Fibroma of the nasopharynx: causes, symptoms, diagnosis, treatment

Fibroma of the nasopharynx is a fibrous tumor of dense consistency, characterized by significant bleeding, which is why it was called angiofibroma. This tumor has been known since the time of Hippocrates, who proposed the so-called transnazomedial approach by splitting the nose pyramid to remove this tumor.

The place of the tumor outcome is most often the nasopharynx arch, its pharyngeal fascia (the basilar type of tumor, according to A.Glikhachev, 1954). Studies of the last years of the XX century. Showed the possibility of developing juvenile fibrous nasopharynx from the pterygo-mandibular and wedge-shaped lattice regions.

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10]

What causes nasopharyngeal fibrosis?

The etiology of this disease is almost unknown. The existing "theories" of disembriogenesis and endocrine dysfunction have not completely solved the problem. "Endocrine theory" is based on the fact that the tumor develops simultaneously with the development of secondary sexual characteristics and the period of its development terminates with the end of the periods of puberty. It was also noted that in the presence of nasopharyngeal fibrosis, 17-ketosteroid secretions and the ratio of androsterone and testosterone are observed.

Pathological anatomy of the nasopharyngeal fibroma

Fibroma of the nasopharynx is a very dense tumor, sitting on a wide base, closely welded to the periosteum. The strength of the fusion with the periosteum is so great that in other cases, when the tumor is removed by tearing, fragments of the underlying bone are removed along with it. The surface of the tumor is covered with smooth papillate formations of pale pink or reddish light, depending on the degree of vascularization of the tumor. Density of the tumor is determined by its fibrous nature. The tumor has a pronounced extensive growth, dislocating and destroying all tissues and filling all adjacent cavities that are on this path (the choana, the nasal cavity, the orbit, the sphenoid sinus, the lower sections of the nasopharynx, the paranasal sinuses, the malar and temporal fossae, etc.). . When spreading anteriorly, the tumor fills the nasal passages, destroying the opener, the nasal septum, the upper and middle nasal conchae, penetrating the cells of the latticed labyrinth, the frontal and maxillary sinuses, deforming the nose pyramid and disrupting the facial features. When spreading to the back and forth, the tumor destroys the anterior wall of the sphenoid sinus and penetrates into it, sometimes reaching the pituitary gland, spreading downwards, often reaching the soft palate and oropharynx.

Thus, due to extensive growth and destructive effects on surrounding organs and tissues, nasopharyngeal fibroma can be classified as "malignant" clinically, but it does not give metastases and histologically refers to benign tumors. Morphologically it consists of different manifestations of vascular plexuses and connective tissue stroma, consisting of collagen fibers and fibroblasts. The tumor is covered with epithelium, which in the region of the nasopharynx has the character of a single-layered epithelium, and in the nasal cavity - cylindrical ciliate epithelium - a fact explained by the fact that the tumor appears in the zone of transition of the planar epithelium to the cylindrical epithelium. The vascular filling of the tumor, especially the venous plexuses, is very large. Veins by resorption of their walls merge, forming whole "lakes" of blood with very fragile "banks", traumatization of which (for example, when sneezing) or spontaneously cause profuse, hardly stopping nasal and pharyngeal bleeding. With the reverse development of the tumor in the vascular endothelium, there are phenomena of necrobiosis, hyalinization, and in the stroma - phenomena of an inflammatory nature. As a result, non-viable tumor tissues undergo resorption, the tumor is sharply reduced and replaced by scar tissue.

Symptoms of nasopharyngeal fibroma

Symptoms of nasopharyngeal fibrosis develop slowly and are subdivided into subjective and objective. At the initial stages of tumor development there is a gradually increasing difficulty of nasal breathing, sometimes unjustified headaches and "deaf" pain in the base of the nose, high mental and physical fatigue, because of which children begin to lag behind in the mastery of the school program, in physical development, often sick catarrhal diseases. Discharge from the nose are mucopurulent. Appear complaints first to minor, then to all the increasing nasal bleeding, because of which the child develops anemia. Subsequently, the tumor fills the nasal cavity, causing an increasing difficulty in nasal breathing, until its complete absence. The child is always open mouth, his speech becomes nasal (rhinolalia operta), obturation of the auditory tube causes deafness, while loss of smell and a decrease in taste sensitivity. When the tumor contacts the soft palate, patients complain of impaired swallowing function, frequent pinches. Depression of sensitive nerve trunks causes neuralgic eye and facial pains.

In an objective examination, the doctor sees a pale child or boy with a constantly open mouth, with blue divorces under his eyes and a swollen base of the nose. In the nasal passages there are mucopurulent discharge, which the patient can not remove by means of a blow-out because of obturation with the tumor of the khohan (expiratory valve). After the removal of these secretions, the gaze appears as a thickened hyperemic mucosa, enlarged cyanotic red nasal conchas. After lubricating the mucous membrane of the nose with epinephrine and contracting the nasal concha in the nasal passages, the tumor itself becomes visible in the form of a sedentary smooth, grayish-pink or reddish formation, which is highly bleeding when touched by a sharp instrument.

Often, the tumor filling the nasal canopy is combined with one or more common secondary polyps of the nose.

With a posterior rhinoscopy of a typical picture, which clearly shows the choana, the vomer and even the posterior ends of the nasal concha, it is not determined. Instead, a massive bluish-red tumor is detected in the nasopharynx, completely filling it and sharply differing in appearance from the banal adenoid vegetation. When finger examination of the nasopharynx, which should be carried out carefully, so as not to cause bleeding, a dense fixed solitary tumor is determined.

Associated objective symptoms may include lacrimation, exophthalmos, enlargement of the root of the nose. In the bilateral manifestation of these signs, the patient's face acquires a peculiar form, called "frog face" in foreign literature. When oropharyngoscopy, the swelling of the soft palate due to the presence of a tumor in the middle parts of the pharynx can be detected.

The undeveloped tumor grows to enormous sizes, filling the entire space of the nasal cavity, the orbit and, beyond their limits, causes severe functional and cosmetic disorders. The most formidable complication is the perforation of the tumor by the tumor of the latticed plate and its penetration into the anterior cranial fossa. Initial signs of this complication are the syndrome of increased intracranial pressure (drowsiness, nausea, vomiting, incurable headache), then signs of retrobulbar syndrome (loss of visual fields, ophthalmoplegia, amaurosis) are added. Frequent hemorrhages and intolerable headaches, vomiting and rejection of food lead the patient to a severe general condition, cachexia, anemia, not unlike a condition in the presence of a malignant tumor. Often, such neglected conditions, formerly found in low-civilization areas and countries, were complicated by meningitis and meningoencephalitis, which inevitably lead to the death of patients.

Fibromixoma, or choanal polyp

Fibromixoma, or choanal polyp, originates in the choanal or etmoidosphenoidal region. Externally, this benign tumor has the appearance of a polyp on the leg, which can be easily removed without bleeding with a special hook. In comparison with the mucous polyps of the nasal cavity, the consistence of the choanal polyp is more dense. It grows in the direction of the pharynx and the nasal cavity. "Old" polyps thicken, acquire a reddish color and often simulate nasopharyngeal fibrosis, however, unlike it, do not bleed and do not have extensive growth.

Treatment is surgical.

[11], [12], [13], [14]

Sinus-choanal polyp

The sinus-choanal polyp is actually a manifestation of polypous sinusitis, because it originates from the maxillary sinus and prolaps through the hoan into the nasopharynx. Less often this "tumor" comes from the sphenoid sinus. Externally, the sinus-choanal polyp can be compared with the tongue of the bell, which hangs into the nasopharynx and sometimes reaches the oropharynx, settling between its posterior wall and the soft sky. According to its structure, this polyp is a pseudo-cystic cystic formation of a whitish-gray ovoid form that completely fills the nasopharynx and causes nasal breathing difficulties in tubular hearing impairment.

Treatment is surgical.

Nasopharyngeal cysts

Nasopharyngeal cysts occur either as a result of blockage of the duct of the mucous gland (retention cyst), or Thornwald's pharyngeal sac. Nasopharyngeal cysts are rare tumors that are characterized by a long evolution and are manifested by disorders of nasal breathing and hearing due to overlapping of the nasopharyngeal mouth of the auditory tube. At a back rinoscopy the smooth roundish tumor of gray color of an elastic consistence is defined. Remove the cyst with an adenotome.

Dermoid cysts of the nasopharynx

Dermoid cysts of the nasopharynx refer to very rare congenital benign tumors, most often observed in infants. They develop very slowly and produce minimal disruption of functions, mainly a dry reflex cough and some inconveniences when swallowing. As a rule, the foot of this "polyp" originates from the side wall of the nasopharynx in the circumference of the nasopharyngeal mouth of the auditory tube and often reaches the pharyngeal-epiglottis fold. At usual pharyngoscopy it is not revealed, however at a gag reflex can appear in a pharyngeal in the form of the oblong solitary polyp whitish-gray color with a smooth surface. Its surface has a leathery appearance with papillas, sweat and fat glands, and also hairs. Under this layer is a tissue, permeated with venous vessels. In the center of the tumor, a nucleus is formed, formed by a dense connective tissue, sometimes containing fragments of bone or cartilaginous tissue, as well as cross-striated muscle fibers (a typical "set" of tissues for embryonic tumors). Treatment consists in crossing the cyst's leg. Subsequently, dermoid cysts of the nasopharynx are sclerosed and after a while (months - several years) dissolve.

[15], [16], [17]

Papillomas and pharyngeal lipomas

Papillomas and lipomas are benign tumors that rarely occur in the upper parts of the pharynx and are visually very difficult to distinguish from the banal adenoid tissue. The final diagnosis is established by histological examination. They are removed using the adenotome.

Meningocele base of the skull can sometimes occur in the nasopharynx and more often in children. It differs from other benign tumors in that it increases during crying. Such "tumors" can not be treated, because they are accompanied by other severe abnormalities of the development of the skull and other organs, usually incompatible with life.

Benign plasmacytoma

A benign plasmacytoma originates from the reticuloendothelial tissue and arises most often in the bone marrow; more than 80% of extramedullary tumors of this species are located in the upper respiratory tract. In appearance, they resemble polypoid formations of grayish or pinkish-violet color, they do not ulcerate. The diagnosis is based on a histological examination. A solitary plasmoditoma can be either benign or malignant. A benign plasmacytoma is rare, it should not be confused with a simple granuloma consisting of a large number of plasmocytes. Malignant plasmacytoma is excluded because radiography does not reveal bone lesions, with sternal puncture, pathological changes in the bone marrow are not detected, there are no myeloma cells, the Bens-Jones protein is not detected in the urine, and finally, there is no relapse when the tumor is removed. Also, protein blood fractions, which in benign tumors persist at a normal level, are also investigated. Most of the plasmacytomas are malignant tumors of either extramedullary localization or having the character of a "diffuse" myeloma, which is the essence of myeloma.

Benign plasmacytomas, if they cause certain functional disorders, depending on their size, are removed by various surgical methods, after which they do not give relapses. Malignant plasmacytomas can not be surgically treated. They use non-operative antitumor methods.

[18], [19], [20], [21], [22], [23]

Diagnosis of nasopharyngeal fibroma

Diagnosis of nasopharyngeal fibrosis is established mainly in the clinical picture, the occurrence of nasopharyngeal fibrosis in males in childhood and adolescence. The prevalence of the tumor is determined by X-ray or MRI, or CT-examination, as well as using angiography.

Differential diagnosis of nasopharyngeal fibroma is of significant importance, since the outcome depends on the tactics of treatment and, possibly, the prognosis. Fibroma nasopharynx differentiate from adenoids, cysts, fibromixomas, cancer and sarcoma of the nasopharynx. The main distinguishing feature of fibroids is their early and frequent bleeding, which is not observed at all other benign tumors of this localization, and in malignant tumors bleeding is observed only in the developed clinical and morphological stage of them.

[24], [25], [26]

Treatment of nasopharyngeal fibroma

Numerous attempts to apply non-operative methods of treatment (physiotherapeutic, sclerosing, hormonal) did not provide a radical cure; The only way in which the patient often fully recovered, especially in combination with non-operative methods, was surgical. However, the classical method of extracting the tumor, used for its removal from the place of attachment by means of special forceps with oral access, was not justified because of the impossibility of removing the fibrous base of the tumor, firmly welded to the periosteum (hence the imminent recurrence) and the strongest, difficult to stop intraoperative bleeding. To facilitate access to the tumor, the French rhinohurist Nelaton proposed to split the soft and hard palate. Other accesses to the tumor have also been developed, for example, the use of the rhinotomy method with the help of the para-latero-nasal Moore cut or the sublabial rhinotomy of Denker.

Operation Denker is a preliminary surgical intervention for the formation of wide access to internal pathological formations of the nasal cavity, and in particular to the maxillary sinus, the middle and posterior sections of the nasal cavity and the base of the skull (nasopharynx, sphenoid sinus). In neglected cases, when the tumor grows into the zygomatic area, in the paranasal sinuses, the orbit or the retro-oxillary region is removed after the creation of preliminary accesses. According to AG Likhachev (1939), the most beneficial access to the tumor is transmaxillary according to Denker. But in some cases, depending on the histological features and prevalence of the tumor, the use of radiotherapy leads to a delay in tumor growth by its reduction and compaction, which helps to reduce intraoperative bleeding and facilitates the surgical removal of it. The same effect is obtained by infiltration of the tumor with 96% ethyl alcohol. In some cases, surgery is performed several times until the end of puberty, when the relapses cease, the tumor ceases to develop and acquires a reverse development.

Operative intervention provides thorough preoperative preparation (improvement of the general condition of the body, normalization of blood counts, vitaminization, administration of calcium preparations to strengthen the vascular wall, measures to increase haemostatic parameters). Preparation for the operation should provide for the possibility of carrying out during it resuscitation, blood transfusion and blood substitutes. Operative intervention is performed under intratracheal anesthesia. In the postoperative period, the same measures are carried out as in the period of preoperative preparation of the patient.

Non-operative methods of treatment as independent agents do not always give a positive result; they are expedient for carrying out in the order of preoperative preparation or as an attempt of non-operative cure.

With small tumors, diathermocoagulation can be used in the endonasal approach in persons aged 18-20 years with the expectation that they will soon end their puberty and relapse will no longer occur. At the same age, diathermy can be used for small postoperative relapses.

Radiation treatment is shown mainly as protective, because fibromatous tissue is not X-ray sensitive, but it stabilizes the growth of young cells, new vascular plexuses that are obliterated, and thus restrict the access of nutrients to the tumor and slow its growth. Usually the course of treatment takes place within 3-4 weeks at a total dose of 1500 to 3000 r.

Treatment with radioactive elements introduced into the tumor is not practically applied at present due to possible secondary complications (atrophy of the nasal mucosa and nasopharynx, necrotizing etmoiditis, sky perforation, etc.). Now radium and cobalt are replaced by radon, which is placed in capsules of gold. The latter are implanted into the tumor at a distance of 1 cm from each other in an amount of 5-6. The procedure can be repeated in a month; this method does not cause such serious complications, as with the application of radium or cobalt.

The main hormonal drug in the treatment of nasopharyngeal fibrosis is testosterone, which has androgenic, antitumor and anabolic effects. It regulates the development of male sexual organs and secondary sexual characteristics, accelerates the sexual maturation of males, and also performs a number of other important biological functions in the developing organism. Apply it in various dosage forms (capsules, tablets, intramuscularly and subcutaneously implants, oily solutions of individual esters or combinations thereof). At a fibroma nasopharynx use from 25 to 50 mg / week of a preparation within 5-6 ned. Treatment is carried out under the control of the 17-ketosteroids in the urine - normal recovery of 17-ketosteroids in men is on average (12.83 ± 0.8) mg / day (from 6.6 to 23.4 mg / day), in women - (10,61 ± 0,66) mg / day (6,4-18,02 mg / day) and can be repeated at normal content in the urine of this excreta. An overdose of testosterone can lead to testicular atrophy, early appearance of secondary sexual characteristics in children, osteogenesis and psyche disorders, and an increase in the content of 17-ketosteroids in the urine.

What is the prognosis of a nasopharyngeal fibroma?

The prognosis depends on the prevalence of the tumor, timeliness and quality of treatment. With small tumors, recognized at the very beginning of their occurrence, and the corresponding radical treatment, the prognosis is usually favorable. In neglected cases when radical treatment is not possible, and frequent palliative surgical and other interventions tend to result in relapses and, possibly, malignancy of the tumor - the outlook is pessimistic. According to the most extensive foreign statistics, the lethality in the second half of the XX century. At surgical interventions for a fiber of a nasopharynx has made 2%.