CRANYOPHARYNGIOMA (craniopharyngioma; grech, kranion a skull + pharynx of a drink + - oma a tumor; synonym: tumor of a pocket of Ratke, tumor of the pituitary course, Erdgeym's tumor) — the inborn tumor of a brain of an epithelial structure developing from embryonic cells of the pituitary course — a so-called pocket of Ratke. To. — usually benign tumor; its malignancy is observed extremely seldom. This type of tumors of a brain was in details described for the first time by J. Erdheim in 1904.
Frequency To. in relation to tumors of a brain from 2 to 4,5% hesitate, and children have from 7 to 13,4%. To. meet at any age, and P. Wertheimer points to the considerable frequency of a disease To. (27%) aged after 40 years.
In the direction of growth (fig.) distinguish three groups of a cranyopharyngioma: 1) localized under a diaphragm of the Turkish saddle — endosellyarny; 2) over a diaphragm — suprasellyarny; 3) growing subsellyarno and getting into wedge-shaped and trellised bosoms towards nasal cavities and drinks.
Distinguish two main types of a tumor: consisting of dense fabric and cystous.
To. — the tumor (is more often in the form of a single dense node) growing slowly, expansive. The size of its from 2 to 5 cm in the diameter, is more rare more (tsvetn. fig. 6). Kistozno the regenerated sites of a tumor contain from 10 to 50 ml (in rare instances to 200 ml) dense liquid of yellow, amber or coffee color. To. usually has the dense capsule which is quite closely connected with the brain fabric surrounding it, covers and vascular network. Blood supply of a tumor is carried out from branches of an arterial circle of a great brain.
Structure To. eventually can be exposed to considerable changes. In its compact layers there is a kollikvatsionny necrosis to formation of the cysts containing liquid with a large amount of protein (from 20 to 100 per milles and more), the amorphous remains of the died cellular elements and blood, crystals of cholesterol, fat to - t to adjournment of salts on an inner surface of the capsule and in fabric of the tumor. Histologically To. consist of epithelial cells of different extent of differentiation. Along with epithelial cells of embryonal type also the epithelium of epidermal type meets. Cellular accumulations in some cases can remind adamantinomas. V K. dystrophic changes of different degree of manifestation in the form of a kistoobrazovaniye, calcifications or even ossifications of a stroma are always observed. Capsule K. consists of connecting or glial fabric. A variety in a structure To. it is regarded by a number of authors as result of staging of its development.
A clinical picture
Clinically To. flows wavy, sometimes with long stabilization patol, process with the subsequent sharp aggravation and progressing. It is connected with the phenomena regressive a metamorphosis, occurring in tumoral fabric. Duration of a disease fluctuates from several months to 10 — 15 and more years.
Against the background of symptoms, the general for all tumors brain (see), three main signs act.
1. Bitemporal hemianopsia (see), observed approximately at 70% of patients, and at first loss of fields of vision for the colors is noted. At the bottom of an eye take place of the phenomenon of primary atrophy, and at emergence of the likvorny block — usually a papilledema (see. Eyeground, table. ). The considerable difference in visual acuity of the right and left eye is sometimes noted. At hemilesion a tumor of a visual tract the gomonimny hemianopsia develops, ptosis (see).
2. The endocrine and exchange disturbances noted approximately at 85% of patients. With an endosellyarny growth early there is an oppression of functions of a hypophysis that is shown by a nanism (see. Dwarfism ) and infantility (see); at development of a disease at the advanced children's age and at adults fatty, water and carbohydrate metabolism sharply changes, the sexual potentiality decreases, there comes the amenorrhea. The more influence of the growing tumor on diencephalic departments of a brain and the third ventricle, the vegetative disturbances with disorder of thermal control, a sleep disorder, the general adynamia, a polydipsia, a polyphagia, arterial hypotonia act more sharply (see. Hypothalamic syndrome ). Further the cachexia develops.
3. Petrifikata over the Turkish saddle are observed in 60 — 75% of cases; their existence confirms the diagnosis To. Besides, also porozna the back inclined shoots usually are considerably changed, sometimes the dorsum sellae almost completely atrophies. With an endosellyarny growth To. there occur the changes in the Turkish saddle typical for adenomas of a hypophysis.
With a growth of a tumor of a kpereda from decussation of an optic nerve the progressing loss of sense of smell is noted (see. Anosmia ). With a retrosellyarny growth the hl comes to light. obr. basal symptomatology with a number of trunk disturbances and oppression of functions of subcrustal educations. At approach of disorders of circulation of cerebrospinal liquid and progressing of a secondary edema of a brain gipertenzionno-gidrotsefalny symptoms begin to appear. The progressing all-brain phenomena join local brain pathology. With a growth of cysts in a cavity of the third ventricle and obstruction of interventricular foramens or a water supply system of a brain there are heavy gipertenzionny crises of an occlusal origin with sharp chetverokholmny and trunk symptoms (see. Occlusal syndrome ).
At occlusion of outflow tracts of cerebrospinal liquid in bones of a calvaria the changes characteristic come to light for hydrocephaly (see) that is especially noticeable at development To. at children's age.
Approximately in 50% of cases To. cerebrospinal liquid on the structure normal. When there is a formation of cysts and more rapid growth of a new growth begins, in cerebrospinal liquid small increase in amount of protein is noted, there is a cytosis (see. Cerebrospinal liquid ). Approximately in 25% of observations breaks of tumoral cysts are noted that is clinically shown by sharply arisen sharp headache, repeated vomitings, motive concern, temperature increase to 40 — 41 °, disturbance of consciousness, quite often epileptic seizures, coma, disorder of cardiovascular activity. In cerebrospinal liquid the xanthochromia, increase in protein to 10 per milles and a big pleocytosis are observed. High temperature at patients can keep several days; the rough shell phenomena, falling of visual acuity (it is sometimes temporary to a blindness), icteric coloring of scleras and integuments are noted.
the Diagnosis does not represent difficulties when over the Turkish saddle there are petrifikata. For detection To. make lumbar and ventrikulyarny punctures (see. Ventriculopuncture , Spinal puncture ), pneumoencephalography (see), ventrikulografiya (see) and an angiography (see. Vertebralnaya angiography , Carotid angiography ). Valuable information on the size and an arrangement of a tumor and its relation to ways of circulation of cerebrospinal liquid can be received at a pnevmotsisternografiya (pneumoencephalography). Accuracy of a method increases if it is combined with tomography (see). At passability of ways of circulation of cerebrospinal liquid it is possible to fill with air the third ventricle that even more specifies the size and the location of a tumor.
At occlusion of ways of circulation of cerebrospinal liquid it is necessary to apply a ventrikulografiya through front horns of lateral cerebral cavities. For the purpose of detection of cysts the needle is directed to the centerline to depth of 8 — 10 cm. If the needle got to a cyst, then after extraction of its contents the cavity is filled with air or contrast medium then it is possible to receive its image on roentgenograms. With a retrosellyarny growth of a tumor, in addition to a carotid angiography, contrasting of vertebrata and basilar arteries is reasonable.
For topical diagnosis To. use isotope scanning (see) and a so-called axial computer tomography (see).
Depending on the size and an arrangement To. apply different types of the operational or combined treatment.
With development of the microsurgical method allowing to carry out difficult operations more carefully, keeping the vital structures of a brain and their krovosnabzhayushchy vessels (see. Microsurgery ), attempts of the most radical oncotomy are the most reasonable. For this purpose apply different quick accesses. Use unilateral subfrontal approach more often. Even at To. the big sizes it is possible to remove from this access considerably a tumor, especially if it contains big cysts. Emptying of a cyst in an initial stage of operation gives an additional scope for the surgeon. At the phenomena of occlusal hydrocephaly the puncture of a front horn of a side ventricle and removal of cerebrospinal liquid also considerably facilitate the subsequent actions.
In some cases at very widespread tumors it is reasonable to resort to bilateral trepanation in frontal area with crossing of a sagittal sine and crescent shoot in their front departments. If the tumor has preferential retrosellyarny distribution, it is reasonable to apply subtemporal approach with a section is mashed a cerebellum. With a growth of a tumor in a side ventricle it can be removed by the combined approach: subfrontal — for removal hiazmalno the located part K. and transventrikulyarny (with a section of a brain in the field of frontal crinkles) — for removal of its intraventricular part. Also other approaches are in some cases applicable (e.g., a section of front departments of a corpus collosum and transsphenoidal — with preferential subsellyarny growth To.). Removal To. it is necessary to begin with opening of cysts. Then a tumor, whenever possible fully, delete intrakapsulyarno. After that the capsule of a tumor is very carefully separated from a bottom of the third ventricle. In some cases during the use of the microsurgical equipment it is possible to find and keep a leg of a hypophysis.
If the tumor extends in the Turkish saddle, then originally the capsule of a cyst together with the diaphragm of the Turkish saddle stretched on it cut, a little otstupya from edge of a saddle. Then the dissector separate the capsule K. also delete from walls of the Turkish saddle.
Tissue of a hypophysis should be kept. Thus, in most cases it is possible to remove considerably To. even at its big sizes if it is not implemented into a cavity of the third ventricle.
The most difficult task is represented by removal To., located in the third ventricle. Removal them is especially dangerous because of an opportunity to damage walls of the third ventricle and structures adjoining to it, with to-rymi a tumor often has no clear boundary. However and in these cases an attempt at least of a partial oncotomy or emptying intraventrikulyarno of the located cysts can be made.
It is possible to approach such tumors by a section on the centerline of the stretched lobby of a wall of the third ventricle (a final plate) or through an expanded interventricular foramen after opening of a front horn of a side ventricle.
At the serious condition of patients caused by occlusal hydrocephaly and at impossibility to eliminate impassability of ways of circulation of cerebrospinal liquid during an operative measure originally it is necessary to resort to palliative operation (e.g., to a ventrikulotsisternostomiya). After recovery of circulation of cerebrospinal liquid the oncotomy or emptying of cysts can be carried out.
At very big cysts and serious condition of patients it is possible to resort to repeated punctures of a cyst with emptying of contents and washing of her cavity.
Due to the sensitivity To. to beam influence surgical treatment can be combined with radiation therapy (tele-irradiation or direct introduction to a cavity of a cyst or fabric of a tumor of radioisotopes of phosphorus, gold, yttrium, etc.).
At operation damage of brain structures, a bottom of the third ventricle, large vessels of the basis of a brain, adjacent to a tumor, which are quite often included in a tumor is possible. Use during operation of the microsurgical equipment allows to reduce danger of similar complications considerably. The phenomena of aseptic meningitis can be observed.
At dislocation of a brain as a result of emptying of big cysts the hyperthermia, symptoms of disturbance of consciousness, subcrustal symptoms, endocrine disturbances can be observed. A frequent complication is not diabetes mellitus. Changes of mineral and carbohydrate metabolism are possible.
For the purpose of prevention and treatment of postoperative complications in the postoperative period use drugs of bark of adrenal glands (a hydrocortisone, Prednisolonum, etc.). At the phenomena of not diabetes mellitus Adiurecrinum is shown, at a hyperthermia — antipyretics (pyramidon, analginum) and methods physical. coolings. Usually appoint the drugs reducing wet brain (lasixum, Diacarbum, Mannitolum, etc.).
For more bystry sanitation of cerebrospinal liquid and reduction of intracranial hypertensia carry out repeated lumbar and ventrikulyarny punctures.
the Forecast at a timely oncotomy and lack of a malignancy favorable, return to work is possible.
Bibliography: Arendt A. A. Klinika and diagnosis of cranyopharyngiomas, Vopr, neyrokhir., t. 21, Kya 5, page 18, 1957; it, Surgical treatment of cranyopharyngiomas, in the same place, t. 22, No. 5, page 11, 1958; it, Analysis of surgical treatment of cranyopharyngiomas, in the same place, t. 23, No. 1, page 17, 1959; Babchin I. S. About surgical treatment of cranyopharyngiomas, in the same place, t. 16, No. 5, page 14, 1952; V. V. Struktur's Sins of a parenchyma of cranyopharyngiomas (Erdgeym's tumors), in the same place, t. 23, No. 5, page 1, 1959, bibliogr.; Tumors of a brain, under the editorship of A. I. Arutyunov, page 114, M., 1975, bibliogr.; N. A. priests. Tumors of a hypophysis and pituitary area, page 106, L., 1956; Bartlett J. R. Craniopharyngiomas, Brain, v. 94, p. 725, 1971; Clinical microneurosurgery, ed. by W. Th. Koos a. o., Stuttgart, 1976, bibliogr.; K o o s W. T h. a. M i 1 1 e r M. H. Intracranial tumors of infants and children, p. 188, Stuttgart, 1971; M a t s o n D. D. a. With r i g 1 e r J. F. Management of craniopharyngioma in childhood, J. Neurosurg., v. 30, p. 377, 1969; R a n d R. W. Micro-neurosurgery, St Louis, 1969; Wertheimer P. et Corradi M. Les craniopha-ryngeomes aprfcs 40 ans, Neuro-chirurgie, t. 3, p. 3, 1957, bibliogr.; Ziilch K. J. Kraniopharyngeome, Handb, d. Neurochir., hrsg. v. H. Olivecrona u. W. Tonnis, Bd 3 S. 504, B. u. a., 1956.
A. A. Arendt, A. H. Konovalov.