Estezioneyroblastoma — the malignant new growth from cages of an olfaktorny neuroepithelium arising in a cavity of a nose and quickly extending in paranasal bosoms, an eye-socket cavity, a trellised bone and the basis of a skull. The main symptoms are: nose congestion, plentiful mucous allocations, anosmiya, puffiness of malar area. In diagnostics of an estezioneyroblastoma rely on these rinoskopiya, KT of okolonosovy sine, MRT of a brain, the histologic analysis of biopsiyny material. Treatment depends on features of a tumor, as a rule, represents a combination of radiological, surgical and chemotherapeutic methods.
Estezioneyroblastoma (from Greek "esthesio" — I feel) originates in neurons of an olfactory (olfaktorny) epithelium in this connection the olfaktorny neuroblastoma is known under the name. The Olfaktorny epithelium covers a nose cavity, a nasopharynx and a trellised labyrinth. The neurons which are in it carry out allocation and transfer to the cerebral olfactory centers of information on quality and intensity of a smell. Estezioneyroblastoma rather rare malignant tumor. Most often it is observed at children aged after 10 years. About 3% of all new growths of a cavity of a nose fall to the share of an olfaktorny neuroblastoma. As the tumor quickly sprouts in cells of a trellised bone and on the skull basis, it is a subject of a joint kuration of experts in the field of otolaryngology and neurology.
Pathogenesis and morphology of an estezioneyroblastoma
Originally olfaktorny neuroblastoma settles down in the top departments of a nasal cavity. Having local aggressive growth, the estezioneyroblastoma quickly fills a nose cavity, extends in a nasopharynx, paranasal bosoms, a forward cranial pole and an orbit. Regionarny metastasis occurs in verkhnesheyny, zaglotochny, submandibular and parotid lymph nodes. The remote metastasises, as a rule, affect lungs, a pleura, a liver, bones. It is characteristic that at emergence of an estezioneyroblastoma at patients of children's age regionarny and remote metastasis is observed rather seldom.
On the morphological structure the estezioneyroblastoma is neyroepiteliomy, it consists of undifferentiated ektodermalny cages. The microscopy of fabrics of a tumor reveals typical lines of a neurogenetic tumor: presence of the specific granulated membranes and formation of sockets. Features of a histologic structure allow to distinguish 3 types of a tumor: estezioneyroepiteliy, actually estezioneyroblasty and estezioneyrotsity.
Classification of an estezioneyroblastoma
According to the International classification of tumors of a nose allocate 4 stages of an estezioneyroblastoma. In the I stage the tumor does not go beyond 1 wall of a cavity of a nose and a nasal sink, does not cause bone destruction and does not give metastasises. In IIA of a stage local bone destruction is observed, the estezioneyroblastoma takes other wall of a nasal cavity, but does not give metastasises. IIB a stage is similar to previous and differs from them in existence of a single regionarny metastasis on the party of developing of a tumor. Estezioneyroblastoma IIIA passes stages to the second half of a nasal cavity or out of limits of bone structures, extends to the next anatomic zones, but does not give metastasises. IIIB a stage — an estezioneyroblastoma, as in the previous stage or with smaller prevalence, but with multiple regionarny one - or bilateral metastasis. Estezioneyroblastoma IVA of a stage is characterized by germination in a nasopharynx, a cavity of a skull or soft facial tissues, but without metastasis. Identification of the remote or not movable regionarny metastasises corresponds to stage IVB, irrespective of degree of prevalence of tumoral process.
Characteristic manifestatsionny symptom of an olfaktorny neuroblastoma is the persistent congestion of a nose. It is followed at first by mucous, and then mucopurulent allocations. Originally such symptoms can be accepted to displays of rhinitis, an adenoidit or antritis. The guarding moment is progressing of violation of nasal breath, despite the carried-out therapy of a rinosinusit, and also development of a full anosmiya — impossibility to perceive smells. It should be noted that in an initial stage the congestion of a nose has unilateral character. In process of growth of an estezioneyroblastoma there is a shift of a nasal partition in the healthy party that leads to narrowing of the intact nasal course and bilateral disorder of nasal breath.
Further development of a disease depends on the direction of growth of a tumor and the place of its regionarny metastasis. At germination in forward departments of a cavity of a nose, an estezioneyroblastom fills it so that it can be found at visual survey of the nasal courses. If growth of a tumor happens in a maxillary bosom, then the swelling of malar area on the party of defeat and puffiness of edge of the top jaw appears. Germination of an estezioneyroblastoma in a mouth results in unsteadiness and loss of teeth.
Distribution of an estezioneyroblastoma to a cavity of a skull demonstrates symptoms of hydrocephaly and the increased intra cranial pressure. Treat them: a constant headache, pressure sense on eyeballs, the nausea which does not have communication with meal. In later stages there are violations from craniocereberal nerves which backs are located on the basis of a skull.
In certain cases through cells of a trellised labyrinth of an estezioneyroblastom extends in an orbital cavity. At the same time also difficulty of movements is noted by an eyeball . In the investigation of shift of an eye there is a diplopiya (doubling in eyes). Estezioneyroblastoma metastasis in cervical lymph nodes leads to some swelling of a neck. At metastasis in zaglotochny lymph nodes patients note the complicated swallowing.
Diagnostics of an estezioneyroblastoma
Diagnostic search at an estezioneyroblastoma is often carried out by joint efforts of the otolaryngologist, neurologist, neurosurgeon and ophthalmologist. These rinoskopiya allow to suspect a tumor. Before its carrying out by an electroaspirator suck away the dense slime preventing inspection from a nose cavity. Rinoskopiya finds the sharp shift of the nasal partition and presence of the tumoral weight having reddish coloring and a hilly surface, often bleeding when carrying out a research. Rinoskopiya gives the chance to differentiate estezioneyroblasty from a foreign matter, polyps of a nose and adenoides.
Estezioneyroblastoma can be found according to a X-ray analysis of okolonosovy bosoms. More informative methods are KT of nasal bosoms and MRT of a brain giving exhaustive information on the sizes of a tumor and borders of its distribution. In 70-50% of cases of an estezioneyroblastoma tumor cells are found in the cytologic analysis nasal separated. However their absence in dab does not allow to exclude a tumor. Finally it is possible to verify the diagnosis of Estezioneyroblastom only by results of a histologic research of the fabrics of a new growth taken by a biopsy during a rinoskopiya.
Search of the regionarny and remote metastasises of an estezioneyroblastoma is carried out with use of KT of a throat, ultrasonography of tissues of neck, a stsintigrafiya of a skeleton, KT of a thorax, MRT of a liver and MSKT of an abdominal cavity.
Treatment of an estezioneyroblastoma
Depending on the sizes and an arrangement of an estezioneyroblastoma, existence and localization of metastasises, and also age of the patient and the general state of his health, medical tactics can consist in surgical removal of a tumor, its radiation or system chemotherapy. As a rule, the combination of these methods is expedient.
Surgical removal of an estezioneyroblastoma is especially effective at the localized nature of a tumor without germination on the basis of a brain and metastasis. At the small size of a tumor surgery can be carried out transnazalno by means of the endoscope. Distribution of process to a forward cranial pole demands carrying out difficult neurosurgical operation. At the same time full removal of a tumor is not always possible. If the estezioneyroblastoma sprouts in an eye-socket, then with participation of oftalmokhirurg or oftalmoonkolog the eye-socket ekzenteration with the subsequent glazoprotezirovaniye is made.
Radiation therapy is effective as concerning an estezioneyroblastoma, and its regionarny metastasises. It can be carried out in the form of protonew therapy, the intensive modulated radiation therapy or brachytherapy. The radio surgery acts as a special type of radiation therapy. The last is suitable for treatment estezioneyroblasty with a remote arrangement, at impossibility of performing open surgery in connection with a serious somatic condition of the patient.
The chemotherapy is necessary at considerable distribution of an onkoprotsess and existence of metastasises. Usually it to be carried out at a preoperative stage of treatment and is combined with radiation.
Forecast of an estezioneyroblastoma
In the predictive plan of an estezioneyroblastom inspires not enough optimism. Aggressive growth of a tumor, its germination in a cavity of a skull and an eye-socket, metastasis and a retsidivirovaniye lead to bystry progressing of an onkoprotsess and death of the patient. Earlier 5-year survival did not exceed 25%. Recently, thanks to application of new techniques of the combined himioluchevy therapy, it increased to 50-60%. Adverse predictive factors are: the age is more senior 50 and 20 years, intrakranialny germination of an estezioneyroblastoma, its remote metastasis, high degree of an anaplaziya of cells of tumor are younger.