INSULOMA

From Big Medical Encyclopedia

INSULOMA (insuloma; lat. insula the island + - oma; synonym nesidioblastoma) — hormonal and active tumor of pancreatic islands (islets of Langerhans). It was for the first time found in 1902 by A. Nicholls at autopsy. In 1904 L. V. Sobolev described a hypertrophy and a hyperplasia of insular cells, having designated this phenomenon as «a struma of islets of Langerhans». Prizhiznenno I. is for the first time diagnosed in 1927 by R. Wilder and sotr. First successful operation of removal And. it is executed by E. Graham in 1929. In the USSR the first investigated a wedge, manifestations And. also V. R. Klyachko, O. V. Nikolaev, A. D. Ochkin offered her surgical treatment.

Statistics

according to Palyumbo and Greco (L. Palumbo, L. Greco, 1953), hormonal and active tumors of pancreatic islands meet very seldom. V. V. Vinogradov (1959), being based on results of a research of 21 056 autopsies, established that one And. meets on 3509 autopsies. According to S. G. Genes (1973), one And. find on each 900 openings.

Among patients whose insulyarny device allocates a large number insulin (see), at 70% adenoma, and is found in 30% — an adenocarcinoma. According to different authors, malignant And. are observed in 10 — 60% of cases. O. V. Nikolaev and E. G. Veynberg (1968) established that women are ill And. almost twice more often than men. And. meets generally at the age of 35 — 55 years; at children — it is exclusively rare.

The etiology

the Aetiology is not clear. By origin And. divide on alpha and cellular, beta and cellular and delta and cellular. Alpha and cellular tumors meet very seldom (see. Glucagonoma ); they cosecrete excess quantity glucagon (see). The tumor, coming from delta cells, cosecretes gastrinopodobny substances; it is clinically shown by Zollinger's syndrome — Ellisona, described in 1955 (see. Zollingera — Ellisona a syndrome ).

In modern literature the insulinoma is most studied; it develops from basphilic insulotsit (beta cells) of pancreatic islands and produces excess amount of insulin.

Pathological anatomy

Fig. 1. Remote insuloma (diameter of 2 cm).

To differentiate And. a different origin on a basis only morfol, the picture is impossible. The tumor is more often localized in a tail or a body of a pancreas, occasionally out of it, proceeding in such cases from a malrelated tissue of a pancreas (see. Additional bodies ). Macroscopically And. represents the dense node from 0,5 to 7,0 cm in the diameter which is accurately delimited by the capsule (fig. 1); color of a tumor varies from whitish-gray to brown. In most cases the single tumor meets; several primary tumors are found in 12 — 14% of patients. In certain cases And. has infiltriruyushchy growth and gives metastasises. On a consistence And. much more densely than surrounding fabric.

At microscopic examination the tumor consists of cells of a polygonal, round or ovoidny form. The amount of cytoplasm variously, its coloring can be basphilic or acidophilic. Kernels of usually rounded or oval shape with the thin sprayed chromatin, the compact arrangement of chromatin is less often noted. In smaller cells of a kernel of a round form are located it is central, in cubic and prismatic they are more often than an oval form, are located at one of poles of a cell. In cytoplasm of other pole granules of a secret accumulate. Multinucleate cells — an exception. Quantity variously — from single to multiple; atypical mitoses are rare even in obviously malignant tumor. One of the main features of a tumor, coming from basphilic insulotsit — insulinoma, degranulation of beta cells, loss of specific granularity by them is. The quantity of the degranulated cells reflects degree of hormonal activity of these tumors.

Fig. 2. Microscopic picture of an insuloma of alveolar type: 1 — the alveoluses created from tumor cells; 2 — the connecting fabric surrounding alveoluses; coloring hematoxylin-zozinom; X 100.

Irrespective of an origin And. subdivide on trabecular and alveolar types of a structure. The trabecular structure meets more often, for to-rogo trabeculation, surrounded with vessels is characteristic; in walls of vessels considerable growth of fibrous fabric, accumulation of hyaline masses and amyloid is quite often noted. Alveolar structure And. it is characterized by formation from tumor cells of the alveoluses surrounded with connecting fabric (fig. 2) and blood vessels.

Typical for And. formation of so-called sockets is. One sockets consist of the central vessel, around to-rogo are located a cylindrical or cubic form of a cell with apically with the located kernels; a part of their cytoplasm (about a vessel) clearly a vakuolizirovana, between cytoplasm and a vascular wall the granular masses which, according to E. V. Uranova (1971), are a secret of tumor cells are quite often noted. A number of authors considers that dominance of such sockets is characteristic for. tumors, coming from alpha cells. Sockets of other look are formed of the same cells, but without the central vessel, with basally the located kernels; in apical department of cytoplasm the secret accumulates, at allocation to-rogo forms is central the located cavity. It is considered that dominance of such sockets is characteristic of tumors from beta cells. However in And. existence of both structures is possible.

The stroma of a tumor can be formed by both friable, and fibrous connecting fabric. Depending on expressiveness of a stroma And. can be the parenchymatous, fibrous and mixed type. Occasionally in the center I. necroses, sometimes are formed in And. it is possible to find the huge islets of Langerhans surrounded with the hyalinized fibrous fabric.

And., as a rule, grow accurately surrounded with the capsule and to carry out a differentiation between malignant and d about r about to and the p e with t to e and and y mi options happens very difficult, it is often impossible. Only existence of the remote metastasises (in a liver, lungs, limf, nodes) allows to speak with reliability about a zlokachestvennost of a tumor. At the same time the insulinoma having gistol signs of a zlokachestvennost, can not have metastasises. According to Priestley (J. Priestley, 1962), the metastasizing insulinoma make 10%. The metastatic centers can hormonal be also active.

A clinical picture

Clinical manifestations at And. depend on hormonal activity of a tumor and amount of hormone which it produces.

At a glucagonoma at patients the diabetes mellitus develops (see. diabetes mellitus ).

Syndrome 3ollingera — Ellisona it is clinically characterized by formation of recurrent round ulcers went. - kish. path, hypersecretion of a gastric juice, hyperacidity, dyspepsia. The spontaneous hypoglycemia, as a rule, does not arise; disturbances of secretion of insulin and a glucagon are not noted (see. Zollingera — Ellisona a syndrome ).

Insulinoma is characterized by a syndrome hyper dysinsulinism (see), the caused very large amount of insulin (1 g of a tumor contains up to 80 PIECES of insulin, normal fabric of gland — 2 PIECES). The hyper dysinsulinism leads to heavy attacks of a hypoglycemia., O. V. Nikolaev and E. G. Veynberg (1968) distinguish three options a wedge, currents of an insulinoma: the galloping current, attacks of a hypoglycemia with a long interval between the first and the subsequent and option with the long period of harbingers to the first attack of a hypoglycemia. At women perhaps atypical disease when attacks are observed only during the menstrual period or at pregnancy.

Attacks of a hypoglycemia most often develop early morning hours on an empty stomach or in 3 — 4 hours after food, especially at physical. loading, mental injuries. The glycemia decreases to 50 mg of % and below, however at meal symptoms of a hypoglycemia quickly disappear. The hypoglycemic syndrome at an insulinoma develops suddenly and proceeds hard. The attack is characterized by strong weakness, the concern, excitement reaching nonsense, strong sweating, a passing hemiplegia, visual disturbances, loss of memory, drowsiness, a loss of consciousness. The attack lasts 15 — 20 min.; at the beginning of it there can be a sharp motive concern; quite often there are epileptiform attacks, sometimes patients fall into coma. Epileptiform attacks, unlike true epilepsy, can last for hours, being followed by neurovegetative symptomatology. The hypoglycemic attack is rather easily stopped by introduction to the patient of digestible carbohydrates, in more hard cases — intravenous injection of glucose.

In process of increase of attacks of a hypoglycemia and reduction of a sugar content in blood signs of damage of a cerebral cortex amplify (see. Hypoglycemia ). In the mezhpristupny period into the forefront psychological frustration act (decrease in memory, indifference to surrounding, loss of an art, impotence, asymmetry of tendon and periosteal jerks, emergence patol, reflexes, etc.). As a rule, patients have the increased appetite that leads to obesity, however in connection with dysfunction of a nervous system some patients lose flesh.

Diagnosis

Diagnosis is difficult in a wedge, and morfol, aspects. For differential diagnosis And. various origin special coloring and electronic microscopic examination of pathoanatomical drugs, comparison of the obtained data with normal structures of pancreatic islands of a pancreas is applied.

Polymorphism of symptoms often conducts to diagnostic mistakes. At a glucagonoma diagnostic value has definition of a sugar content in blood and urine, a glucagon in blood. At Zollinger's syndrome — Ellisona important is endoscopic and rentgenol, definition of ulcers went. - kish. a path in combination with a sharp hypertrophy of a mucous membrane.

At an insulinoma important diagnostic value has definition of insulin in blood during a spontaneous attack, and also test with starvation (it is better in combination with easy physical. loading). During test by the patient give only tea without sugar or water; the sugar content in blood is defined by each 2 — 3 hours. Almost at all patients insulinomy the expressed hypoglycemia develops within 12 — 16 hour. This test allows to reveal Whipple's triad, pathognomonic for an insulinoma: 1) emergence of attacks of a hypoglycemia at starvation; 2) reduction of a glycemia to 50 mg of % and below; 3) the bystry termination of an attack after administration of glucose. Apply as well some other diagnostic tests (with Tolbutamidum, a leucine, a glucagon, insulin, a cortisone, with loading glucose).

Treatment

Treatment operational. Apply an upper median or cross laparotomy with approach to gland through a gastrolic sheaf to access to a pancreas. The small sizes of a tumor, a possibility of its bedding in the thickness of gland cause need at operation of careful audit of all departments of a pancreas. Removal And. most reasonablly by enucleation, the partial pancreatectomy is less desirable. For prevention of postoperative pancreatitis which is the most terrible complication use of anti-fermental drugs is shown (Trasylolum, Contrykal, etc.).

In cases of not operability malignant And. patients are transferred to a proteinaceous and fatty diet; each 2 — 3 hours they shall receive a little food for the prevention of an attack.

The forecast

leads the Timely oncotomy to recovery; recurrence And. after operations are rather rare. At detection of the remote metastasises the forecast bad.

See also Pancreas, tumors .


Bibliography: Veynberg E. G. and Kalinin A. P. Hypoglycemia, Owls. medical, No. 10, page. From, 1969, bibliogr.; Vinogradov V. V. Tumors and cysts of a pancreas, M., 1959; H and to about l ayev O. V. and Veynberg of E. G. Insulom, M., 1968, bibliogr.; H and to about l and-e in O. V., Veynberg E. G. and M and N at-sharova R. A. Comparative assessment of tests about Tolbutamidum and a leucine at insulomas, Surgery, No. 8, page 109, 1972, bibliogr.; The guide to pathoanatomical diagnosis of tumors of the person, under the editorship of N. A. Krayevsky and A. V. Smolyannikov, page 172, M., 1976; The guide to endocrinology, under the editorship of B. V. Alyoshin, etc., page 388, M., 1973; G rei der M. H., R about sa i J. McGuigan J. E. The human pancreatic islet cells and their tumors, Cancer (Ptii-lad.), L a b h a r t A. Clinical endocrinology, p. 760, B. u. a., 1974; Marks V. Diagnosis of insulinoma, Gut, v. 12, p. 835, 1971, bibliogr.; Textbook of endocrinology, ed. by R. H. Williams, p. 649, Philadelphia a. o., 1974.

E. G. Veynberg; H. H. Petrovichev (stalemate. An.).

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