Ganglioneuroma (ganglioma, ganglionic neuroma, gangliocytoma) is a benign tumor formed from elements of sympathetic nerve nodes (ganglia). Disease can have a very different localization. Most often it is associated with various parts of the spine, can be located in the brain, less often — in the adrenal glands, gastrointestinal tract, skin, bladder wall. The clinical symptoms that manifest ganglioneuroma are not specific and depend on the location of the tumor. Diagnosis is carried out mainly by computer and magnetic resonance imaging, as well as by histological examination of a biopsy sample of tumor tissues. Ganglioneuroma treatment is only possible surgically.
D36.1 Benign neoplasm of peripheral nerves and autonomic nervous system
Ganglioneuroma is a tightly elastic node that does not have a distinct capsule. The shape of the node may be irregular or rounded. The ganglioneuroma has a lobular or fibrous structure on the incision; the whitish-gray color of the tumor is characteristic. Microscopically, ganglioneuroma consists of nerve fibers, ganglion cells and connective tissue fibers. The ratio of the structural elements of ganglioneuroma can be very different. Thus, ganglion cells may be isolated in the preparation, and may be present in large numbers; the number of connective tissue fibers can be both significant and minimal; nerve fibers are more often of the limpid type, but there are ganglioneuromas, which include myelin nerve fibers.
In modern neurology, the opinion comes to the fore that ganglioneuroma occurs as a result of disorders occurring in the intrauterine period during the laying of the sympathetic nervous system. This assumption is confirmed by the frequent presence of other congenital malformations in patients with ganglioneuroma (abnormalities of the spine, cleft palate, cleft lip, congenital basilar impression, assimilation of Atlas, Kimerli anomaly, etc.).
Probably the factors contributing to the formation of ganglioneuroma are adverse effects on the mother and fetus. This is not only radiation exposure, but also various infections (rubella, cytomegalovirus, measles, syphilis, chlamydia, etc.), intoxication (alcohol, narcotic substances, chemicals), chronic diseases of the mother, leading to fetal hypoxia.
Depending on the localization of the process, the following types of tumors are distinguished: ganglioneuromas of the brain, ganglioneuromas of the sympathetic nerve trunk, ganglioneuromas of the adrenal glands and ganglioneuromas of other localization (skin, bladder, stomach or intestinal wall). Of significant clinical importance is the division of ganglioneuromas into mature and maturing ones, of which the former have a more favorable prognosis.
More than 50% of cases of ganglioneuroma are found in people younger than 20 years. According to some reports, this tumor more often affects women. The clinical manifestations of ganglioneuroma depend on its location, size and distribution into the anatomical structures adjacent to it.
Most often ganglioneuromas originate from the cells of the sympathetic trunk at the level of various parts of the spine. At the same time, depending on the level of location, the tumor spreads into the spinal canal and posteriorly — into the posterior mediastinum, retroperitoneal space or pelvic tissue. Cervical tumors are less common. Ganglioneuroma, which grows into the spine, has the appearance of an hourglass, since it consists of two nodes, one of which is located paravertebral, and the second in the spinal canal. Clinically, such a tumor is manifested by symptoms of compression of the spinal cord, and when germinating into it — by a nonspecific clinic of a spinal cord tumor. Ganglioneuroma is characterized by slow growth, which, when localized in the retroperitoneal space, allows it to reach enormous sizes without causing any clinical symptoms.
Ganglioneuroma of the brain is more often called gangliocytoma. It accounts for 0.1 to 0.4% of all intracranial tumors. The tumor can be localized in any part of the brain, but the most common are ganglioneuromas of the bottom of the III ventricle, hypothalamus, subcortical structures, temporal and frontal lobes, as well as diffuse ganglioneuroma of the cerebellar cortex. As a rule, a gangliocytoma has a size of no more than 3-4 cm, is characterized by slow growth and complete absence of a capsule. Such a tumor can spread in the form of outgrowths into the brain tissue and germinate the meninges. Ganglioneuroma of the brain has no specific symptoms. Like other intracerebral tumors, ganglioneuroma manifests itself with cerebral and focal symptoms corresponding to its location. Intracerebral ganglioneuroma can undergo a blastomatous transformation, degenerating into a malignant neuroblastoma (ganglioneuroblastoma).
In rare cases, ganglioneuroma has an organ localization. It can affect the adrenal glands, gastrointestinal organs, and bladder. At the same time, there is a clinical picture characteristic, respectively, of tumors of the adrenal glands, tumors of the stomach, esophagus or small intestine, a benign tumor of the bladder.
The characteristic slow growth leads to prolonged asymptomatic development of ganglioneuroma and makes it difficult to diagnose it in a timely manner. In some cases, it is an accidental finding during the examination of the patient. Thus, during radiography of the spine or thoracic organs, the ganglioneuroma can be visualized as a homogeneous darkening of a rounded shape adjacent to the spine. In the case of a large tumor, there may be a symptom of “spreading of the ribs” and the appearance of “usures” (indentations) on them due to the constant pressure of the tumor. When ganglioneuroma is malignant, destructive changes of vertebrae and ribs located next to it are radiologically determined.
A wide range of localization of ganglioneuroma contributes to the fact that not only neurologists, but also vertebrologists, gastroenterologists, endocrinologists, urologists and other specialists face it in their practice. A neurologist can clinically suspect a tumor based on the results of a neurological examination of the patient. To confirm the diagnosis, depending on the location of the tumor, it is necessary to conduct an MRI or CT of the spine, CT of the chest and mediastinum, MRI of the brain. It is possible to confirm the diagnosis of a tumor formation and verify it as a ganglioneuroma only with a histological examination of a tissue sample obtained during a puncture biopsy of a vertebra or a stereotactic biopsy of the brain.
With regard to ganglioneuroma, only surgical treatment is possible. It is carried out in accordance with the principles of spinal surgery and surgical interventions for brain tumors. The complexity of radical removal of the tumor lies in the fact that it often has significant dimensions and is located in vital structures of the central nervous system, surgical intervention on which is fraught with danger to the life of the operated. The possibility of surgery and its scope is considered by the neurosurgeon individually in each case.
Complete removal of an hourglass-type tumor often requires two surgical approaches: laminectomy and opening of the thoracic or abdominal cavity. Such an operation may require the participation of not only neurosurgeons, but also thoracic surgeons. Radical removal of an intramedullary tumor is often not possible. In such cases, the ganglioneuroma is partially removed, decompression of the spinal cord is performed and restoration of cerebrospinal fluid circulation.
Removal of ganglioneuroma of the brain is often a palliative intervention with partial removal of the tumor due to its large size and significant spread in the brain tissue. If it is impossible to completely remove ventricular tumors, surgical intervention is supplemented with a bypass operation — ventriculoperitoneal bypass or external ventricular drainage. After removal of the ganglioneuroma, a mandatory histological examination of the tumor is performed to exclude its transformation into ganglioneuroblastoma.
Mature ganglioneuromas of small size, successfully removed surgically, have a favorable prognosis, although in some cases their recurrence is observed. Large tumors and maturing ganglioneuromas are less favorable prognostically. Tumors with blastomatous degeneration have the worst prognosis for both recovery and life.