Medical expert of the article
New publications
Esthesioneuroblastoma
Last reviewed: 04.07.2025
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
[ Symptoms of Esthesioneuroblastoma
The tumor is localized in the area of the upper nasal passage in the cells of the ethmoid labyrinth. It is a soft tissue polyp, often filling the entire half of the nose. Therefore, its first clinical signs are difficulty breathing through the corresponding half of the nose, serous-purulent, and often bloody discharge from the nose. The tumor quickly grows into the paranasal sinuses, orbit, base of the skull, frontal lobe of the brain, metastasizes to the lymph nodes of the neck, mediastinum, lungs, pleura and bones.
Depending on the routes of spread of esthesioneuroblastoma, clinical and anatomical variants are distinguished:
- rhinological variant - spread of the tumor to the anterior and middle cells of the ethmoid labyrinth, into the orbit, maxillary sinus, nasal cavity;
- nasopharyngeal - tumor spread to the posterior cells of the ethmoid labyrinth, into the choana and nasopharynx;
- neurological - tumor spread to the base of the skull.
What do need to examine?
How to examine?
Differential diagnosis of esthesioneuroblastoma
In the early stages, tumor diagnostics are difficult, and in this case, it is differentiated from chronic ethmoiditis. When a tumor appears in the upper nasal passage, it is differentiated from a polyp and other benign neoplasms. In a widespread process, when there is destruction of adjacent bone structures, differential diagnostics are carried out with other malignant neoplasms in this area.
Who to contact?
Treatment of esthesioneuroblastoma
For a long time, there was an opinion that esthesioneuroblastoma is not sensitive to radiation and drug treatment methods. The development of new chemotherapy regimens in combination with radiation therapy allowed to obtain a significant clinical effect in the treatment of esthesioneuroblastoma, sometimes with complete regression of the tumor process.
The main chemotherapy regimen is ASOR + cisplatin, which is carried out in the following mode: 1st day - doxorubicin 40 mg / ml, vincristine 2 mg, cyclophosphamide 600 mg / m 2 intravenously, jet; on the 4th day, cisplatin is administered at a dose of 100 mg / m 2 against the background of a load of 0.9% sodium chloride solution (2000 ml); from the 1st to the 5th day, prednisolone at a dose of 1 mg / kg orally. After the first course of chemotherapy, radiation therapy is connected without a break, which is carried out in two stages according to the radical program. During the break between the two stages of radiation therapy, a repeat course of chemotherapy is given.
Surgical treatment is performed in the same volume as for widespread cancer of the maxillary sinus and nasal cavity and has its own characteristics only in case of intracranial spread. Since the cribriform plate of the ethmoid bone is quite thin, the tumor often spreads into the cranial cavity. In these cases, craniofascial resections have been performed in recent years, in which the volume of tissue removed includes not only the bones of the facial but also the cerebral part of the skull. Access during operations is combined: external - through facial tissue and intracranial. The pericranial flap, protected during this incision, separates the brain from the defect in the area of the nose and paranasal sinuses.