severe disorders and sequelae are to be expected in the event of malignant growth. If left untreated, the tumor leads to premature death. Complaints such as headaches or bleeding of the nose increase and lead to a reduced quality of life. In addition, limitations in memory as well as concentration are to be expected. If medical treatment is sought, the treatment option is determined depending on the size and location of the tumor. Surgical removal of the diseased tissue can lead to complications and risks. These worsen the prognosis. If surgery is performed without further disturbances, recovery occurs in most cases. The existing symptoms disappear immediately after the removal of the tumor. In some patients, radiation therapy is used prior to surgery. In this case, numerous side effects occur, which lead to a further deterioration of the quality of life. However, in the long term, radiotherapy represents the possibility of complete removal of the tumor. The necessity of radiotherapy is made according to individual conditions depending on the size of the tumor as well as its further growth characteristics.
Prevention
Juvenile nasopharyngeal fibroma cannot yet be effectively prevented, because the causes of tumor development are still largely unclear.
Follow-up
In most cases of this disease, no direct measures of aftercare are available to the affected person. First and foremost, a doctor must be consulted quickly and, above all, at an early stage so that no further complications occur and so that the tumor does not continue to spread. The earlier a doctor is consulted, the better the further course of the disease usually is, so that the affected person should consult a doctor at the first symptoms and signs. The treatment itself is carried out by means of a surgical intervention. After this operation, the affected person should take it easy and rest. Efforts or physical and stressful activities should be avoided in order to avoid unnecessary strain on the body. The oral cavity should also be particularly well protected to prevent infections or inflammations. Even after successful removal of the tumor, regular checks and examinations by a doctor are necessary so that further tumors can be detected and removed at an early stage. Possibly the life expectancy of the affected person is reduced by this disease, if the tumor is detected late. However, a general prediction about the further course of the disease can not be made in this case.
What you can do yourself
First of all, it is of high importance for the patients with Juvenile Nasopharyngeal Fibroma that a physician diagnoses the disease quickly. This is because timely detection of juvenile nasopharyngeal fibroma increases the likelihood that the disease can be successfully treated, thereby also increasing the survival rate of affected individuals. In order to play an active role in the success of the therapy, patients attend all necessary examination appointments and always follow the doctors’ instructions, especially before and after surgical procedures. In numerous cases, physicians remove the juvenile nasopharyngeal fibroma during a surgical procedure, which varies depending on the size of the fibroma. Often, this surgery is associated with an inpatient stay in the hospital for the patients. In this case, the affected patients adhere to the regulations of the nursing staff, such as bed rest and the punctual intake of medication. In severe cases, surgery is not possible, so radiation therapy is necessary. At the same time, patients work harder to strengthen their physical defenses and avoid infections. In general, for juvenile nasopharyngeal fibroma, it is important for patients to attend regular medical follow-up visits even after successful surgical removal of the fibroma to quickly detect any recurrences. This allows subsequent fibromas to be removed at an early stage.
