Hypernephroma

HYPERNEPHROMA, represents a tumor of the adrenal gland, specifically from its cortical cells. Distinguish between benign and malignant H.; the latter are also known under the name of Grawitz tumors. Anatomically, the first type of H. is more common, clinically however it usually manifests itself in no way, in contrast to the second type, which, although less common, has great practical significance, and by the very term H. the malignant form is meant. Benign H. has the appearance of a rounded, sometimes clearly encapsulated node, ranging in size from a pinhead to a cherry, ochre-yellow in color due to its richness in lipoids; histologically, the tumor closely resembles the typical cortex of the adrenal gland. This form is usually observed in the adrenal gland itself, where it is also called an adenoma, a struma (adenoma, resp. struma suprarenalis), or one speaks of nodular hyperplasia of the cortex; occasionally similar formations are found in the kidney cortex, usually just under the capsule (struma suprarenalis renis, struma lipomatodes aberrata renis). Some authors classify them as hamartomas, although they are more often observed at a later age. Malignant H. also vary in size (from a small hazelnut to the head of an adult), rounded, sometimes irregular in shape, bumpy, of firm or uneven consistency, sometimes cystic in places. On section, these H. are variegated (see separate table, fig. 2) due to the alternation of areas of the main tumor masses rich in lipoids (yellow color) with areas of light gray, white color (devoid of lipoids), as well as with dark red foci (hemorrhages into the tumor); individual parts of the tumor are dry (necroses), others are succulent, gelatinous, markedly edematous. Histological examination reveals either the typical cell columns of the adrenal cortex (see separate table, fig. 3), rich in lipoids and glycogen (so-called malignant typical H.), or the cells are arranged completely atypically in the form of glandular passages, cysts with capillary outgrowths, or in the form of solid cellular masses, most resembling a polymorphous-cell, sometimes giant-cell sarcoma (so-called malignant atypical H.); the abundance of gaping vessels (see separate table, fig. 3) completes the picture. Sometimes the tumor has such diverse structure in different places that its classification into one or another tissue encounters insurmountable obstacles—hence the variety of terms by which these tumors have long been designated (adenosarcoma, carcinoma, sarcomatoid malignant adenoma-epithelioma, etc.); avoiding precise designation of histogenesis, Birch-Hirschfeld proposed the term H. Malignant H. are predominantly found outside the adrenal glands (heterotopic H.), and specifically in the kidneys; occasionally they have been found in the liver, broad ligaments, testicles, ovaries. The frequent development of H. in the kidneys, as well as certain features of their structure, bringing them closer to kidney adenomas and carcinomas, have long caused doubt in the correctness of interpreting kidney (malignant) H. as tumors from wandering parts of the adrenal cortex, inclining many authors to the idea of a nephrogenic (from kidney epithelium) origin of these tumors and designating them by the general terms hypernephroid tumors, or Grawitz tumors. Some authors occupy an intermediate position, pointing to the possibility of the origin of malignant kidney tumors of the H. and cancer type from an early and common anlage of the kidneys and adrenal glands, with, in their opinion, development in some cases proceeding in the direction of H., in others—kidney cancer. The malignancy of kidney H. (not always obvious histologically, especially in its typical form) is expressed mainly in the tendency to early invasion of blood vessels (see separate table, fig. 4), especially veins, which often causes early and abundant metastasis; the favorite organs for metastases are the lungs and bones, especially the skull, lumbar vertebrae; via the lymphatic system the tumor also spreads (involvement of retroperitoneal and mediastinal glands). Sometimes the tumor itself proceeds latently, and the patient dies from various complications associated with the mentioned metastases. H. of the kidneys do not have any special general effect on the body; only in individual cases are disturbances of carbohydrate (glycosuria), protein metabolism (amyloidosis of organs) observed. Greater attention in this regard is deserved by those H., whose origin from the adrenal cortex is beyond doubt. These tumors (including benign forms) sometimes have a specific effect on growth and sexual development processes, causing (more often in female individuals) pictures of pseudohermaphroditism, virilism, hirsutism, macrogenitosomia (in boys), pubertas praecox (see). The very fact of the absence of these complications in kidney H. is correctly evaluated by some authors as an argument against the adrenal origin of kidney hypernephromas. According to data from Moscow morgues for 1923-27, mortality from hypernephroma is 32:10,000 ADULTS (OVER 16 YEARS).

I. Davydovsky.

Hypernephroma of the kidney is observed mostly in the age range of 50-60 years; before the age of 20 and after 70 it occurs very rarely. According to the statistics of most authors, the age from 10 to 20 years remains as if immune to this disease. Hypernephroma is much more common in men than in women. According to Israel, ⁴ Hypernephroma occurs in males and only in ¹/₅ of females. According to Fedorov, 67.3% occurs in men and 32.7% in women. The right kidney is affected more often than the left. Bilateral kidney disease with hypernephroma has also been described. The classic cardinal symptoms of hypernephroma are hematuria, kidney tumor, and pain. But sometimes hypernephroma proceeds hidden for a long time without giving symptoms, and finally manifests clinically with metastases, or when it has already become inoperable. According to the opinion of most authors, hematuria in hypernephroma occurs in 70-80%. It occurs either due to the destruction of vessel walls by the tumor or due to blood stagnation caused by the tumor. Hematuria is usually painless and sometimes may be accompanied by increases in temperature and vomiting. In other cases, pain in the kidney area and along the ureter appears with hematuria. This pain takes on the character of colic due to the passage of long worm-like blood clots through the ureter. Only in rare cases is hematuria a single occurrence during the entire disease; usually it occurs periodically, at various, sometimes very long, intervals, and may last for hours, weeks, and even a month. The amount of blood in the urine varies. Sometimes its presence is determined only microscopically. In other cases, profuse bleeding occurs, which depends mostly on the tumor growing into the renal pelvis. In cases of severe, threatening hematuria, when cystoscopy cannot be performed due to blood, it is necessary to make a high incision of the bladder to establish the source of bleeding. Usually hematuria occurs when a kidney tumor can already be palpated. In rare cases, hematuria is an early symptom of hypernephroma, appearing 6-8 and even 10 years before the clinical recognition of hypernephroma in the form of a clearly palpable tumor. - As for other changes in the urine, protein is found in hypernephroma in 60-80%, and pus in 35-50%. In rare cases, pieces were found in the urine which, upon microscopic examination, consisted of groups of cells characteristic of hypernephroma. The second cardinal symptom of hypernephroma is the kidney tumor. It can be palpated in 70-80%. It is recommended to palpate as carefully as possible so as not to cause hematuria by rough manipulations. The kidney affected by hypernephroma in most cases remains relatively mobile for a long time, differing in this from other malignant kidney tumors, which as a rule very early become fused with surrounding tissues. The possibility of palpating the tumor depends not only on the size of the tumor itself, but also on its location; therefore, if the tumor grows from the upper pole of the kidney, then even of medium size (for example, like a mandarin), hypernephroma sometimes cannot be palpated. Hypernephroma more often develops in the lower pole of the kidney. Regional lymph nodes in hypernephroma are affected by metastases from the tumor already in the late stages of the disease, when the tumor has reached large sizes. But metastases through blood vessels are often found in the initial stages of hypernephroma growth, when the kidney tumor cannot yet be palpated. Pain in hypernephroma, in terms of its character and location, is not specific and occurs on average in 40-50%. Usually it appears in a later stage of tumor growth than in other malignant kidney tumors. Pain can last for many years, be accompanied by periodic hematuria, while the general condition of patients suffers little. Pain can be constant or intermittent, localized in the lumbar region with irradiation to the sacrum, thigh, groin area, and bladder area. Colicky pain is usually observed with hematuria (see above). - Besides these main symptoms, a number of secondary, so-called accompanying symptoms are found in hypernephroma. Varicocele, i.e., dilation of the veins of the spermatic cord, occurs relatively rarely and depends on compression by the tumor or lymph nodes of the kidney veins or spermatic cord veins. This dilation of veins is painless and does not decrease when lying down or when lifting the scrotum upward. Often an increase in temperature is observed, sometimes even in the initial stages of the disease, which is the only symptom of a secretly developing hypernephroma. The cause of the temperature increase is not exactly known. Some authors explain it by the absorption of tissue decay products caused by the growing kidney tumor. In addition, frequent nocturnal urination, shortness of breath, and gastrointestinal phenomena in the form of vomiting, nausea, heartburn, etc. have been observed as accompanying symptoms. Sometimes pigmentation of the skin on the hands and back in the form of small brown spots occurs. The general condition of patients often remains quite satisfactory for a long time, despite even deep changes in the kidney. The onset of cachexia depends either on very large blood losses or on metastases to lymph nodes and internal organs. As for the clinical course of hypernephroma, it is extremely diverse, and therefore it is not always easy to make the correct prognosis. Fedorov distinguishes three periods in the development of hypernephroma. - 'In the first period, these are tumors of negligible size, embedded in the cortical layer of the kidney and absolutely not manifesting themselves with any symptoms. This is the hidden period, which often does not manifest itself throughout a person's entire life. In the second period, kidney bleeding already mostly occurs, rarely pain, and usually uneven enlargement of the kidney is noted. This period of relatively benign growth of the new formation sometimes lasts for many years. Finally, in the third period - of malignant growth - the tumor rapidly increases, causing more or less constant pain and affecting the general condition of patients. Mostly in this period, metastases are also observed.' - The diagnosis of hypernephroma in the presence of three main symptoms - hematuria, a palpable kidney tumor, and pain - does not present particular difficulties, if one also remembers that according to the opinion of most authors, hypernephroma constitutes on average 65-75% of all clinically observed kidney tumors. The diagnosis of hypernephroma in its first period of development is extremely difficult with the current state of research methods, when the tumor is very small and does not manifest itself with any symptoms. Recently, for the diagnosis of kidney tumors in general and for the diagnosis of hypernephroma in particular, radiography in the form of pyelography alone or pneumorenography, or in the form of a combination of both methods, has been used. In pneumorenography, changes in the contours of the kidney and asymmetry in its upper and lower halves sometimes allow to determine exactly in which part of the kidney the tumor is located. In pyelography, a partial filling defect of one or two calyces, the absence of a shadow of the renal pelvis on the X-ray, or, as the Americans say, a shadow of the renal pelvis resembling spider legs, are characteristic in general for kidney tumors, and in particular for hypernephroma. In addition, radiography can recognize hypernephroma metastases in bones or lungs. Pyelography and pneumorenography acquire special value in those cases when hypernephroma has not yet manifested clinically with a palpable kidney tumor or hematuria, or at least one of these symptoms is absent. Such cases of hidden forms of hypernephroma are found in the literature in almost 20% and can sometimes be determined only with the help of radiography. It is necessary to remember that sometimes radiodiagnosis can lead to erroneous conclusions. This happens in cases where hypernephroma is accompanied by a kidney anomaly or a stone in it, or when hypernephroma is combined with pyelitis, pyonephrosis, tuberculosis, or syphilis of the kidney. The long, sometimes lasting for many years, existence of a kidney tumor without signs of cachexia is of importance for the diagnosis of hypernephroma, which is not observed in other malignant kidney tumors. Atypical cases of hypernephroma present great interest and great difficulty for differential diagnosis. In the literature, cases of hypernephroma have been described that clinically resembled bone tuberculosis, carcinoma of the vagina with metastases to the skin, brain tumor, tuberculosis of the peritoneum, joint rheumatism, sarcoma of the spine, tumor of the clavicle, stenosis of the pylorus of the stomach, and finally ileus. In differential diagnosis, it is very important to know that hypernephroma has the property of sometimes giving only one metastasis, with the picture of the disease being determined by this single metastasis, while the primary kidney tumor remains unrecognized. At present, the so-called specific reactions, for example, the pupil reaction, which should indicate the presence of adrenaline in the blood, can hardly be attributed any particular value for the diagnosis of hypernephroma.'

This reaction can no longer be considered as proof for H., firstly because now very few authors believe that H. originates from the adrenal gland (see above), and secondly because dilation of the pupil due to irritation of the sympathetic nerve, which ends in the m. dilatator pupillae, can also be explained by the circulation in the blood of proteinogenic amines, which occur in it in many malignant tumors, especially if they are disintegrating. (For the influence of some H. on growth and sexual development processes, see above.) The treatment of H., as with other kidney tumors, must be exclusively surgical and must consist of nephrectomy, if the other kidney exists and is functionally capable. However, it must be remembered that even with surgery in the early stages of H., the results of nephrectomy may be unreliable, because H. can give very early metastases through the circulatory system to the most distant organs. True, these metastases are often single in number, and if accessible for surgical removal, the patient can count on radical cure of the disease; nevertheless, there are patients with multiple metastases that cannot be removed. Regarding indications for surgery, there are currently no disagreements among surgeons. It is necessary to operate on all cases of H., if the patient does not have significant general weakness, does not have cachexia and multiple metastases, especially in internal organs (see Autonephrolysin). The presence in the urine of the healthy kidney even up to 10%_o protein and cylinders and some decrease in its functional ability still do not serve as an absolute contraindication to nephrectomy, because all these phenomena in most cases disappear after the operation. Much greater importance is attached to the state of cardiac activity, because anesthesia, shock and significant blood loss in removing a large H. can have a strongly depressing effect on cardiac activity and the heart muscle is sometimes unable to maintain the necessary blood pressure for urine secretion. Most authors consider surgical intervention in H. contraindicated if the tumor is immobile or if the tumor has grown through the renal capsule, because in these cases the regional lymph glands are already affected. It is impossible to save the patient under such conditions, even if all affected glands and tissues around the kidney are carefully removed. H. with a single metastasis without cachexia must be operated on, first performing nephrectomy and then removing the metastasis. After such operations, patients lived for many years without recurrence and remained completely healthy. Severe functional insufficiency of the other kidney and diabetes should be considered as contraindications to surgical intervention. In such inoperable cases, some relief is sometimes provided by the application of X-rays. In addition, X-ray therapy is used as additional treatment after surgery. Some authors claim that the long-term results after such combined treatment are much better than after only surgical intervention. For nephrectomy in H., six methods have been proposed. 1. Laparotomy, in which the incision is made along the outer edge of the rectus abdominis muscle, and in large tumors - along the midline from the xiphoid process of the sternum to the pubis. Sometimes even through such an incision it is not possible to reach the outer surface of the tumor, therefore a transverse incision is added to the longitudinal incision. This method gives a clear picture of the adhesions of the affected H. kidney with the diaphragm, liver, intestines and other internal organs. This is actually the advantage of this incision. Subsequently, the operation does not proceed more easily than with the extraperitoneal lumbar incision. As for the disadvantages of laparotomy for removal of H., firstly, with laparotomy it is often necessary to widely open the posterior wall of the peritoneum and carefully prepare the large intestine, with the risk of injuring its vessels, and secondly, when the tumor almost completely covers the hilus and the vessels of the renal hilus lie in infiltrated and vessel-rich tissue, the pedicle of the tumor is not always possible to isolate and ligate. In addition, with laparotomy the peritoneum is strongly cooled, the intestines are traumatized, there is a danger of peritonitis, and it is inconvenient to drain the wound. 2. The extraperitoneal method (proposed by Gregoire): with the patient lying on the healthy side, the incision begins medially from the spina ant. sup., curves around the iliac crest to the mid-axillary line, then rises upward to the edge of the ribs, where it curves medially and runs along the rib edge to the end of the XII rib. With this method, the kidney is removed together with the fat capsule, adrenal gland and lymph glands. This method is now almost never used, as it gave a colossal mortality rate. Moreover, radical surgery is considered by many surgeons to be futile if the lumbar lymph glands near the aorta are already affected, as they anastomose with the lymph glands of the posterior mediastinum, on which Fedorov first pointed out. 3. High transverse incision with division of the XII and even X ribs, at which all nerves originating from the XII intercostal nerve are also divided. The advantage of this incision is that it provides free access to the dome of the diaphragm. This facilitates nephrectomy for those kidney tumors that grow upward. 4. The anterior oblique incision, beginning laterally as a classic oblique incision, goes downward and medially and ends at the mid-axillary line. The direction of this incision is parallel to the fibers of the external oblique muscle. This incision does not always provide sufficient access to the kidney tumor. 5. The Cabbot incision begins from the upper edge of the m. erectoris trunci, proceeds to the lower edge of the XII rib and descends downward to the midline of the abdomen in the umbilical region. This incision can be extended by an additional perpendicular incision along the midline of the abdomen. This incision differs little from the next incision proposed by Fedorov. 6. The lumbo-abdominal or extraperitoneal incision begins from the lumbar side, at the level of the XII rib or immediately above it, and runs obliquely downward, then on the anterior surface of the abdomen it turns into a transverse one. Before the operation, it is necessary to determine as accurately as possible where difficulties may arise in removing the tumor: in the area of the ureter and at the lower pole of the kidney or at the renal hilus and upper pole. Accordingly, it is necessary to change the direction of the abdominal end of the oblique lumbar incision, i.e., in the first case - downward toward the spina ant. super. and sometimes lower to the groin, or in the second case - transversely to the navel and even somewhat higher. If necessary, this incision can be converted into a peritoneal one and its anterior end extended parallel to the rib edge to the linea alba. Further, the operation can be performed in two ways: first, the kidney can be removed extracapsularly, i.e., excised together with the fibrous capsule, and secondly, intracapsularly, i.e., enucleate the kidney from the fibrous capsule. Extracapsular nephrectomy in H. is used in cases where the layers of the fat capsule are not very developed and there are no dense adhesions between the fibrous and fat capsules. Fedorov recommends using intracapsular nephrectomy in cases when the kidney is large and immobile and when it is not possible to quickly go deep between the fat and fibrous capsules, because abundant and even life-threatening bleeding may occur before it is possible to approach the renal hilus and clamp its vessels with clamps. The difference of Fedorov's intracapsular nephrectomy method from others consists in a special technique for isolating the pedicle of the tumor (hilus renis). The technique consists in that after the kidney is extracted from the fibrous capsule, the operator deviates and pulls the kidney first downward and outward, pressing with fingers on the anterior surface of the kidney, while the assistant retracts medially the upper-anterior edge of the abdominal part of the incision with a wide renal retractor introduced between the kidney and the detached fibrous capsule. This provides access to the hilus in depth. Then a semicircular incision is made with a scalpel in the area of the hilus, penetrating only through the fibrous capsule and making it possible to penetrate into the tissue around the renal pelvis and ureter. By inserting a finger into this incision, the hilus and the anterior surface of the renal pelvis and ureter are very carefully isolated by blunt dissection. Then the operator elevates and pulls the kidney medially and upward, while the assistant with a wide renal retractor pulls the lower-posterior edge of the lumbar incision outward so as to make the posterior surface of the renal hilus accessible to the operator. Here an incision is made in the capsule around the hilus, and thus the latter is then surrounded on all sides by a circular incision through the fibrous capsule. From this incision, an attempt is made to completely free the hilus from the fatty tissue with a finger and make the kidney more mobile. After isolating the renal pelvis and hilus, the operator applies clamps to the vessels and ureter outside the fibrous capsule, cuts off the kidney outward from the clamp, and ligates the vessels and ureter separately.

It must be remembered that when applying a clamp to the hilus of the right kidney, it should not be applied close to the spine, so as not to compress the vertical part of the duodenum and not cause necrosis of the intestinal wall with the subsequent formation of a duodenal fistula, from which patients usually die. - Postoperative mortality with various methods of operation, according to the statistics of Ploschner, is as follows: 1) transperitoneal nephrectomy - 37.5%, 2) retroperitoneal nephrectomy - 13.5%, 3) retroptransperitoneal nephrectomy - 33%. As for the results of surgical treatment of H., in the latest literature we have an average postoperative mortality of about 13%; 70-80% die from recurrences within three or more years after the operation; 7% to 17% remain healthy for a long time. In the absence of a recurrence within 4-5 years, the patient can be considered radically cured of H., although it should be remembered that recurrences have been observed after even longer periods of time. The long-term results of nephrectomy for H. are worsened, firstly, by non-radically operated cases, when the impossibility of radical removal of the tumor is established only during the operation itself; secondly, by the often observed early recurrences, or rather metastases, especially in the brain, lungs, and bones, the origin of which can be explained by tumor emboli that occur during the operation when the kidney is being separated from the surrounding tissues.

D. Kuvnetsky.

in the bones (see figure), also often in the bones of the skull. Along with the inflammatory process, venous hyperemia can play a certain role in the development of hyperostoses of the distal ends of bones, which is observed at the ends of the fingers of the hand (drumstick fingers) in cardiac and pulmonary diseases. Here should also be mentioned those hyperostoses of the bones that are observed in connection with lesions of the appendages of the brain in acromegaly, as well as in partial and general gigantism and finally in a rare form of disease of the bones of the skull - leontiasis ossea (see).