Hordoma – seldom found new growth which is presumably coming from the chord remains. Now is considered as malignant more often. Arises near a backbone, can settle down at any level, from a tailbone to the skull basis. Usually develops in sacral and coccygeal department or occipital parts of a skull. Symptoms of a hordoma are defined by localization of a tumor and extent of damage of various nerves. Pains, paresis, violations of sensitivity and disorder of functions of pelvic bodies are possible. The diagnosis is exposed taking into account data of neurologic survey, a X-ray analysis, KT, MPT and biopsy. Treatment – operation, radiation therapy.
Hordoma – the tumor of various degree of a zlokachestvennost which is presumably arising from the embryonic predecessor of a backbone. Always settles down near a spine column. It is usually localized in the field of a sacrum or the basis of a skull, but can strike also other departments. Can meet at any age. Hordoma in the field of the basis of a skull tumors of sacral department – at the age of 40-60 years are more often diagnosed at the age of 20-40 years. Men suffer twice more often than women. The reasons and the contributing factors influencing probability of development of oncological process remain obscure so far. Prevalence of a disease – 1-3 new cases of a hordoma a year on 1 million population. Treatment is carried out by experts in the field of oncology, neurology and a vertebrologiya.
Pathological anatomy of a hordoma
The question of a zlokachestvennost of a new growth remains diskutabelny. One researchers allocate good-quality and malignant forms to chords, others regard such tumors as rather good-quality because of their slow growth and rare metastasis. At the same time, because of high tendency to a retsidivirovaniye and a large number of failures modern experts are inclined to consider hordy as malignant process.
Hordoma represents the soft single knot covered with well expressed capsule. At a malignant current encapsulation is looked through poorly. The new growth has a lobular structure, on a section of a hordom translucent, light gray, in fabric sites of a necrosis are visible. At microscopy polymorphism of cages comes to light. Cellular kernels small, hyperchromic. In cages large vacuoles are visible. In malignant hordoma the cellular anaplaziya and atypical figures of a mitosis come to light.
Symptoms depend on localization, the sizes and the direction of growth of a tumor. To the forefront in a clinical picture usually there are signs of damage of these or those nerves. Hordoma, the skull bases located in a zone, can extend to the area of the Turkish saddle, raise deep into skulls, in the direction of a nasopharynx or an orbit. At a sdavleniye of a hypophysis there can be hormonal violations. The bulbarny frustration resulting from damage of the wandering, hypoglossal and yazykoglotochny nerve are characteristic.
At the patients suffering hordomy the skull bases a dizartriya, violations of swallowing and change of a voice (weakness, twang) up to an aphonia can come to light. At widespread hordoma the violations of a rhythm of breath and warm activity caused by involvement of the respiratory and cardiovascular centers located in a medulla are observed. Violations of sight owing to a sdavleniye of an optic nerve are possible. At distribution of process germination of an orbit or a nasopharynx can be observed.
Hordoma of a sacrum can extend as it is superficial, and in the direction of an abdominal cavity or a small pelvis. At a sdavleniye of a spinal cord and nervous backs there are pains, weakness of the lower extremities, disorders of sensitivity and movements, paresis and violations of activity of pelvic bodies. At a hordoma arrangement in the atypical place (in the field of cervical, chest or lumbar department of a backbone) pains and the neurologic frustration corresponding to defeat level are observed.
At a malignant current rapid growth of a new growth is noted. Hordoma sprouts vertebras or bones of a skull, gives metastasises in regionarny lymph nodes, a liver and lungs. At late stages the picture of cancer intoxication comes to light. Patients of hordomy lose the weight and appetite, feel constant weakness. Mood swings, depressive frustration, anemia and a hyperthermia are observed. At metastasises in lungs there are short wind and cough with blood, at metastasises in a liver – a gepatomegaliya, jaundice and ascites.
Diagnostics of a hordoma
The diagnosis is exposed taking into account the anamnesis, complaints of the patient, the yielded general and neurologic survey and results of inspection. To patients with suspicion on hordy appoint a X-ray analysis of a skull or a X-ray analysis of sacral and coccygeal department of a backbone (depending on localization of a new growth). On roentgenograms large sites of destruction of a bone tissue are found. Sometimes in the field of bone defect the thin partitions dividing a cavity into several cameras are looked through. At a hordoma arrangement in sacral department increase in the perednezadny size of a sacrum comes to light. At localization of a tumor in a skull the rassasyvaniye of a wedge-shaped bone is noted.
For more exact determination of the size, structure and prevalence of a hordoma appoint KT of a brain, MRT of a brain, KT of a backbone and MRT of a backbone. For definition of an involvement of vessels carry out an angiography. The final diagnosis of a hordoma is established on the basis of a biopsy and the subsequent histologic research of a sample of fabrics. Material is taken under X-ray control. The condition of regionarny lymph nodes is estimated taking into account data of ultrasonography of the struck area. For identification of metastasises in lungs carry out a X-ray analysis of a thorax and KT of lungs, for detection of the secondary centers in a liver – KT of abdominal organs and MRT of a liver.
For assessment of the general condition of an organism to the patients suffering from hordomy appoint the general blood test, the general analysis of urine and biochemical blood test. During preoperative preparation define a blood type and a Rhesus factor. In the presence of somatic pathology of the patient direct to consultation to doctors of the corresponding profile (the cardiologist, the gastroenterologist, the endocrinologist etc.). Differential diagnostics of a hordoma is performed with new growths back and a brain, hondrosarkomy a sacrum, and also the tumors of female genitals and cancer of a large intestine extending to area of a backbone.
Treatment and the forecast at a hordoma
The main method of treatment of a hordoma is surgical intervention. Because of features of an arrangement and growth of a tumor radical removal is possible only at an insignificant part of patients. At a possibility of radical surgery carry out a full resection of a hordoma together with the capsule. In other cases perform palliative operations. Partial excision of a hordoma allows to reduce a sdavleniye of nervous structures and it is essential to improve quality of life of patients.
Radiation therapy at hordoma is ineffective, however because of big technical difficulties when performing surgery this method quite often gains essential value in the course of palliative therapy. The radiotheraphy provides decrease in intensity of a pain syndrome and some reduction of the sizes of a hordoma. Radiation is carried out, using the x-ray device or a gamma-ray irradiation plant. Along with traditional methods of radiation therapy apply stereotaksichesky surgery during which aim influence by thin bunches of gamma beams on area of a hordoma is carried out. The chemotherapy in treatment of this pathology is not applied because of resistance of a tumor to action of himiopreparat. Because of a frequent retsidivirovaniye repeated operations and courses of radiation therapy are required for many patients.
The forecast at hordoma is defined by the new growth zlokachestvennost level, prevalence of oncological process and success of the first surgical intervention. Average five-year survival at malignant forms of a hordoma makes about 30% Average life expectancy of the patients having this pathology fluctuates from 5 to 10 years. The recurrence of a hordoma is considered as predictively an adverse sign.