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

Malignant granuloma of the nose (malignant mesenchymoma of the midfacial region) is an extremely rare disease and therefore difficult to diagnose. In the entire world literature by the end of the 20th century, slightly more than 100 cases of this disease were described.

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

Cause and pathogenesis of malignant granuloma of the nose. The cause is unknown. In pathogenetic terms, malignant granuloma of the nose is not a systemic disease. It occurs more often in men and at any age. By analogy with local and disseminated lupus erythematosus, some authors consider malignant granuloma of the nose to be a local manifestation of Wegener's granulomatosis. Most researchers disagree with this opinion and consider these diseases to be different nosological forms. The presence of a history of trauma and inflammatory diseases of the rhinosinus region in the anamnesis are noted as risk factors. In the process of studying the pathogenesis of this disease, various hypotheses were put forward for its occurrence. Thus, the presence of Paltoff-Sternberg cells in the biopsy of malignant granuloma of the nose allowed us to suggest that the disease is a consequence of lymphogranulomatosis, but the absence of adenopathy in malignant granuloma rejected this suggestion. It was also assumed that malignant granuloma of the nose is a manifestation of “specific” mycosis, malignant reticulosis, chronic pseudotumor infection with gangrenous development of the process without a specific pathogen, a kind of facial allergy, but to date the pathogenesis of this disease is unclear.

Pathological anatomy of malignant granuloma of the nose. The destructive-necrotic changes in malignant granuloma of the nose, as in Wegener's granulomatosis, are based on a granulomatous process, which is characterized by the absence of eosinophilic leukocytes in granulomas.

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

• Stage I - prodromal, lasts from several weeks to several years. Periodic nasal congestion is observed, accompanied by watery or serous-bloody discharge. Sometimes superficial ulcers appear in the vestibule and on the nasal septum, as well as on the soft palate.
• Stage II - active process. Nasal discharge becomes purulent or purulent-bloody with an unpleasant putrid odor. Nasal breathing becomes severely difficult, the sense of smell worsens or disappears. Green necrotic plaque appears on the mucous membrane of the nose, followed by creeping ulceration. The process moves to the nasopharynx, soft tissues of the external nose and other parts of the face. As the process progresses, the palatine, nasal bones and alveolar process of the upper jaw together with soft tissues become necrotic. At the same time, damage to the lacrimal ducts (dacryocystitis) occurs.
• Stage III is terminal and is characterized by the appearance of large defects on the face due to necrosis of soft tissues; the nasal septum, nasal conchae and nasal wings are destroyed, necrotic ulcers may be observed in the pharynx and larynx, but the tongue is not involved in the pathological process. With such extensive destruction, pain syndrome is usually absent or minor soreness is noted in the phase of initial necrosis. As a result of vascular erosion, bleeding is observed, which, due to massive and ongoing tissue necrosis, is difficult to stop only temporarily. As the disease progresses, there is a steady increase in body temperature of the continue type, reaching high values (40-41 ° C). Death occurs from bleeding or cachexia in combination with high body temperature.

Diagnosis of the disease is very difficult due to the lack of pathognomonic symptoms. In most cases, patients are treated for other somewhat similar diseases before the true diagnosis is established, from which malignant granuloma of the nose should be differentiated - from syphilis, malignant neoplasms, chronic paranasal sinusitis, etc. In addition, malignant granuloma of the nose should be differentiated from Wegener's granulomatosis, noma in people under 15 years of age, leprosy, lupus, actinomycosis, scleroma, yaws, leishmaniasis, rhinosporidiosis, etc.

Treatment of malignant granuloma of the nose. There is no specific treatment for malignant granuloma of the nose. Insignificant, often temporary, results are obtained with X-ray therapy, massive use of corticosteroids. Large doses of broad-spectrum antibiotics are prescribed to combat superinfection. Necrotic tissue is excised surgically (galvanocautery, surgical laser) to reduce intoxication. Recovery is extremely rare.

The prognosis for life is generally unfavourable.

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