From Big Medical Encyclopedia

PARAGANGLIOMA (paraganglioma; Greek para near + ganglion a nerve knot + - oma; synonym hemodektoma) — tumor, coming from cells paragangliyev. According to the WHO classification of a paraganglioma are included in group apudy, the soft tissues relating to tumors.

Paragangliya (see) consist of chromaffin and not chromaffin cells. Carry marrow of adrenal glands, Tsukker-kandl's body, sympathetic nodes to the first. From them there is chromaffin P., or pheochromocytoma (see). Not chromaffin a paraganglion (yugulyarny, glomus vagum, orbital, pulmonary, marrowy, a paraganglion of covers of a brain, and also the trunks which are located on the course of vessels of soft tissues, and especially extremities) are formed by the cells relating to hemoretseptorny, however, capable, as well as chromaffin to emit biologically active agents like monoamines, including a catecholamine and serotonin that gives the grounds to carry a paraganglion to APUD system; the term is constructed of the first letters of the English name making this system of the cells having ability to allocation of predecessors of amines with their decarboxylation and biogenic amines (see. APUD-system ). In not chromaffin paragangliya, according to A. Albertini, in a small amount it is possible to find also chromaffin cells. The term «paraganglioma» is applied by hl. obr. in relation to tumors from not chromaffin paragangliyev. These tumors are called also granular cell, alveolar tumor, carcinoid, a receptoma, adenoma (in a carotid little body), a carotid hemodektomy, not chromaffin paraganglioma. The item meets seldom, more often at the age of 40 — 50 years. Localization is various that is connected with a wide spread occurance in an organism not chromaffin paragangliyev.

Pathological anatomy

the Sizes of a tumor vary from 0,5 to 5 cm and more in the diameter. The consistence and color of nodes are various and hl depend. obr. from the number of vessels and a stroma; they more often soft, friable, are more rare dense, have color from white to bloody-brown. In cavities, napr, in drum (at P. of a middle ear), they can have the form of the polyp which is quite often bleeding. Borders of nodes not always accurate that is connected with ability of a tumor to burgeon in surrounding fabrics.

The microscopic structure of P. of any localization is same. Cells of its rounded or polygonal shape, remind the cosecreting cells paragangliyev, light or dark, large, with roundish kernels and a wide rim of cytoplasm. Light cells often have signs of secretory activity in, a type of vacuolation of cytoplasm and existence in it of the osmiofilny granules revealed at electronic microscopic examination. It is supposed that distinction in morphology of cells of a tumor is connected with dynamics of their secretory cycle. The group of cells of P. reminds group of cells in paragangliya. Tumor cells form balls, to-rye can press in a gleam of vessels. The stroma of a tumor is developed unevenly. It is presented by argyrophil and collagenic fibers and vessels of preferential sinusoidny type. At electronic microscopic examination in intercellular intervals the main substance presented by fine-grained and tonkofibrillyarny material with formation of homogeneous osmiofilny masses by places comes to light.

Mikropreparata of various morphological options of a paraganglioma: and — alveolar (alveolar and trabecular) option — polygonal, preferential light cells form alveoluses (1), closely adjacent to vessels of sinusoidny type (2); — trabecular option — layers of cells in the form of the trabeculas (1) divided by narrow layers of a stroma with numerous vessels of sinusoidny type (2) prevail; in — the angiomatous option — is noted abundance of wide thin-walled vessels (1) between which in well developed stroma groups of tumor cells are visible (2); — sblidny option — accumulations of the tumor cells (1) divided by not numerous wide vessels of sinusoidny type (2) are visible.

Depending on group of cells, quantities of a stroma and E. V. Uranov's vessels recommends to allocate the following morfol, options P.: alveolar (alveolar and trabecular), trabecular, angiomatous and solid. These options are often combined in the same tumor, but usually one of them is prevailing. The alveolar (alveolar and trabecular) option is presented by the alveolyarnotrabekulyarny structures (fig., a) divided by gentle connective tissue layers with various, often plentiful maintenance of vessels. The group of cells reminds group of cells in hemadens — they closely prilezhat to sinusoids also are included places of a vascular vystilka that is confirmed at electronic microscopic examination. The trabecular option is characterized by dominance tyazhy (trabeculas) from 2 — 3 rows of the cells of a polygonal or prismatic form reminding epithelial (fig., b). The trabeculas divided by friable connective tissue layers widely anastomose among themselves. In the thickness of cellular tyazhy small cavities meet homogeneous contents that is regarded as a sign of secretion. Trabeculas prilezhat to vessels, often forming figures of so-called sockets. The angiomatous option is characterized by abundance of vessels of capillary and sinusoidny type, between to-rymi in well developed stroma groups of cells, typical for P. are located (fig., c). Sometimes there is a lot of vessels that there are structures reminding a cavernous or capillary angioma. The solid option is allocated because in its structure layers of cells with a scanty stroma between them prevail (fig., d).

The malignant option P. meets extremely seldom. The tumor is characterized by the expressed infiltriruyushchy growth. Grows slowly, forming dense, slow-moving, indistinctly konturirovanny node; it is microscopically characterized by atypia and polymorphism of cells, a sharp hyperchromia of kernels. Stroma of a tumor scanty; quite often vascular component so characteristic of high-quality P. is expressed poorly. Malignant P.'s metastasises appear rather late, in the beginning in regional limf, nodes, then quickly there comes hematogenous innidiation. Malignant the wedge, P.'s current, according to nek-ry researchers, is not always combined with corresponding change of structure of a tumor.

The clinical picture

the Clinical picture depends on degree of hormonal activity of P., its localization and ability to infiltriruyushchy growth. Due to the hormonal activity of a tumor emergence of symptoms of a hypercorticoidism is possible (see. Adrenal glands ), a carcinoid syndrome (see. Carcinoid ) and arterial hypertension (see. arterial hypertension ). At one of frequent localizations of P., in a middle ear symptoms of defeat of an acoustic organ are noted if the tumor proceeds from a drum glome, at localization in glomus jugulare the IX—XII function of pairs of cranial nerves is broken, and at germination of a tumor in a middle ear symptoms of its defeat appear; further in these cases destruction of bones of a skull, with development of symptoms of a prelum of a brainstem is possible. At P.'s localization in an orbit there is a shift and a prelum of an eyeglobe.

The diagnosis

the Diagnosis is established by hl. obr. on the basis of data morfol, researches. The differential diagnosis is difficult. E.g., P., located on a side surface of a neck, it is necessary to differentiate with metastasises cancer (see), branchyogenic tumors (see), etc. Morfol, the differential diagnosis is carried out with angiogenic tumors and cancer; an important diagnostic character is establishment of organoid structure of a tumor. Characteristic light cells of P. and its stroma well come to light during the coloring by azocarmine. At impregnation, napr, according to Masson — Fontana and across Grimelius, in cytoplasm of cells of P. it is possible to find the monoamines reducing silver, and also lipofuscin, melanin, lipoida.

Treatment and Forecast

Treatment operational.

High-quality P.'s forecast favorable. After operation the recurrence connected with impossibility of radical removal of P., coming from drum, orbital or yugulyarny paragangliyev is observed. The forecast for malignant P.'s life adverse.

See also Glomal tumors .

Bibliography: Vikhert A. M., and l and flor ly G. A. and Poroshin K. K. Tumors of soft tissues, Microscopic diagnosis, M., 1969; Golovin D. I. Atlas of tumors of the person, page 62, L., 1975; Lebkova N. P., Ur E. V. and Vayshen-k of e r P. G. Ultrastruktura of glomal tumors of a middle ear, Arkh is new. patol., t. 36, No. 9, page 42, 1974; Masson P. Tumors of the person, the lane with fr., page 438, M., 1965; The Guide to pathoanatomical diagnosis of tumors of the person, under the editorship of N. A. Krayevsky and A. V. Smol-yannikova, page 340, M., 1976; Le Compte P. M. Tumors of the carotid body and related structures, sect. 4, Washington, 1951.

A. V. Smolyannikov.