Malignant tumors of the middle ear

Malignant tumors of the middle ear are a rare disease in otolaryngology. Interesting statistics are given by the Romanian author Cornelia Paunescu. So, according to her information, in the Bucharest (Romania) Hospital named. Koltsiya ratio of malignant tumors and other diseases of the middle ear by 1960 was 1: 499. According to the combined statistics of Adams and Morrisson (1955), collected in the hospitals of Birmingham for the entire period of their existence, only 18 patients with malignant tumors of the ear had a 29.067 different ear diseases, which was 0.06%. The sarcomas of the middle ear are even more rare.

Malignant tumors of the middle ear appear in equal proportions in both sexes, epithelioma occur between 40 and 50 years, and sarcomas - up to 10 years of age.

Malignant tumors of the middle ear are divided into primary and secondary.

Primary tumor is the most frequent and typical disease for the middle ear from all the malignant tumors that arise here. Primary cancer can develop from the epithelium of the mucosa of the tympanic cavity, and the sarcoma from the fibrous tissue of the periosteum, most often preceded by either benign connective tissue tumors or long-term purulent processes in the middle ear.

Secondary tumor of the middle ear arises from the penetration of tumors from neighboring anatomical formations (base of the skull, nasopharynx, parotid region) or metastasis from distant tumors.

[1], [2], [3], [4], [5], [6]

What causes malignant tumors of the middle ear?

The most common cause of middle ear cancer is a long-term chronic epitimpanitis complicated by caries of bone and cholesteatoma. Primary malignant tumors of the middle ear are caused by the repeated use of silver nitrate solution to extinguish granulations in the tympanic cavity, and secondary ones are eczema, exostoses, benign tumors of the external auditory canal leading to the formation of malignant tumors of the external auditory canal and their spread in the tympanum.

Among the reasons for the occurrence of sarcoma, a number of authors describe injuries and the presence of remains of gelatinous mesenchymal tissue in the above-drum space, the resorption of which is delayed, which leads to its malignant degeneration. The mechanism of development of malignant tumors of the middle ear is explained by metaplasia of the mucosa of the above-drum space during a long-term inflammatory process in this area or as a result of migration of the cutaneous epithelium of the external auditory canal in the middle ear through the perforation of the tympanic membrane.

Pathological anatomy of malignant tumors of the middle ear

The most common form of middle ear cancer is the spinocellular epithelioma. Then follows the basal cell, the cylinder-cell and glandular epithelioma. Sarkomas of the middle ear are undifferentiated, fibrosarcomas, rhabdomyosarcomas, angiosarcomas, osteosarcomas, lymphoreticulosarcoma.

Symptoms of malignant tumors of the middle ear

Typically, the first symptoms of middle ear malignant tumors drown in the signs of chronic purulent inflammation, I suspect the presence of a tumor occurs only when an unusually extensive destruction of bone tissue that extends beyond the drum cavity, overdrug space and cave is revealed during radiographic examination.

The main symptom is deafness, which, however, does not have a specific significance for diagnosis. The only sign that can alert a doctor is an unusually fast progressive hearing loss, down to total deafness in one ear. Constant symptoms of malignant tumors of the middle ear are noise in the ears of a non-tonal nature, and when the tumor spreads in the direction of the medial wall of the tympanum and windows, there are signs of vestibular disorders (mechanical pressure on the staple base, the penetration of cancer toxins through the windows) and attachment of perceptive hearing loss.

Many authors consider pathognomonic symptom for malignant tumors of the middle ear paralysis of the facial nerve. Indeed, in far-reaching cases, when there is a destruction of the facial canal in the medial wall of the tympanum, passing between the window of the vestibule from below and the arch of the lateral semicircular canal, paresis or paralysis of the nerve occurs, but this complication often accompanies the chronic purulent process in the middle ear carious process and cholesteatoma, so that this sign "too" pathognomonic should not be considered. The suppuration, even of a sacred nature, can also be attributed to a banal chronic purulent inflammation. Pain syndrome, perhaps, can play a decisive role in the diagnosis of malignant tumors of the middle ear: it is distinguished by a special persistence, not characteristic of common chronic otitis media; the pain is of a permanent deep nature, intensified at night, sometimes reaching painful paroxysms. Even with abundant discharge from the ear, indicating that there is no delay, this pain does not subside, but, on the contrary, is steadily increasing.

When otoscopy through a completely destroyed eardrum in the external auditory canal prolapses fleshy formation of red, usually "bathing" in purulent-bloody masses, often mistaken for granulation. The sensation of this formation by the bell-shaped Voyachek probe reveals its friability, bleeding, and the probe practically freely penetrates into the deep sections of the tympanic cavity whose contents look like a homogeneous bleeding mass. It is better to avoid probing the medial wall with a probe, since it is easy to penetrate the destructively altered bone into the vestibule or the main curl of the cochlea and thereby cause a severe labyrinthitis with unpredictable consequences.

When the process is extended to the retro-auricular space, it is possible to define visually and palpationally dense tumor-like formation, smoothing the BTE and protruding the auricle. Secondary infection of an extraterritorial tumor leads to a periauricular inflammatory infiltrate, pain intensification, and endoural infection when the medial wall is maligned to a rapidly developing labyrinthitis and multiple intracranial complications.

In other cases, malignant tumors of the middle ear occur without particularly vivid manifestations under the guise of a chronic banal otitis media, and only in case of surgery the experienced otosurgeon may suspect the presence of a tumor in the reddish and dense homogeneous tissue, therefore, in operative interventions in the middle ear for chronic purulent inflammation to direct for histological examination all removed pathologically changed tissues.

In advanced cases, malignant tumors of the middle ear can be complicated by the defeat of the parotid salivary gland, the latticed bone, nasopharyngeal structures, the ear maze, the pyramid of the temporal bone, the nerve trunks of the MMU (rupture hole syndromes, Gradenigo, Colle-Sicara, etc.).

The general condition of the patient progressively worsens (anemia, cachexia). In untreated or incurable cases, patients die in cachexia or as a result of such complications as bronchopneumonia, meningitis, profuse arthrosis bleeding from the internal carotid artery, sigmoid sinus or internal jugular vein.

Diagnosis of malignant tumors of the middle ear

Diagnosis of malignant tumors of the middle ear includes the collection of anamnesis, assessment of subjective and objective clinical data, and the conduct of histological and radiologic (CT and MRI) studies. X-ray examination is carried out in the projections of Schueller, Stenvers, Highway II and Highway III. At the initial stages, a more or less intensive shading of the middle ear cavities appears without signs of destruction of bone tissue (if this destruction did not occur earlier, as a result of the previous chronic purulent epitimpanitis). Further development of the process leads to the destruction of bones, manifested in the resorption of bone tissue, uneven osteolysis with the formation of its defects with uneven and fuzzy edges.

In some cases, the destructive process can spread to the entire stony-mastoid massif, while the boundaries limiting the lost bone tissue are characterized by fuzziness, blurring, which distinguishes the x-ray picture of malignant tumors of the middle ear from that of a cholesteatoma or glomus tumor, in which the cavities formed A smooth surface and clear boundaries. With epitheliomas, bone destruction occurs much earlier and progresses much faster than in benign middle ear tumors. The middle ear sarcomas have no significant differences either in the clinical course or in the results of the x-ray examination. They are identified by histological examination.

Differential diagnosis of malignant tumors of the middle ear is carried out with chronic purulent otitis media, a glomus tumor and other benign tumors of the external and middle ear, which were mentioned above. In many cases, the final diagnosis can be made only on the operating table when taking a biopsy specimen for histological examination, which is of fundamental importance both for the prognosis and for the development of the subsequent treatment tactics. It should be noted that the intracranial spread of the glomus tumor with defeat of VII, IX and XII cranial nerves significantly complicates the differential diagnosis.

As Cornelia Paunescu (1964) notes, the ear manifestation of certain systemic diseases can simulate a malignant tumor of the middle ear, especially sarcomas such as leukemias, leukosarcomatoses, myeloma, eosinophilic granulomas, etc. Among these diseases, we particularly note the Hend-Schuiller-Crischen syndrome described above (and the Letterser disease - Siwa).

At first, the ear infection is often observed in children between 2 and 5 years old, characterized by primary localization in the mastoid process and can be taken for sarcoma. However, multiple enlightenments in the bones of the skull and other bones, along with other symptoms (eyelids, pituitary symptoms, enlargement of the liver, etc.) serve as differential diagnostic signs excluding malignant neoplasm.

In the case of Letterter-Sieve disease, an exceptionally severe reticuloendotheliosis in children, a primary manifestation in the SFA region may simulate sarcoma, but typical changes in the cytological picture of the blood, an increase in body temperature, a histological pattern of the infiltrate (monocytosis, giant ugly cells) determines the final diagnosis.

[7], [8], [9], [10], [11],

Treatment of malignant tumors of the middle ear

Treatment of malignant tumors of the middle ear includes surgical, chemotherapeutic and radiation methods, the sequence of which is determined mainly by the prevalence of the tumor process. The symptomatic treatment of malignant tumors of the middle ear includes the use of analgesic drugs, normalization of CBS and composition of red blood, prevention of super infection.

To date, the main method of treatment is surgical and in those cases when the operative method can not only eliminate the disease, but also extend the life of the patient, preventing at least temporarily spread of the tumor process and the occurrence of relapse.

Indications for surgical intervention and its nature, according to Cornelia Paunescu (1964), are defined as follows.

With limited tumors that show conduction and hearing loss, an expanded stony-mastillary extirpation occurs with the exposure of the dura mater and sigmoid, in some and transverse sinus. Simultaneously, remove the cervical and submandibular lymph nodes on the affected side.

If the malignant tumor of the middle ear is affected by a secondary infection and infiltrates the tragus region, the operation is supplemented by removal of the parotid salivary gland.

When the tumor is injured in the above-drum space, remove all the anatomical entity called the ear region with the monoblock, which includes the middle ear, the mastoid region, the external auditory meatus and the auricle; all cervical and submandibular lymph nodes on the side of the lesion are also removed. Then the patient is subjected to radiation therapy.

If there is paralysis of the facial nerve at the level of the above-drum space, signs of defeat of the ear maze (deafness, deactivation of the vestibular apparatus), then the previous surgical interventions supplements removal of the entire array of the labyrinth node and the pyramid of the temporal bone using the Ramadier method.

In this operation, the segregation of the labyrinth node and the pyramid is facilitated by the total removal of the middle ear structures, in which the medial wall of the tympanic cavity is completely removed, as well as the preliminary resection of the lower and anterior bone walls of the external auditory canal. As a result, access to the bone channel of the auditory tube is opened, the wall of which is also resected. This provides access to the canal of the internal carotid artery, which is trepanized with the exposure of the latter. The sleepy artery lies here between the bone wall of the auditory tube and the snail. The sleepy artery is pulled anteriorly, after which an array of the pyramid of the temporal bone opens. The path to the top of the pyramid is laid from the side of the exposed medial wall of the bony canal of the internal carotid artery. After careful excision of the internal carotid artery, previously taken for provigory ligature, the pyramid of the temporal bone is removed together with the remains of the labyrinth node and the pre-vertebral nerve located in it. Then, the sigmoid sinus is exposed, down to the bulb, and the hard brain clusters and adjacent formations are examined. The resulting postoperative defect is covered with a skin flap on the leg taken from the surface of the head. Prescribe antibiotics and according to the indications appropriate symptomatic and rehabilitative treatment. After 3 weeks, radiotherapy begins.

What prognosis are the malignant tumors of the middle ear?

Malignant tumors of the middle ear usually have a serious or pessimistic prognosis (due to the often late recognition of the disease), especially with sarcomas occurring in childhood.