Angiofibroma on the upper eyelid causes. Juvenile angiofibromas of the base of the skull

Angiofibroma is a rather rare disease characterized by the formation of a benign neoplasm that includes blood vessels and connective tissue. Most often, the pathology affects the skin and nasopharynx, less often the base of the skull suffers. The exact causes of the formation of the disease remain unknown today, however, clinicians have developed several theories regarding the possible mechanism of occurrence.

The symptomatic picture will differ depending on the area in which such a neoplasm is localized. The main signs are considered to be facial deformity, the appearance of brown or yellowish nodules on the skin.

The correct diagnosis is made on the basis of data obtained during a thorough physical examination of the patient and a number of instrumental procedures. Laboratory studies in this case are of an auxiliary nature.

Treatment of pathology is carried out only surgically and consists in excision of the neoplasm. In the vast majority of situations, the operation is performed using endoscopy. The prognosis of the disease is almost always positive.

Etiology

Currently, it is generally accepted that angiofibroma is a consequence of abnormal development of the fetus in the embryonic period, however, experts from the field of oncology have made several assumptions regarding the pathogenesis and reasons for the formation of such a neoplasm.

Thus, distinguish:

• genetic theory - is considered the most common. This is due to the fact that all patients who are subsequently diagnosed with such a diagnosis have chromosomal abnormalities;
• hormonal theory - the frequent diagnosis of such a pathology in children in adolescence suggested that the most likely cause is;
• age theory - some doctors argue that the risk of such an ailment increases with age and directly depends on natural process aging of the human body.

In addition, there are also assumptions about the impact of such predisposing factors:

• a wide variety of injuries to the face, nose and head;
• chronic course inflammatory diseases of the larynx, for example,;
• addiction to bad habits;
• living in a bad environment.

The main risk group is males aged 9 to 18 years. It is for this reason that the disease is also called juvenile or juvenile angiofibroma. It is extremely rare that such a tumor is found in people older than 28 years.

Classification

Based on the location of the focus of the pathological process, there are:

• angiofibroma of the skin - mainly found among patients of mature age;
• angiofibroma of the face;
• juvenile angiofibroma of the base of the skull - is a consequence of damage to the larynx;
• education in the kidneys - diagnosed in isolated cases;
• juvenile angiofibroma of the nasopharynx - is considered the most common type of pathology;
• soft tissue formation.

Depending on the clinical and anatomical features, it is customary to distinguish:

• basal-common;
• intracranially widespread.

When the nasopharynx is affected by such a benign tumor, the disease goes through several stages of progression:

• Stage 1 - the neoplasm does not extend beyond the nasal cavity;
• Stage 2 - there is an proliferation of pathological tissues in the pterygopalatine fossa, as well as in some sinuses, in particular, the maxillary, ethmoid and sphenoid;
• Stage 3 - proceeds in two versions. The first is the spread of the pathological process to the orbit and infratemporal region. The second is the involvement of the hard shell of the brain in the disease;
• Stage 4 - also has two forms of flow. The first is damage to the dura mater, but without the involvement of such parts as the cavernous sinus, pituitary gland and optic chiasm. The second - the tumor spreads to all of the above areas.

Symptoms

As mentioned above, the clinical picture will depend entirely on the localization of the focus of such a benign neoplasm in the larynx. It follows from this that in order to defeat skin the following symptoms are typical:

• formation of a single convex node;
• the formation may have a brown, yellow or pale pink hue;
• dense consistency of the tumor;
• bright manifestation of capillaries;
• slight skin itching.

Most often, the place of localization is the upper and lower limbs as well as the neck and face. This form of the disease is most typical for females aged 30 to 40 years.

Juvenile angiofibroma of the nasopharynx has the following clinical manifestations:

• bias eyeball;
• decreased visual acuity;
• chronic nasal congestion;
• facial asymmetry;
• frequent bouts of severe headache;
• weakened sense of smell;
• nasal voice;
• hemorrhages from the nasal cavity;
• swelling of the face;
• hearing loss;
• difficulty in nasal breathing.

Angiofibroma of the face can be located on any of its parts. The main symptom is the sudden appearance of a small dense or elastic growth. With its constant injury, bleeding and a rapid increase in the size of the node are observed. Often, neoplasms are found in the nose, in the auricle or on the eyelids.

A neoplasm in the base of the skull is the most severe form of the disease (since the tumor is prone to rapid growth), which affects boys and men aged 7 to 25 years. By clinical manifestations similar to the defeat of the nose or larynx.

In the case of soft tissues, the pathology is often localized to:

• tendons;
• mammary glands;
• torso;
• neck and face;
• ovaries;
• uterus
• lungs.

This form of the disease does not have specific symptoms.

• the occurrence of the formation of an uneven structure located on the leg;
• reddish or bluish tint of the tumor;
• hoarseness of voice;
• complete inability to make sounds.

In cases of kidney damage, symptoms may be completely absent.

Diagnostics

The diagnosis in a child or an adult is confirmed only after the patient undergoes a series of instrumental procedures.

However, the first stage of diagnosis includes:

• study of the medical history - to search for a provoking factor;
• collection and analysis of life history;
• careful physical examination and palpation of the affected area of ​​the skin;
• evaluation of hearing and vision acuity;
• a detailed survey of the patient or his parents - to compile a complete symptomatic picture.

Instrumental procedures necessary to confirm the diagnosis are presented:

• anterior and posterior rhinoscopy;
• radiography;
• ultrasonography;
• CT and MRI;
• fibroscopy;
• biopsy;
• angiography.

Laboratory studies in this case include:

• general clinical blood test;
• hormonal tests;
• blood biochemistry.

In addition to the otolaryngologist, the following also takes part in the diagnosis process:

• oncologist;
• pediatrician;
• dermatologist;
• ophthalmologist;
• therapist.

Treatment

You can get rid of any type of disease only with the help of surgical intervention, which involves excision of the neoplasm. This can be done:

• minimally invasive methods - are used only when an evil occurs on the skin. To do this, use laser radiation, cryodestruction, vaporization, coagulation;
• endoscopically - through several small incisions;
• cavity method - through one large incision.

After removal of the tumor, treatment must necessarily include:

Prevention and prognosis

specific preventive measures, preventing the development of such a disease does not exist. This is due to the fact that today the reasons for the formation of angiofibroma are not fully known.

However, you can reduce the likelihood of developing an ailment with the help of:

• maintaining an active and healthy lifestyle life;
• proper and balanced nutrition;
• avoiding nervous and physical overwork, as well as any injuries;
• spending enough time outdoors;
• regular passage of a comprehensive preventive examination in the clinic with a visit to all specialists.

The prognosis in the vast majority of cases is favorable - the danger of such a disease lies only in extensive bleeding, which can lead to anemia, as well as a tendency to frequent relapses. No lethal outcome was recorded.

Juvenile (juvenile) angiofibroma of the nasopharynx- a benign tumor that grows in the internal cavity of the nose; occurs almost exclusively in boys during puberty. It is based on connective tissue (fibroma) and vascular tissue of varying maturity (angioma).

Juvenile angiofibroma is one of the most common tumors of the nasopharynx in adolescents: 1 in 50-60 thousand ENT patients or 0.5% of all head and neck tumors.

Angiofibroma, formally being a benign neoplasm, has the features of malignant tumors due to localization, a tendency to recurrence and germination in structures adjacent to the nasopharynx and nasal cavity (pterygopalatine fossa, anterior and middle cranial fossae, orbit).

Intracranial (intracranial) growth is observed in 10-20% of cases. The most dangerous and prognostically unfavorable is the spread of the tumor around the chiasm. optic nerves and the pituitary gland, which makes it inoperable.

Types of angiofibromas depending on the source of tumor growth:

1. Sphenoethmoidal (begins to grow from the sphenoid bone, ethmoid bone, pharyngeal-basic fascia).

2. Basal (arch of the nasopharynx) - occurs most often.

3. Pterygomaxillary (pterygoid process of the sphenoid bone).

Classification of juvenile angiofibroma (Fisch, 1983; Andrews, 1989)

This classification is used by almost all modern authors who publish articles on this issue.

Stage I - the tumor is limited to the nasal cavity;

Stage II - spread into the pterygopalatine fossa or maxillary, ethmoid, or sphenoid sinuses;

IIIa stage - propagation into the orbit or infratemporal fossa without intracranial (intracranial) growth;

IIIb - stage IIIa with extradural (without involvement of the dura mater) extension;

Stage IVa - intradural spread without involvement of the cavernous sinus, pituitary gland, or optic chiasm;

Stage IVb - involvement of the cavernous sinus, pituitary gland, or optic chiasm.

Symptoms and course of juvenile angiofibroma of the nasopharynx

The earliest and most common symptoms are:

Recurrent nosebleeds;

Unilateral nasal congestion;

Increasing difficulty in nasal breathing;

Hyposmia and anosmia (decrease and disappearance of smell);

nasality;

Hearing loss in one (more often) or both ears;

Increasing headaches;

Deformation of the facial skeleton (in the later stages leads to displacement of surrounding tissues).

Depending on the direction of growth of angiofibroma, there may be:

Facial asymmetry;

Deformation of the soft and bone tissues surrounding the tumor;

Violation of the blood supply to the brain;

Compression of nerve endings;

Visual impairment (reduced visual acuity, exophthalmos (displacement of the eyeball forward (bulging eyes), sometimes with a shift to the side), limitation of eyeball mobility, double vision (diplopia), etc.).

With aggressive tumor growth, it can grow into the cranial cavity, which can be fatal.

Diagnosis of juvenile angiofibroma of the nasopharynx:

1. Collection of complaints and anamnesis.

2. Rhinoscopy.

3. Endoscopy of the nasal cavity and nasopharynx.

4. Radiography

5. CT, MRI.

6. Bilateral carotid angiography.

7. Biopsy. A cytological biopsy test is necessary to clarify the diagnosis and type of tumor.

8. Examination by an ophthalmologist, neuropathologist, neurosurgeon and oncologist.

They try to conduct an analysis for tissue biopsy only in a hospital (in the operating unit), because after taking a biopsy, bleeding from the vessels of the nose may occur.

Differential diagnosis of juvenile angiofibroma of the nasopharynx with diseases such as:

Adenoids in children;

Brain tumor;

Choanal polyps;

Sarcoma;

Papilloma.

Treatment of angiofibroma of the nasopharynx

Treatment is only surgical! To reduce trauma, angiofibroma is removed using endoscopic methods. The disease can progress over several years, but in some cases it manifests itself as a malignant neoplasm, that is, it grows rapidly. Therefore, if an angiofibroma of the nasopharynx is suspected and the diagnosis is established, a surgical operation is performed as early as possible.

Operation types:

Surgery using access to the tumor through natural routes (through the nose or mouth);

Operation using sparing access through the maxillary sinus and nasal cavity (with an incision under the lip) - Denker operation;

Operation using extended access through the maxillary sinus and nasal cavity (with a facial incision) - Moore operation, Weber-Fergusson operation;

Operation using access through the sky - operation according to Owens.

During the operation, there is usually heavy bleeding, which requires a massive blood transfusion. To reduce blood loss, before removing the tumor, ligation of the external carotid artery.

AT postoperative period ENT doctor prescribes:

Antibiotics (protection against possible infection);

Transfusion of solutions that replenish blood loss;

Medicines that promote blood clotting.

Juvenile angiofibroma of the nasopharynx does not imply preventive measures - it is impossible to prevent the formation of a tumor. However, in order not to start the course of the onset of the disease, you should immediately contact an ENT doctor at the first characteristic manifestations.

Prognosis for juvenile angiofibroma of the nasopharynx

The prognosis of the disease is usually favorable. Timely surgical operation in combination with radiation therapy leads to a complete cure for a cancer patient.

In rare cases, a negative result of antitumor treatment is observed in the form of relapse or malignancy (malignancy) of the neoplasm. Therefore, operated patients are advised to undergo annual examinations by an otolaryngologist.

Take care of yourself and, if necessary, contact an ENT surgeon on time.

In children adolescence hormonal changes are sometimes accompanied by the appearance of neoplasms, benign and malignant. They can be formed from different tissues.

The great Hippocrates back in the 5th century BC. described the disease, which in modern medicine is called angiofibroma. This is a benign tumor that appears in the nasopharynx. Its basis is tissue - fibroma (connective) and (vascular). It affects the nasopharynx of boys from 10 years old and young men up to 21 years old (after 20 years, as a rule, regression begins), therefore it is also called "juvenile". Very rarely, the disease overtakes men 28-30 years old.

This neoplasm can grow with deep tissue damage extending to the vessels. And this is a big problem if you need to remove it.

Forms of juvenile angiofibroma

Focusing on the direction of growth of juvenile angiofibroma of the nasopharynx, experts call its forms:

• vault of the nasopharynx (beginning), develops into the base of the skull;
• the body of the sphenoid bone (genesis), growing, strikes nasal cavity, eye orbit, sinuses, ethmoidal labyrinth;
• from the beginning - the pterygopalatine fossa - into the nasal cavity.

The growth trend of the neoplasm affects the distortion of tissues adjacent to the nasopharynx. A tumor that grows towards the orbit is fraught with displacement of the eyeball, causes a disruption in the blood supply to the brain, and puts pressure on the nerve endings.

With clinical and anatomical classification, two forms are called this disease: basal- and intracranial-common.

The development of neoplasms - angiofibroma of the nasopharynx - is benign character, however, due to the characteristics of growth, predisposition to repetitions, the neoplasm is considered to be malignant.

Symptoms

Since with angiofibromas nearby tissues are very quickly affected, the disease reveals itself as vivid signs:

• a sharp deterioration in hearing (one or two ears);
• (more frequent and become stronger with the growth of the tumor);
• nasality;
• difficulty breathing through the nose;
• debilitating headaches;
• decrease (often disappearance) of smell.

Depending on the direction in which angiofibroma of the nasopharynx spreads, the clinical picture is supplemented by:

• distortion of the facial skeleton, soft and hard tissues;
• weakening the ability to distinguish the smallest details;
• limitation of mobility and damage to the eyeball (displacement or protrusion);
• diplopia;
• asymmetry of the face, lowered upper eyelids;
• swelling of the cheek (on the side affected by the tumor).

Disease recognition

Diagnosis of pathology - angiofibroma of the nasopharynx - the result of examining the patient, analyzing his complaints and conducting:

• MRI - subject to the skull or nasopharynx (to determine the size and border of the tumor);
• x-ray examination of the arteries;
• endoscopy ();
• a biopsy (of affected tissues for research) is fraught with bleeding, is performed in cases of emergency;
• a thorough examination of the nasal cavity with the help of instruments (rhinoscopy) shows the surface (nature and color of the tissue) of the neoplasm;
• x-ray examination of blood vessels shows whether the carotid artery is affected by a tumor (important to know in case of an inevitable operation).

Mandatory general analysis blood to determine the level of hemoglobin. Sometimes a consultation with an oncologist is prescribed.

When establishing a diagnosis, it is important to distinguish angiofibroma from the clinic with adenoids, brain tumors, polyps, papillomas, and sarcomas.

Treatment

Angiofibroma of the nasopharynx involves decisive action in the treatment - surgery. Before surgical removal of the neoplasm, radiation therapy is usually performed. Since there is a high risk of bleeding during operations, the external carotid artery is often ligated.

Operations to remove angiofibromas are carried out using an endoscope for a detailed examination of remote areas of the nasal cavity.

Surgical intervention under general anesthesia, is chosen depending on the place malignancy through:

• approach to the tumor through the nose or mouth;
• in an easier way - with a dissection under the lip (through the nasal cavity and sinus of the upper jaw);
• deployed passage - facial incision;
• access through the sky.

If during the operation the patient suffers a large loss of blood, a donor transfusion is required. In the postoperative stage, the doctor prescribes:

• antibiotics (protection against possible infection);
• transfusion of solutions that replenish blood loss;
• medicines that promote blood clotting.

Angiofibroma is a benign neoplasm that has its own connective tissue and blood vessels. This disease is quite rare, it can affect the base of the skull, the nasopharynx, nose, cheeks, kidneys. Angiofibroma of the nasopharynx is the most common. It occurs among men of adolescence and forty years of age. If such a neoplasm is formed in boys at the age of puberty, then separate therapy, as a rule, is not required, the neoplasm resolves itself over time.

The exact causes of the disease in modern medicine are still unknown. However, people who expose themselves to the sun too often are at risk. This phenomenon is called photoaging. It is under the influence of the sun that the cells located in the deep layers of the skin undergo mutations, and then begin to divide abnormally quickly, forming a focus benign tumor which has its own blood vessels.

In the case when many tumor foci appear, the reason probably lies in heredity. For example, if parents or close relatives had diseases such as neurofibromatosis or tuberous sclerosis, then the risk of multiple formations, including angiofibromas of the skin and nasopharynx, is much higher.

In addition, there is a theory that the appearance of benign tumors is a consequence of disturbances in the development of the fetus at the stage of gestation. Experts note that all patients have some kind of chromosomal abnormalities. The fact that adolescents are susceptible to diseases during maturation suggests that hormonal changes may be the cause, and the fact that aged men are also at risk may be one of the factors accompanying the natural aging of the body.

In addition to those who are constantly exposed to sunlight, at risk are also people who often get injuries to the nose, head and face, which has bad habits living in poor environmental conditions, as well as having chronic diseases nasopharynx.

This tumor is benign. Its cells remain in the layers of the skin, although rather deep. in vessels and The lymph nodes they do not penetrate, metastases are not formed. The disease is unpleasant, especially from an aesthetic point of view, but, nevertheless, it is not contagious and does not pose a danger to the patient's life.

Classification of species depending on

The disease is divided into subspecies depending on the characteristics of the tumor and the place of its occurrence.

Localizations

The disease can affect the following areas:

• Skin (mainly observed among age-related patients);
• Face;
• Base of the skull (often affected in adolescents, especially those with chronic diseases nasopharynx, then the disease is called juvenile angiofibroma);
• Kidneys (very rare);
• soft tissues;
• Nasopharynx.

Clinical and anatomical features

In accordance with the clinical and anatomical features of a benign tumor, a basally advanced neoplasm and an intracarnially advanced neoplasm are distinguished.

Clinical manifestations depending on localization

Symptoms of the disease may vary depending on the location of the tumor.

In case of damage to the skin

For a benign formation on the skin, the following symptoms are characteristic:

• Slow tumor growth, without damage to neighboring tissues;
• The lesions do not hurt, but can sometimes itch;
• Self-formation is similar to a single dense, but elastic knot of small size (from 1 mm to 3 cm);
• The color of the knot can vary from light beige to pink or brown;
• The upper layer of the skin becomes thinner, and through it the capillaries inside the formation are easily visible.

This type of tumor most often affects the face, neck, upper and lower extremities. According to statistics, this variety is most often affected by women aged 30 to 40 years.

With damage to the nasopharynx

In the case of angiofibroma of the nasopharynx, the following symptoms can be observed:

• Decreased vision, smell and hearing;
• swelling of the face;
• difficulty breathing through the nose, congestion;
• Displacement of the eyeball;
• Facial asymmetry;
• Paroxysmal pain;
• nasality of voice;
• Nosebleeds.

A neoplasm can appear on any part of the face or in the nose. It is advisable not to injure the formed node, as this will only increase the rate of its growth.

With soft tissue injury

When amazed soft tissues there are no specific symptoms. Most often, such angiofibroma affects the uterus, ovaries, lungs, torso, neck, face, mammary glands, tendons.

Stages of the disease

The disease is divided into 4 stages:

• Stage 1. The neoplasm is localized where it appeared, growth is not observed;
• Stage 2. The tumor begins to grow, new foci may appear;
• Stage 3. The spread of the tumor process, the area and number of lesions increase;
• Stage 4. The defeat of almost the entire occupied area, sometimes including the nerves.

Diagnostic methods

Establishing an accurate diagnosis usually takes about six months. During this period, the patient undergoes various studies.

At the first stage of diagnosis, the doctor reviews the medical history, interrogates and examines the patient, feeling suspicious nodes. Then the patient is sent to undergo instrumental diagnostic procedures: CT, MRI, X-ray, fibroscopy, rhinoscopy, biopsy, ultrasonography, angiography. Laboratory tests are also needed: hormone tests, complete blood count and biochemistry.

The following specialists may be involved in the diagnostic process: otolaryngologist, oncologist, dermatologist, ophthalmologist, therapist.

To distinguish angiofibroma from others benign neoplasms, can be carried out and differential diagnosis. The following manifestations can be confused with this disease: lipoma, birthmark, hemangioma, nevi, fibroma, melanoma, skin basalioma.

Methods of treatment

In fact, such a tumor is not dangerous, but it can interfere, for example, with constant injury when rubbing against clothes or purely from an aesthetic point of view. That's why modern medicine offers quite a few ways to treat angiofibroma, the specialist chooses the best one depending on each specific case. If the neoplasm does not create any problems, then therapy can be dispensed with, especially if it is a juvenile angiofibroma, it is enough just to see a doctor.

Medical

In this case drug therapy mainly aimed at eliminating the unpleasant symptoms of the disease. For example, when painful sensations it is recommended to drink no-shpu or baralgin. Stimol, Duovit, Vitrum can be prescribed to strengthen blood vessels and maintain the general immunity of the body.

In some cases, combination chemotherapy may be given before or after surgery. It uses the following groups of drugs: vincristine, adriamycin, sarcolysin or vincristine, adriamycin, cyclophosphamide, decarbazine.

In addition, during treatment it is desirable to make sure that the body receives all essential vitamins with food or by taking special complexes. Vitamins do not slow down the growth of neoplasms, but they help to cope well with the accompanying negative factors. For example, improve immune system, prevent the appearance of anemia, strengthen blood vessels.

Endoscopic

This method of removal is most effective for neoplasms of small and medium sizes. The operation is performed under general anesthesia. The doctor removes the tumor through the sinuses (or another area, depending on the location) under the control of a special device - an endoscope. Then the damaged surface is treated, if necessary, sutures are applied. It is important to understand that this is also surgical intervention, although less dangerous than surgery. And in this case, there is also a risk of bleeding and infection.

Minimally invasive methods

Tumor tissue can be removed with a laser. The beam evaporates them in layers, down to healthy skin. The procedure is performed under local anesthesia and lasts only 10-15 minutes. This method has quite a few advantages and a minimum of consequences for the patient. Laser removal is considered one of the most modern methods.

Surgical

This is one of the most effective ways getting rid of angiofibroma. The operation is simple, so it is performed on an outpatient basis under local anesthesia. The neoplasm is removed, and then sutures are applied to the wound. The removed tissue is then sent for further histological analysis. During the operation, the tumor is completely removed, although this does not guarantee the absence of recurrence. However, it is still worth remembering that this is a surgical intervention and in the process there is a risk of infection or bleeding.

Radiation therapy

The tumor can also be removed with radiation therapy. In this case, a beam of radio waves is directed to the knot. In this case, surgical intervention is not performed, that is, the risk of bleeding and infection is excluded, there will not even be a scar. However, exposure to radio waves on the skin can cause dermatitis or local tissue necrosis.

After any of the chosen methods of removing the tumor, it is worth monitoring the affected area. If possible, eliminate friction with clothing and avoid other methods of mechanical injury. If a bandage is applied, then it must be changed on time, and measures should be taken to prevent inflammation.

Forecast

The prognosis of the disease is positive. Fatalities has not yet been recorded. The greatest danger of angiofibroma is due to the fact that it can provoke extensive bleeding, that is, create prerequisites for the occurrence of anemia.

In addition, even after surgical removal of the neoplasm, new tumors may appear, that is, relapses of the disease - a phenomenon, although very unpleasant, but quite frequent.

It is important to know that self-medication, including homeopathy and folk methods will not lead to a result, and besides, it can even harm the body.

Therefore, any alternative methods, like medicines, should be used only after consultation with a specialist and often in combination with the removal of a neoplasm.

Prevention

Since the exact causes of the onset of the disease are still unknown, no specific preventive measures have been developed. Therefore, only general advice can be given: lead an active and healthy lifestyle, avoid overload, stress and physical injuries, eat right and balanced, spend more time outdoors, and regularly undergo preventive examinations.

As a rule, if the body has enough resources, that is, it has access to proper rest, all the necessary nutrients and trace elements, then it is able to independently prevent the growth of tumor cells. Therefore, maintaining a healthy lifestyle is so important for the prevention of not only angiofibroma, but also most of the known diseases. modern science diseases.