Esthesioneuroblastoma - Malignancy of the olfactory neuroepithelial cells that occurs in the nasal cavity and paranasal rapidly spreading in the sinus cavity of the orbit, the ethmoid bone and the base of the skull. The main symptoms are nasal congestion, copious mucus, anosmia, swelling of the malar area. The diagnosis esthesioneuroblastoma rely on rhinoscopy data CT of the paranasal sinuses, brain MRI, histological analysis of biopsy material. Treatment depends on the characteristics of the tumor, as a rule, is a combination of radiological, surgical and chemotherapeutic techniques.
Neuroblastoma accounts for about 3% of all tumors of the nasal cavity. As the tumor grows rapidly in the ethmoid cells and base of the skull, it is a matter of joint Supervision specialists in otolaryngology and neurology.
Metastasis typically affect the lungs, pleura, liver, and bone. It is characteristic that in the event of esthesioneuroblastoma patients pediatric regional and distant metastasis is relatively rare.
According to its morphological structure is esthesioneuroblastoma neyroepiteliomoy, it consists of undifferentiated ectodermal cells. Microscopic tumor tissue reveals the typical features of neurogenic tumor: presence of specific granular membranes and the formation of rosettes. Features histological structure allows to allocate three types of tumors: estezioneyroepiteliomu actually estezioneyroblastomu and estezioneyrotsitomu.
rhinitis, sinusitis or adenoiditis. Alarming point is the progression of the disorders of nasal breathing, despite ongoing rhinosinusitis therapy, and the development of complete anosmia - the inability to perceive odors. It should be noted that in the initial stage of nasal congestion is one-sided. As growth esthesioneuroblastoma septum is shifted to the healthy side, which leads to a narrowing of an intact nasal passage and bilateral nasal breathing disorder.
The further development of the disease depends on the direction of growth of the tumor and the place of its regional metastasis. Upon germination in the anterior nasal cavity, esthesioneuroblastoma fills it so that it can be detected by visual inspection of the nasal passages. If tumor growth occurs in the maxillary sinus, then there is swelling of the malar area on the affected side, and swelling of the upper jaw edge. Germination esthesioneuroblastoma the oral cavity leads to tooth loss and unsteadiness.
Distribution esthesioneuroblastoma into the cranial cavity manifests symptoms of hydrocephalus and increased intracranial pressure. These include: constant headache, a feeling of pressure in the eyeballs, nausea, having no connection with the meal. In later stages there are disorders of the cranial nerves, the roots of which are located on the base of the skull.
In some cases, through a trellised labyrinth cell esthesioneuroblastoma it extends into the orbital cavity. It is noted exophthalmos and difficulty of movements of the eyeball. In consequence of the eye displacement occurs diplopia (double vision). Esthesioneuroblastoma Metastasis in cervical lymph nodes leads to a swelling of the neck. When metastases in retropharyngeal lymph nodes, patients reported difficulty swallowing.
otolaryngologist, neurologist, neurosurgeon and ophthalmologist. Suspected tumor allows rhinoscopy data. Before its implementation elektroaspiratorom aspirated from the nasal cavity which prevents examination of thick mucus. Rhinoscopy reveals a dramatic shift of the nasal septum and the presence of the tumor mass, which has a reddish-bluish color and bumpy surface, often bleeding during the study. Rhinoscopy allows differentiate estezioneyroblastomu by a foreign body, nasal polyps and adenoids.
Esthesioneuroblastoma can be detected by X-ray diffraction data of the paranasal sinuses. A more informative methods include CT scan of the sinuses and MRI of the brain, which give detailed information on the size of the tumor and the boundaries of its circulation. In 70-50% of cases esthesioneuroblastoma tumor cells detected by cytological analysis of nasal discharge. However, their absence in the smear does not exclude tumor. Finally verify the diagnosis "esthesioneuroblastoma" is possible only on the results of histological examination of tumors tissue taken by biopsy in rhinoscopy.
Search of regional and distant metastases esthesioneuroblastoma performed using CT throat, neck tissue ultrasound, scintigraphy of the skeleton, chest CT, MRI and liver MDCT of the abdomen.
surgeons or oftalmoonkologii performed exenteration of the orbit, followed glazoprotezirovaniem.
Radiation therapy is effective as regards esthesioneuroblastoma and its regional metastases. It may take the form of proton therapy intensely modulated radiation therapy or brachytherapy. A special type of radiotherapy, radiosurgery appears. Last suitable for treatment with estezioneyroblastom inaccessible location, if you can not hold an open surgery due to severe somatic condition of the patient.
Chemotherapy is necessary when a significant distribution onkoprotsessa and presence of metastases. Usually it is held in the preoperative treatment and combined with irradiation.
In terms of prognostic esthesioneuroblastoma inspires little optimism. The aggressive growth of the tumor, its germination in the cavity of the skull and eye socket, metastasis and recurrence lead to rapid progression onkoprotsessa and patient death. Previously, 5-year survival does not exceed 25%. In recent years, thanks to the use of new techniques combined chemoradiotherapy, it increased to 50-60%. Adverse prognostic factors include age older than 50 and younger than 20 years, intracranial germination esthesioneuroblastoma her distant metastasis, a high degree of malignancy of tumor cells.