Malignant granuloma of the nose: causes, symptoms, diagnosis, treatment

Malignant granuloma of the nose (malignant mesenchymoma of the mid-facial region) is an extremely rare disease, and therefore difficult to diagnose. In all the world literature by the end of the XX century. A little more than 100 cases of this disease have been described.

Malignant nasal granuloma was first described by McBride in 1897. Since then, many different names of this disease have appeared: malignant nasal granuloma, disfiguring nose granuloma and fatalities, progressive fatal granulation ulcers of the nose and face, granuloma gangrenesces, fatal median granuloma.

The cause and pathogenesis of malignant nasal granuloma. The reason is unknown. In pathogenetic terms, malignant nasal granuloma does not belong to systemic diseases. It occurs more often in men, occurs at any age. By analogy with local and disseminated lupus erythematosus, some authors consider malignant nasal granuloma as a local manifestation of Wegener's granulomatosis. The majority of researchers with this opinion do not agree and consider these diseases different nosological forms. As a risk factor, there is a history of trauma and inflammatory diseases of the rhinosinus region. In the process of studying the pathogenesis of this disease, various hypotheses of its occurrence have been expressed. Thus, the presence in the biopsy of malignant granuloma of the nose of the cells of Paltoff-Steenberg allowed to suggest that the disease is a consequence of lymphogranulomatosis, but the absence of adenobate in malignant granuloma rejected this assumption. It was also suggested that the malignant granuloma of the nose is a manifestation of the "specific" mycosis, malignant reticulosis, chronic pseudotumorous infection with a gangrenous development of the process without a specific pathogen, a kind of face allergy, but to date the pathogenesis of this disease is unclear.

Pathological anatomy of malignant nasal granuloma. At the heart of destructive necrotic changes in malignant nasal granuloma, as in Wegener's granulomatosis, is the granulomatous process, which is characterized by the absence of eosinophilic leukocytes in the granulomas.

The clinical course of the disease usually goes through three stages.

• I stage - prodromal, lasts from several weeks to several years. There is a periodic nasal lining accompanied by watery or serous-bloody discharge. Sometimes superficial ulcers appear on the threshold and on the septum of the nose, as well as in the soft palate.
• II stage - active process. Discharge from the nose becomes purulent or purulent-bloody with an unpleasant putrefactive odor. Nasal breathing is greatly hampered, the sense of smell worsens or disappears. On the mucous membrane of the nose appear green necrotic raids followed by creeping ulceration. The process passes to the nasopharynx, soft tissues of the external nose and other parts of the face. With the progression of the process, the palatine, nasal bones and the alveolar process of the upper jaw along with the soft tissues are necrotic. Simultaneously there is a lesion of the lacrimal canal (dacryocystitis).
• III stage - terminal, characterized by the appearance on the face of large defects due to necrosis of soft tissues; the septum of nose, nose conchas and wings of the nose are destroyed, necrotic ulcers in the pharynx and larynx can be observed, but the tongue is not involved in the pathological process. With such extensive destruction, the pain syndrome is usually absent or there is a slight soreness in the phase of initial necrosis. As a result of vascular arthrosis, bleeding occurs which, due to the massive and ongoing necrosis of the tissues, is difficult to stop only temporarily. As the disease progresses, a steady rise in the body temperature continues, reaching high values (40-41 ° C). Death comes from bleeding or cachexia in combination with a high body temperature.

Diagnosis of the disease is very difficult because of the absence of pathognomonic symptoms. In most cases of patients, before the establishment of a true diagnosis, several similar diseases are treated for others, from which the malignant granuloma of the nose should be differentiated - from syphilis, malignant neoplasms, chronic paranasal sinusitis, etc. In addition, the malignant granuloma of the nose should be differentiated from Wegener's granulomatosis, nomes in persons up to 15 years, leprosy, lupus, actinomycosis, scleroma, yaws, leishmaniasis, rhinosporidiosis, etc.

Treatment of malignant nasal granuloma. There is no specific treatment for malignant nasal granuloma. Minor, often temporary, results were obtained with X-ray therapy, massive use of corticosteroids. To combat superinfection, large doses of broad-spectrum antibiotics are prescribed. Surgical (galvanic caustic, surgical laser) excised necrotic tissues to reduce intoxication. Recovery is extremely rare.

The outlook for life is mostly unfavorable.

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