Esthesioneuroblastoma is a malignant neoplasm of olfactory neuroepithelial cells that occurs in the nasal cavity and spreads rapidly into the paranasal sinuses, the cavity of the eye socket, the latticed bone and the base of the skull. The main symptoms are: nasal congestion, abundant mucous discharge, anosmia, swelling of the zygomatic area. In the diagnosis of esthesioneuroblastoma, they rely on the data of rhinoscopy, CT of the paranasal sinuses, MRI of the brain, histological analysis of biopsy material. Treatment depends on the characteristics of the tumor, as a rule, it is a combination of radiological, surgical and chemotherapeutic methods.
ICD 10
C30.0 Malignant neoplasm of the nasal cavity
General information
Esthesioneuroblastoma (from the Greek “esthesio” — I feel) originates in the neurons of the olfactory (olfactory) epithelium, in connection with which it is known as olfactory neuroblastoma. The olfactory epithelium lines the nasal cavity, nasopharynx and the lattice labyrinth. The neurons located in it secrete and transmit information about the quality and intensity of the smell to the cerebral olfactory centers.
Esthesioneuroblastoma is a fairly rare malignant tumor. It is most often observed in children after the age of 10 years. Olfactory neuroblastoma accounts for about 3% of all neoplasms of the nasal cavity. Since the tumor quickly grows into the cells of the latticed bone and on the base of the skull, it is the subject of joint curation by specialists in the field of otolaryngology and neurology.
Pathomorphology
Initially, olfactory neuroblastoma is located in the upper parts of the nasal cavity. Possessing locally destructive aggressive growth, esthesioneuroblastoma quickly fills the nasal cavity, spreads into the nasopharynx, paranasal sinuses, anterior cranial fossa and orbit. Regional metastasis occurs in the upper cervical, pharyngeal, submandibular and parotid lymph nodes. Distant metastases, as a rule, affect the lungs, pleura, liver, bones. It is characteristic that when esthesioneuroblastoma occurs in pediatric patients, regional and distant metastasis is relatively rare.
According to its morphological structure, esthesioneuroblastoma is a neuroepithelioma, it consists of undifferentiated ectodermal cells. Microscopy of tumor tissues reveals typical features of a neurogenic tumor: the presence of specific granular membranes and the formation of rosettes. The features of the histological structure allow us to distinguish 3 types of tumors: esthesioneuroepithelioma, esthesioneuroblastoma proper and esthesioneurocytoma.
Classification
According to the International Classification of Tumors, there are 4 stages of esthesioneuroblastoma of the nose:
- In stage I, the tumor does not go beyond the 1st wall of the nasal cavity and nasal concha, does not cause bone destruction and does not metastasize.
- In stage IIa, local bone destruction is observed, esthesioneuroblastoma captures another wall of the nasal cavity, but does not metastasize.
- Stage IIb is similar to the previous ones and differs from them by the presence of a single regional metastasis on the side of the tumor origin.
- Esthesioneuroblastoma stage IIIa passes to the second half of the nasal cavity or beyond the bone structures, spreads to neighboring anatomical zones, but does not metastasize.
- Stage IIIb – esthesioneuroblastoma, as in the previous stage or with a lower prevalence, but with multiple regional single or bilateral metastasis.
- Esthesioneuroblastoma stage IVa is characterized by germination into the nasopharynx, skull cavity or soft tissues of the face, but without metastasis.
- The detection of distant or non-displaced regional metastases corresponds to stage IVb, regardless of the extent of the tumor process.
Symptoms
A characteristic manifestation symptom of olfactory neuroblastoma is persistent nasal congestion. It is accompanied first by mucous, and then by mucopurulent secretions. Initially, such symptoms may be mistaken for manifestations of rhinitis, adenoiditis or sinusitis. An alarming moment is the progression of nasal breathing disorders, despite the ongoing therapy of rhinosinusitis, as well as the development of complete anosmia — the inability to perceive odors.
It should be noted that in the initial stage, nasal congestion is unilateral. As esthesioneuroblastoma grows, the nasal septum shifts to the healthy side, which leads to a narrowing of the intact nasal passage and a bilateral disorder of nasal breathing.
The further development of the disease depends on the direction of tumor growth and the place of its regional metastasis. When germinating into the anterior parts of the nasal cavity, esthesioneuroblastoma fills it so that it can be detected by visual examination of the nasal passages. If the growth of the tumor occurs in the maxillary sinus, then there is a swelling of the zygomatic area on the side of the lesion and swelling of the edge of the upper jaw. The germination of esthesioneuroblastoma into the oral cavity leads to shakiness and loss of teeth.
The spread of esthesioneuroblastoma into the cranial cavity manifests signs of hydrocephalus and increased intracranial pressure. These include: constant headache, feeling of pressure on the eyeballs, nausea, which has no connection with eating. In later stages, disorders occur on the part of the cranial nerves, the roots of which are located on the base of the skull.
In some cases, through the cells of the lattice labyrinth, the esthesioneuroblastoma spreads into the orbital cavity. At the same time, exophthalmos and difficulty in moving the eyeball are noted. As a result of the displacement of the eye, diplopia (double vision) occurs. The metastasis of esthesioneuroblastoma into the cervical lymph nodes leads to some swelling of the neck. With metastasis to the pharyngeal lymph nodes, patients note difficulty swallowing.
Diagnostics
Diagnostic search for esthesioneuroblastoma is often carried out by the joint efforts of an otolaryngologist, neurologist, neurosurgeon and ophthalmologist. Instrumental visualization methods are used:
- Rhinoscopy. Before it is carried out, the thick mucus interfering with the examination is sucked out of the nasal cavity with an electric respirator. Rhinoscopy reveals a sharp displacement of the nasal septum and the presence of a tumor mass having a reddish-bluish color and a bumpy surface, often bleeding during the study. Rhinoscopy makes it possible to differentiate esthesioneuroblastoma from a foreign body, nasal polyps and adenoids.
- X-ray. Esthesioneuroblastoma can be detected by radiography of the paranasal sinuses. More informative methods are CT of the nasal sinuses and MRI of the brain, which provide comprehensive information about the size of the tumor and the boundaries of its spread.
- Methods of cytomorphology. In 70-50% of cases of esthesioneuroblastoma, tumor cells are detected by cytological analysis of the nasal discharge. However, their absence in the smear does not exclude a tumor. It is possible to finally verify the diagnosis of “Esthesioneuroblastoma” only based on the results of histological examination of neoplasm tissues taken by biopsy during rhinoscopy.
The search for regional and distant metastases of esthesioneuroblastoma is carried out using CT of the pharynx, ultrasound of neck tissues, skeletal scintigraphy, CT of the chest, MRI of the liver.
Treatment
Depending on the size and location of the esthesioneuroblastoma, the presence and localization of metastases, as well as the age of the patient and his general state of health, therapeutic tactics may consist in surgical removal of the tumor, its irradiation or systemic chemotherapy. As a rule, it is advisable to combine these methods.
Surgical tactics
Surgical removal of esthesioneuroblastoma is especially effective in the localized nature of the tumor without germination to the base of the brain and metastasis. With a small tumor size, surgical intervention can be performed transnasally using an endoscope. The spread of the process into the anterior cranial fossa requires a complex neurosurgical operation. At the same time, complete removal of the tumor is not always possible. If esthesioneuroblastoma grows into the eye socket, then with the participation of ophthalmic surgeons or ophthalmologists, the exenteration of the eye socket is performed, followed by ocular prosthetics.
Antitumor therapy
Radiation therapy is effective both against esthesioneuroblastoma and its regional metastases. It can be carried out in the form of proton therapy, intensely modulated radiation therapy or brachytherapy. Radiosurgery is a special type of radiation therapy. The latter is suitable for the treatment of esthesioneuroblastoma with a hard-to-reach location, if it is impossible to perform open surgery due to the severe somatic condition of the patient.
Chemotherapy is necessary with a significant spread of the cancer process and the presence of metastases. It is usually carried out at the preoperative stage of treatment and combined with radiation.
Forecast
In prognostic terms, esthesioneuroblastoma inspires little optimism. Aggressive tumor growth, its germination into the cranial cavity and eye socket, metastasis and recurrence lead to rapid progression of the cancer process and death of the patient. Previously, the 5-year survival rate did not exceed 25%. Recently, thanks to the use of new methods of combined chemoradiotherapy, it has increased to 50-60%. Unfavorable prognostic factors are: age older than 50 and younger than 20 years, intracranial germination of esthesioneuroblastoma, its distant metastasis, a high degree of anaplasia of tumor cells.
