Juvenile Nasopharyngeal Fibroma

Abstract

The large number of cases of nasopharyngeal fibroma seen in Mexico gives Mexican otolaryngologists considerable experience with this disease. This report includes studies on 322 patients seen by the author. It is important to recognize that nasopharyngeal fibroma is not just a tumor, but a disease with particular characteristics, such as prevalence among young males. Clinically it is quite different from other tumors of the pharynx, nose or accessory sinuses.In nasopharyngeal fibroma, the history includes occasional or frequent epistaxis that sometimes re quires blood transfusion.'"" The tumor is located in the nasopharynx; it is of a hard consistency and can invade the sphenoid sinus in maybe 90% of the cases, and one or both choanae, with consequent nasal obstruction. It also may invade other nasal cavities, such as a fossa, the ethmoidal or maxillary sinus, the orbit, the pterygomaxillary space or even inside the cranium.The tumor appears as a compact mass, generally smooth, grey or reddish, that bleeds at slightest con tact. If a biopsy is performed, control of the subse quent hemorrhage becomes difficult. Packing for several hours or days might be indispensable to avoid deterioration of the general condition of the young patient. ETIOLOGY It is well-known that race, age and sex are the most important factors in the development of naso pharyngeal fibroma. In this series, 80% of the pa tients were Mexican natives, 18% were of mixed race, and only 2% white. Geographic variables, such as altitude and climate, are not significant fac tors, since these patients came from all parts of Mexico. All were male. Their karyotypes demon strated no pathologic features. Follow-up on some patients who were treated more than 20 years before showed that they are married and have normal children, and that the development of primary and secondary sexual char acteristics and the endocrine glands was normal. Flora of the nose and pharynx are usually normal. Heredity is not a factor in this disease; none of the relatives of the patients have had similar tumors. There was no history of syphilis, tuberculosis or other specific infections. Blood tests were normal or showed some anemia or a slight increase in white cells. The age of the patients ranged from 10 to 25 years. The only exception was one patient 35 years old who had suffered the disease since adolescence. PATHOLOGY The tumor is constituted by fibrous tissue with stellar cells supported by immature fibroblasts.Multiple vessels can be seen in a stroma of myx omatous tissue. The most important characteristic of such vessels is that they are devoid of elastic or muscular fibers and are filled with blood. This ex plains the presence of severe hemorrhage when the tumor is touched. It is nourished by the ophthalmic artery and secondarily by the internal maxillary artery. It is very important to remember this when planning treatment. Though nasopharyngeal fibro ma is considered a benign tumor, the consequence of its growth can be fatal. DIAGNOSISClinically, diagnosis is based on th...

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