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Estesioneuroblastoma

 
, medical expert
Last reviewed: 23.04.2024
 
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Estesioneuroblastoma - this tumor among non-epithelial malignant neoplasms is the most frequent. It develops from the olfactory epithelium.

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

Symptoms of estesioneuroblastoma

The tumor is localized in the region of the upper nasal passage in the cells of the latticed labyrinth. It is a soft tissue polyp, which often fills the entire half of the nose. Therefore, the first clinical signs of it are difficulty in breathing through the corresponding half of the nose, serous-purulent, and often bloody discharge from the nose. The tumor quickly sprouts into the paranasal sinuses, the orbit, the base of the skull, the frontal lobe of the brain, metastasizes into the lymph nodes of the neck, the mediastinum, the lungs, the pleura and bones.

Depending on the pathways of the spread of estezioneuroblastoma, clinical-anatomical variants are distinguished:

  • The rhinological variant is the spread of the tumor to the front and middle cells of the latticed labyrinth, to the orbit, maxillary sinus, nasal cavity;
  • nasopharyngeal - the spread of the tumor to the posterior cells of the latticed labyrinth, into the choana and the nasopharynx;
  • neurological - the spread of the tumor to the base of the skull.

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Differential diagnosis of estezioneuroblastoma

In the early stages of diagnosis of the tumor is difficult, and in this case it is differentiated with chronic etmoiditis. When a tumor appears in the region of the upper nasal passage - differentiate with the polyp and other benign neoplasms. In a widespread process, when there is destruction of adjacent bone structures, differential diagnosis is performed with other malignant tumors in this area.

trusted-source[11], [12], [13], [14], [15], [16], [17]

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Treatment of estesioneuroblastoma

For a long time, there was an opinion that esteoneuroblastoma is not sensitive to radiation and drug treatment methods. The development of new chemotherapy regimens in combination with radiotherapy made it possible to obtain a significant clinical effect in the treatment of estezioneuroblastoma, sometimes with complete regression of the tumor process.

The main chemotherapy regimen is ASOP + cisplatin, which is performed in the following regimen: 1st day - doxorubicin 40 mg / ml, vincristine 2 mg, cyclophosphamide 600 mg / m 2 intravenously, strontaneously; on the 4th day, cisplatin is administered at a dose of 100 mg / m 2 against a background of 0.9% sodium chloride (2000 ml); 1 to 5 days of prednisolone at a dose of 1 mg / kg orally. After the first course of chemotherapy without interruption, radiotherapy is connected, which is carried out and two stages by a radical program. In a break between two stages of radiotherapy, a repeated course of chemotherapy is given.

Surgical treatment is carried out in the same volume as with the widespread cancer of the maxillary sinus and the nasal cavity and has its own peculiarities only in intracranial distribution. Since the trellis plate of the trellis is thin enough, the tumor often spreads into the cavity of the skull. In these cases, craniotic-fascial resections are performed in recent years, in which the bones of not only the facial but also the brain portion of the skull are included in the volume of tissues to be removed. Access at operations combined: external - through facial tissues and intracranial. The pericranial flap, guarded at this incision, delimits the brain from a defect in the nose and paranasal sinuses.

Forecast

Estezioneuroblastoma has an unfavorable prognosis. The five-year survival rate does not exceed 20-25%. In recent years, with the development of methods of chemoradiation treatment, it has been possible to improve the 5-year survival rate to 50-60%.

trusted-source[18], [19]

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