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Hemorrhagic Cysts of the Kidney. With Report of a Case
HEMORRHAGIC CYSTS OF THE KIDNEY WITH REPORT OF A CASE
A.
J. SCHOLL
Hemorrhagic cysts of the kidney are different from the ordinary, more common, thin-walled, serous cysts. Hemorrhagic cysts are smooth and continuous with the contour of the kidney surface and do not abruptly project from the renal parenchyma like serous cysts. At times the surface of the cysts may be lobulated or nodular; they are usually opaque or whitish in color; the walls are composed of several layers which may be quite thick, at times as much as 1 cm. wide; the cysts are usually markedly adherent to the surrounding organs, tearing easily on attempted removal. Externally, the renal capsule is continuous over the cyst wall and is usually readily separated from it; next there is a layer of thinned-out renal parenchyma together with a layer of fibrous tissue; the inner layer is composed of the products of old hemorrhagic masses of fibrin and adherent organized clots. The contents of the cyst are made up of partially or completely organized blood clots. The central areas may contain large masses of light brown clots, mixed with grayish white masses of fibrin. Occasionally there is a large quantity of black or brown fluid with, at times, areas suggesting fresh hemorrhage. Hemorrhagic cysts are usually solitary and large in size. Rarely are they multilocular; Judd and Simon reported a case in which three cysts were present. As a rule the cysts are fairly large in size, and occasionally they are enormous. Leopold reported a case in which the cyst had a capacity of four liters. Neff aspirated over 8 liters of chocolate colored fluid from a large hemorrhagic cyst before operation. In Lamson's case the cyst contained over 11 liters. It is not uncommon to find a hemorrhagic cyst associated with a kidney neoplasm as in the following personal case, recently observed, in which an adenocarcinoma and a large hemorrhagic cyst were found associated in the same kidney. Case Report. A man aged 58 came for examination on account of a swelling of the abdomen which had been present for 3 years. There was considerable pain in the left side of the abdomen at the time the swelling was first noticed. The pain was fairly continuous, located in the upper part of the abdomen, and 103
104
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SCHOLL
did not radiate to the back or downward. It was quite severe at the onset but gradually lessened and after 2 months disappeared. A tumor was found in the region of the left kidney by his physician who advised that nothing could be done about it. A blood examination was negative at that time. The mass or "swelling," which was painless except for the first 2 months, increased in size up to 6 months ago. Since then it has increased more rapidly in size, and there was a pulling or stabbing sensation in the abdomen. Six weeks ago the patient had chills and fever and went to bed for 10 days. Following that his side felt so ' 1full" and painful that it was difficult for him to move around. Recently his abdomen had been so distended that breathing was difficult. There was no hematuria or dysuria, but the man had to empty his bladder 3 times a night for many years. The patient had what was apparently a specific urethritis when he was a young man and somewhat later a penile lesion which was possibly a chancre. Four Wassermann tests, all recent, the last 6 months ago, were negative. Thirty years ago he passed a kidney or ureteral stone associated with pain in the left kidney. In 1928 a prostatectomy was done with only partial relief of his urinary symptoms. At this time a roentgenogram showed a mass in the left side of the abdomen. The urine contained a few red blood cells but was otherwise negative. The hemoglobin content of the blood was 69 per cent (Sahli). There were 4,000,000 red blood cells, 8,900 white cells, 13 per cent lymphocytes, 7 per cent mononuclear leukocytes, and 80 per cent neutrophiles. His blood was type IV. The 2-hour intravenous phthalein return was 45 per cent. A roentgenogram of the abdomen showed a large rounded soft tissue tumor mass filling the left side and extending across the mid-line into the right side of the abdomen. On physical examination a large smooth painless tumor mass was found filling the left side of his abdomen. It was firm, movable, rounded, and ballotable. Nothing else of unusual interest was found. Peritoneoscopy (Dr. J. C. Ruddock) was done through a right rectus stab incision. The peritoneal surfaces were seen clear and glistening. The liver was visualized and was not abnormal. The left lobe of the liver was pushed to the right and the stomach displaced upward by a large tumor which occupied three-fourths of the abdominal cavity. The tumor mass was below the omentum. The spleen was not visualized on account of the size and position of the tumor. Cystoscopy and ureteral catheterization: The bladder neck was scarred and contracted, but the cystoscope was passed without difficulty Clear urine w:;i,s spurting from both ureteral orifices. The ureters were catheterized. The intravenously injected phthalein return was not abnormal, the right appeared in 3 minutes, the left in 5; 16 per cent was returned in 15 minutes
HEMORRHAGIC CYSTS OF KIDNEY
105
from the right and 12 per cent from the left ureter. A left pyelogram showed the kidney displaced to the left by a large centrally placed tumor. The renal pelvis and calyces were extremely dilated (fig. 1). They were apparently separate from and not involved in the structure of the tumor mass. The dilatation was caused by pressure of the tumor mass on the upper ureter. In the ureterogram, that portion of the ureter passing underneath the mass shows no evidence of opaque material, but the section projecting below the mass is well outlined.
FIG. 1. Left pyelogram shows pelvis displaced to left by large centrally located tumorRenal pelvis and calices moderately dilated. Lower ureter slightly dilated up to point of pressure by tumor.
Operation (Doctors Toland and Scholl): Under gas and oxygen anesthesia a straight left rectus incision was made. Just below the costal margin the incision was extended to the left. This gave a satisfactory exposure of a large retroperitoneal mass. The colon was pulled toward the mid-line and an opening made in the mesocolon of the descending colon and upper sigmoid. No vessels were cut and the peritoneal cavity was opened for freer exploration. The left kidney was about 3 times normal size, and on both poles and from the under side were several large cystic dilatations which extended from the diaphragm to the pelvis and across to the lower right abdominal quadrant. The entire cystic mass was gently dissected out, most
106
A.
J.
SCHOLL
of it by blunt, but some of it by sharp, dissection. On account of the firm adhesions it was impossible to remove the mass intact. When torn, a large amount of old chocolate colored clots and black fluid escaped from the cystic mass. The entire cyst wall and the kidney were finally freed. The renal pedicle and the ureter were ligated, cut, and the whole mass removed. There was moderate hemorrhage during the course of removal of the mass, but there was no bleeding after the removal. The incision in the mesocolon was sutured and the peritoneum closed. The left rectus muscle which had been cut through was sutured and the abdominal incision closed in the usual manner. Three Penrose drains and one iodoform gauze pack were put in. The iodoform
FIG. 2. Papillary adenocarcinoma with large clear cells
gauze was inserted down to the region of the adrenal, which was plainly visible. The patient's general condition at the end of the operation was good, but on account of the extensive loss of fluid he was given intravenous salt .solution during the time of the operation and a blood transfusion in his room directly following the operation. For several days following the operation there was considerable shock, following which the patient rallied, leaving the hospital in three weeks in good condition, with the wound well healed. Two years later he had had no further trouble. The specimen removed consisted of the enlarged kidney and an extremely dilated sac. The kidney capsule was continuous over the cyst wall which
HEMORRHAGIC CYSTS OF KIDNEY
107
varied from 3 to 6 mm. in thickness. There was no evidence of renal parenchyma in the cyst wall as is usually seen in cases of hemorrhagic cyst. The inner layer of the wall was composed of old hemorrhagic masses of organized clots and fibrin. Some of the clots were very adherent to the cyst wall and could be removed only by tearing. The cyst which on external examination appeared to be multilocular was solitary and was filled with organized and partly organized blood clots together with a large amount of thin black fluid. Some of the clotted masses were apparently quite old, as they were shredded with fibrin, hard, and difficult to tear apart. At the base of the cyst there was a tumor mass about 8 cm. in diameter which had spread through the renal capsule to infiltrate the tissues around the ureter and renal vessels. Histologically this tumor was a rapidly growing papillary adenocarcinoma of the clear cell type (fig. 2) . Comment. The function of the left kidney in this case was normal, and the left pyelogram revealed an obstructive type of dilatation. The lateral position of the kidney, the distension and outline of the pelvis, and calices all suggested that the tumor mass present in the abdomen was not part of the kidney. Neither the recent history of his case nor the pyelogram were suggestive of a renal tumor; consequently, it was impossible to definitely state preoperatively that the mass was renal. Lancereaux in 1858 was the first to separate hemorrhagic cysts from the general group of large single cysts of the kidney. He stated that the contents were different and their walls thicker. Later Souligoux and Gouget in 1882 reviewed the cases of hemorrhagic cysts of the kidney reported up to that time and decided that they merited a special classification and that they had special characteristics. The work of Begg, Judd and Simon, Hepler, and later of Stirling, has done much to establish a separate identity for these cysts and to clarify their pathogenesis. Etiology. One of the simplest etiologic theories of the formation of hemorrhagic cysts of the kidney is that they are formed from bleeding into a solitary serous cyst, but, as brought out by Begg, the thick fibrous wall, the compressed renal substance, and the fibrous capsule of the kidney itself between the cyst contents and the kidney surface are very different from the conditions found in simple serous cysts. Barney has recently reported a case in which the kidney was the seat of both a simple serous cyst and a hemorrhagic cyst. Judd and Simon advanced the idea that large hemorrhagic cysts may arise as aneurysms in the renal parenchyma which, as they enlarge, stretch and compress the overlying parenchyma until only a thin layer of atrophic structure remains. Repeated layers of clotted blood then become deposited on the inner surface of the
108
A.
J.
SCHOL!'.,
aneurysm, the outer layer becoming compressed and organized into layers of fibrous tissue. By the time the cavity is recognized clinically, the process of clotting has advanced to such a degree that the lumen of the vessel has been partially or completely occluded, and the cavity is no longer supplied by fresh blood. The blood already present undergoes autolysis and the aneurysm at this stage ceases to exist, because it has been transferred into a cyst. The extensive hemorrhage reported by several observers which occurred while attempting to evacuate the contents of the cyst might be accounted for by the removal of a clot from the lumen of the vessel in which the aneurysm had occurred. The presence of muscle fibers and bundles within the cyst wall as well as the finding of remnants of intima with elastic fibers and an endothelial lining suggests this explanation of their origin. Hepler, who reviewed 37 cases of kidney hemorrhagic cysts reported in the literature, states that in general large cysts of the kidney are not a distinct entity with a common histogenesis but that their direct etiology varies, and because of this they may differ as to number, size, contents, sac, wall, and associated renal pathology. Hepler's contention, however, is that the mechanism of the production is essentially the same. He thinks that there is a tendency for each author to describe the origin of the condition on the basis of his own particular case, being careful to exclude from his classification others with a different etiology and with slight pathologic differences and losing sight of the fact that these cysts may not be a distinct entity with a common origin. Stirling believes that trauma plays an important part in the development of some of these cysts, as a clear-cut history of trauma followed by the formation of a tumor was seen in his own as well as in other cases. But Begg found that a history of trauma was absent in most cases, and even when it occurs the bleeding ceases and the resulting cysts contains the usual straw-colored fluid common to old traumatic blood effusions. Continuity of bleeding and not a single hemorrhage is essential for the formation of the large blood cysts. Begg holds that a partial hematonephrosis is possibly the cau,se in some cases. The bleeding may take place as the result of an angioma or follow one of the various obscure local conditions such as varices around a papilla or minute papillomata; the remainder of the kidney may be closed off by a blood clot. A slight hematuria, which has been noted in several cases, may pass unnoticed in others. Other theories are that these cysts arise from hemorrhagic infarcts or encapsulated hematomata. With regard to hemorrhagic cysts associated with neoplasm as in my
HEMORRHAGIC CYSTS OF KIDNEY
109
own case, several writers contend that all hemorrhagic cysts develop from neoplasms which have been destroyed in the growth of the cyst. A number of cases of hemorrhagic cysts associated with neoplasm have been reported. Le Comte reported a case of spontaneous rupture of a cyst; a clear cell carcinoma was also found in the kidney. Crabtree found a hemorrhagic cyst in association with a sarcoma. Neff reported the case of a woman of 58 who had a hemorrhagic cyst of the kidney of over 2 liters' capacity associated with a Wilms tumor. Remnants of tumor tissue were found in two of Hepler's personal cases and in 10 of the 37 cases of hemorrhagic cysts he reviewed from the literature. It is possible that the conditions present in my own case could well be explained by long continued hemorrhage from a highly vascular tumor. As has been stated above, the association of hemorrhagic cysts and neoplasm is not uncommon. In some of the reported cases, in which tumors and hemorrhagic cysts were associated in the same kidney, the growth was comparatively small in size, while in others, as in the case presented here, it was quite large. It is not improbable that some of these cases start merely as hemorrhage into a large renal neoplasm. A superimposed infection, repeated hemorrhages, and an increased intrarenal tension may well cause partial or almost complete destruction of the neoplasm, resulting in such a picture as was found in this case. Diagnosis. Large hemorrhagic cysts present much the same clinical features as large serous cysts so far as symptoms and diagnosis are concerned. There is usually very little pain associated with the development of the cyst. At times the only discomfort associated with the cystic mass is caused by the pressure on the surrounding organs. Rarely are there any changes in the urine. The hematuria reported in occasional cases is more typical and suggestive of other lesions than of a hemorrhagic cyst. On physical examination the enlarged kidney is usually readily felt ; in some cases the cystic mass fills the entire abdomen and an abdominal fluid wave may be obtained. Cystoscopy and pyelography identifies the mass as being associated with the kidney. A hydro- or hematonephrosis involves the renal pelvis; the large cysts usually leave the pelvis intact. Occasionally squamous cell tumors of the renal pelvis cause tremendous dilatation of the kidney, almost filling the entire abdomen as do at times the hemorrhagic cysts. There may be considerable functionating parenchyma present, and the phthalein return may be only slightly reduced on the affected side. The X-ray offers our best
110
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SCHOLL
aid in diagnosis. The large rounded outline of th!:! ,cyst may readily be seen, especially if the cyst is at the lower pole of the kidney, as it is most frequently . Cysts of the upper pole may be obscured by upper abdominal organs, the liver and the spleen. The pyelogram may be negative, though, as Braasch has pointed out, the deformity usually seen is an abbreviation of the adjacent calices, compression and fla,ttening of the adjacent portion of the renal pelvis together with a change in the position and axis of the kidney as a result of the weight of the mass. Stirling noted that there was a marked deviation of the ureter if the cyst involved the lower pole. The pyelogram in Lamson's case demonstrated the right pelvis displaced 10 cm. to the left of the mid-line. In a case reported by Neff a pyelogram of the right kidney was not abnormal, the left ureter was pushed over to the right, and the left renal pelvis was displaced by a hemorrhagic cyst 6 cm. to the right of the right renal pelvis and 12 cm. to the right of the mid-line .. In my own case the pyelogram showed the left kidney displaced to the left. Treatment . Begg, in writing on hemorrhagic cysts, summed up the situation when he stated, "The diagnosis is obscure and the treatment total nephrectomy." Resection of the cyst has been suggested by several, but, except in the case of a small cyst, technically this would be difficult, as the cyst is usually an integral part of the kidney itself, and generally very firm adhesions have been. formed between the renal mass and the surrounding tissues. In most cases there would not be enough functioning renal tissue left to justify resection, and this as well as the frequency of associated malignant changes would decidedly indicate nephrectomy if the condition of the opposite kidney was satisfactory. REFERENCES BARNEY : Hemorrhagic cyst of kidney. J. Urol., 36: 602, 1936. BEGG: Solitary hemorrhagic cysts of kidney, etc. Brit. J. Surg., 13: 649, 1926. CRABTREE : Personal communication. HEPLER: Solitary cysts of kidney. Surg., Gynec. and Obst., 60: 668, 1930. JUDD AND SIMON : Hemorrhagic cysts of kidney. Surg. , Gynec. and Obst., 44: 601, 1927. LAMSON : Solitary cyst of kidney. Northwest. Med., 29: 206, 1930. LANCEREAUX: Bull. Soc. Anat. de Paris, 33: 205, 1858. LEOPOLD: Arch f. Gynaek, 19: 129, 1882. LE COMTE: Subcutaneous kidney ruptures. Urol. and Cutan. Rev., 39: 13, 1935. NEFF: Massive hemorrhagic cyst in Wilms' tumor in adult. J . Urol., 28: 65, 1932. SouuGoux AND GouGET: Contribution a l'Etude des· grandes Kystees hemaliques simples du Rein. Arch. Gen. de Med., 11: 833, 1882. STIRLING: Large solitary hemorrhagic renal cyst with review of literature. J . Urol., 26: 213, 1931.
A.
J. SCHOLL
Hemorrhagic cysts of the kidney are different from the ordinary, more common, thin-walled, serous cysts. Hemorrhagic cysts are smooth and continuous with the contour of the kidney surface and do not abruptly project from the renal parenchyma like serous cysts. At times the surface of the cysts may be lobulated or nodular; they are usually opaque or whitish in color; the walls are composed of several layers which may be quite thick, at times as much as 1 cm. wide; the cysts are usually markedly adherent to the surrounding organs, tearing easily on attempted removal. Externally, the renal capsule is continuous over the cyst wall and is usually readily separated from it; next there is a layer of thinned-out renal parenchyma together with a layer of fibrous tissue; the inner layer is composed of the products of old hemorrhagic masses of fibrin and adherent organized clots. The contents of the cyst are made up of partially or completely organized blood clots. The central areas may contain large masses of light brown clots, mixed with grayish white masses of fibrin. Occasionally there is a large quantity of black or brown fluid with, at times, areas suggesting fresh hemorrhage. Hemorrhagic cysts are usually solitary and large in size. Rarely are they multilocular; Judd and Simon reported a case in which three cysts were present. As a rule the cysts are fairly large in size, and occasionally they are enormous. Leopold reported a case in which the cyst had a capacity of four liters. Neff aspirated over 8 liters of chocolate colored fluid from a large hemorrhagic cyst before operation. In Lamson's case the cyst contained over 11 liters. It is not uncommon to find a hemorrhagic cyst associated with a kidney neoplasm as in the following personal case, recently observed, in which an adenocarcinoma and a large hemorrhagic cyst were found associated in the same kidney. Case Report. A man aged 58 came for examination on account of a swelling of the abdomen which had been present for 3 years. There was considerable pain in the left side of the abdomen at the time the swelling was first noticed. The pain was fairly continuous, located in the upper part of the abdomen, and 103
104
A.
J.
SCHOLL
did not radiate to the back or downward. It was quite severe at the onset but gradually lessened and after 2 months disappeared. A tumor was found in the region of the left kidney by his physician who advised that nothing could be done about it. A blood examination was negative at that time. The mass or "swelling," which was painless except for the first 2 months, increased in size up to 6 months ago. Since then it has increased more rapidly in size, and there was a pulling or stabbing sensation in the abdomen. Six weeks ago the patient had chills and fever and went to bed for 10 days. Following that his side felt so ' 1full" and painful that it was difficult for him to move around. Recently his abdomen had been so distended that breathing was difficult. There was no hematuria or dysuria, but the man had to empty his bladder 3 times a night for many years. The patient had what was apparently a specific urethritis when he was a young man and somewhat later a penile lesion which was possibly a chancre. Four Wassermann tests, all recent, the last 6 months ago, were negative. Thirty years ago he passed a kidney or ureteral stone associated with pain in the left kidney. In 1928 a prostatectomy was done with only partial relief of his urinary symptoms. At this time a roentgenogram showed a mass in the left side of the abdomen. The urine contained a few red blood cells but was otherwise negative. The hemoglobin content of the blood was 69 per cent (Sahli). There were 4,000,000 red blood cells, 8,900 white cells, 13 per cent lymphocytes, 7 per cent mononuclear leukocytes, and 80 per cent neutrophiles. His blood was type IV. The 2-hour intravenous phthalein return was 45 per cent. A roentgenogram of the abdomen showed a large rounded soft tissue tumor mass filling the left side and extending across the mid-line into the right side of the abdomen. On physical examination a large smooth painless tumor mass was found filling the left side of his abdomen. It was firm, movable, rounded, and ballotable. Nothing else of unusual interest was found. Peritoneoscopy (Dr. J. C. Ruddock) was done through a right rectus stab incision. The peritoneal surfaces were seen clear and glistening. The liver was visualized and was not abnormal. The left lobe of the liver was pushed to the right and the stomach displaced upward by a large tumor which occupied three-fourths of the abdominal cavity. The tumor mass was below the omentum. The spleen was not visualized on account of the size and position of the tumor. Cystoscopy and ureteral catheterization: The bladder neck was scarred and contracted, but the cystoscope was passed without difficulty Clear urine w:;i,s spurting from both ureteral orifices. The ureters were catheterized. The intravenously injected phthalein return was not abnormal, the right appeared in 3 minutes, the left in 5; 16 per cent was returned in 15 minutes
HEMORRHAGIC CYSTS OF KIDNEY
105
from the right and 12 per cent from the left ureter. A left pyelogram showed the kidney displaced to the left by a large centrally placed tumor. The renal pelvis and calyces were extremely dilated (fig. 1). They were apparently separate from and not involved in the structure of the tumor mass. The dilatation was caused by pressure of the tumor mass on the upper ureter. In the ureterogram, that portion of the ureter passing underneath the mass shows no evidence of opaque material, but the section projecting below the mass is well outlined.
FIG. 1. Left pyelogram shows pelvis displaced to left by large centrally located tumorRenal pelvis and calices moderately dilated. Lower ureter slightly dilated up to point of pressure by tumor.
Operation (Doctors Toland and Scholl): Under gas and oxygen anesthesia a straight left rectus incision was made. Just below the costal margin the incision was extended to the left. This gave a satisfactory exposure of a large retroperitoneal mass. The colon was pulled toward the mid-line and an opening made in the mesocolon of the descending colon and upper sigmoid. No vessels were cut and the peritoneal cavity was opened for freer exploration. The left kidney was about 3 times normal size, and on both poles and from the under side were several large cystic dilatations which extended from the diaphragm to the pelvis and across to the lower right abdominal quadrant. The entire cystic mass was gently dissected out, most
106
A.
J.
SCHOLL
of it by blunt, but some of it by sharp, dissection. On account of the firm adhesions it was impossible to remove the mass intact. When torn, a large amount of old chocolate colored clots and black fluid escaped from the cystic mass. The entire cyst wall and the kidney were finally freed. The renal pedicle and the ureter were ligated, cut, and the whole mass removed. There was moderate hemorrhage during the course of removal of the mass, but there was no bleeding after the removal. The incision in the mesocolon was sutured and the peritoneum closed. The left rectus muscle which had been cut through was sutured and the abdominal incision closed in the usual manner. Three Penrose drains and one iodoform gauze pack were put in. The iodoform
FIG. 2. Papillary adenocarcinoma with large clear cells
gauze was inserted down to the region of the adrenal, which was plainly visible. The patient's general condition at the end of the operation was good, but on account of the extensive loss of fluid he was given intravenous salt .solution during the time of the operation and a blood transfusion in his room directly following the operation. For several days following the operation there was considerable shock, following which the patient rallied, leaving the hospital in three weeks in good condition, with the wound well healed. Two years later he had had no further trouble. The specimen removed consisted of the enlarged kidney and an extremely dilated sac. The kidney capsule was continuous over the cyst wall which
HEMORRHAGIC CYSTS OF KIDNEY
107
varied from 3 to 6 mm. in thickness. There was no evidence of renal parenchyma in the cyst wall as is usually seen in cases of hemorrhagic cyst. The inner layer of the wall was composed of old hemorrhagic masses of organized clots and fibrin. Some of the clots were very adherent to the cyst wall and could be removed only by tearing. The cyst which on external examination appeared to be multilocular was solitary and was filled with organized and partly organized blood clots together with a large amount of thin black fluid. Some of the clotted masses were apparently quite old, as they were shredded with fibrin, hard, and difficult to tear apart. At the base of the cyst there was a tumor mass about 8 cm. in diameter which had spread through the renal capsule to infiltrate the tissues around the ureter and renal vessels. Histologically this tumor was a rapidly growing papillary adenocarcinoma of the clear cell type (fig. 2) . Comment. The function of the left kidney in this case was normal, and the left pyelogram revealed an obstructive type of dilatation. The lateral position of the kidney, the distension and outline of the pelvis, and calices all suggested that the tumor mass present in the abdomen was not part of the kidney. Neither the recent history of his case nor the pyelogram were suggestive of a renal tumor; consequently, it was impossible to definitely state preoperatively that the mass was renal. Lancereaux in 1858 was the first to separate hemorrhagic cysts from the general group of large single cysts of the kidney. He stated that the contents were different and their walls thicker. Later Souligoux and Gouget in 1882 reviewed the cases of hemorrhagic cysts of the kidney reported up to that time and decided that they merited a special classification and that they had special characteristics. The work of Begg, Judd and Simon, Hepler, and later of Stirling, has done much to establish a separate identity for these cysts and to clarify their pathogenesis. Etiology. One of the simplest etiologic theories of the formation of hemorrhagic cysts of the kidney is that they are formed from bleeding into a solitary serous cyst, but, as brought out by Begg, the thick fibrous wall, the compressed renal substance, and the fibrous capsule of the kidney itself between the cyst contents and the kidney surface are very different from the conditions found in simple serous cysts. Barney has recently reported a case in which the kidney was the seat of both a simple serous cyst and a hemorrhagic cyst. Judd and Simon advanced the idea that large hemorrhagic cysts may arise as aneurysms in the renal parenchyma which, as they enlarge, stretch and compress the overlying parenchyma until only a thin layer of atrophic structure remains. Repeated layers of clotted blood then become deposited on the inner surface of the
108
A.
J.
SCHOL!'.,
aneurysm, the outer layer becoming compressed and organized into layers of fibrous tissue. By the time the cavity is recognized clinically, the process of clotting has advanced to such a degree that the lumen of the vessel has been partially or completely occluded, and the cavity is no longer supplied by fresh blood. The blood already present undergoes autolysis and the aneurysm at this stage ceases to exist, because it has been transferred into a cyst. The extensive hemorrhage reported by several observers which occurred while attempting to evacuate the contents of the cyst might be accounted for by the removal of a clot from the lumen of the vessel in which the aneurysm had occurred. The presence of muscle fibers and bundles within the cyst wall as well as the finding of remnants of intima with elastic fibers and an endothelial lining suggests this explanation of their origin. Hepler, who reviewed 37 cases of kidney hemorrhagic cysts reported in the literature, states that in general large cysts of the kidney are not a distinct entity with a common histogenesis but that their direct etiology varies, and because of this they may differ as to number, size, contents, sac, wall, and associated renal pathology. Hepler's contention, however, is that the mechanism of the production is essentially the same. He thinks that there is a tendency for each author to describe the origin of the condition on the basis of his own particular case, being careful to exclude from his classification others with a different etiology and with slight pathologic differences and losing sight of the fact that these cysts may not be a distinct entity with a common origin. Stirling believes that trauma plays an important part in the development of some of these cysts, as a clear-cut history of trauma followed by the formation of a tumor was seen in his own as well as in other cases. But Begg found that a history of trauma was absent in most cases, and even when it occurs the bleeding ceases and the resulting cysts contains the usual straw-colored fluid common to old traumatic blood effusions. Continuity of bleeding and not a single hemorrhage is essential for the formation of the large blood cysts. Begg holds that a partial hematonephrosis is possibly the cau,se in some cases. The bleeding may take place as the result of an angioma or follow one of the various obscure local conditions such as varices around a papilla or minute papillomata; the remainder of the kidney may be closed off by a blood clot. A slight hematuria, which has been noted in several cases, may pass unnoticed in others. Other theories are that these cysts arise from hemorrhagic infarcts or encapsulated hematomata. With regard to hemorrhagic cysts associated with neoplasm as in my
HEMORRHAGIC CYSTS OF KIDNEY
109
own case, several writers contend that all hemorrhagic cysts develop from neoplasms which have been destroyed in the growth of the cyst. A number of cases of hemorrhagic cysts associated with neoplasm have been reported. Le Comte reported a case of spontaneous rupture of a cyst; a clear cell carcinoma was also found in the kidney. Crabtree found a hemorrhagic cyst in association with a sarcoma. Neff reported the case of a woman of 58 who had a hemorrhagic cyst of the kidney of over 2 liters' capacity associated with a Wilms tumor. Remnants of tumor tissue were found in two of Hepler's personal cases and in 10 of the 37 cases of hemorrhagic cysts he reviewed from the literature. It is possible that the conditions present in my own case could well be explained by long continued hemorrhage from a highly vascular tumor. As has been stated above, the association of hemorrhagic cysts and neoplasm is not uncommon. In some of the reported cases, in which tumors and hemorrhagic cysts were associated in the same kidney, the growth was comparatively small in size, while in others, as in the case presented here, it was quite large. It is not improbable that some of these cases start merely as hemorrhage into a large renal neoplasm. A superimposed infection, repeated hemorrhages, and an increased intrarenal tension may well cause partial or almost complete destruction of the neoplasm, resulting in such a picture as was found in this case. Diagnosis. Large hemorrhagic cysts present much the same clinical features as large serous cysts so far as symptoms and diagnosis are concerned. There is usually very little pain associated with the development of the cyst. At times the only discomfort associated with the cystic mass is caused by the pressure on the surrounding organs. Rarely are there any changes in the urine. The hematuria reported in occasional cases is more typical and suggestive of other lesions than of a hemorrhagic cyst. On physical examination the enlarged kidney is usually readily felt ; in some cases the cystic mass fills the entire abdomen and an abdominal fluid wave may be obtained. Cystoscopy and pyelography identifies the mass as being associated with the kidney. A hydro- or hematonephrosis involves the renal pelvis; the large cysts usually leave the pelvis intact. Occasionally squamous cell tumors of the renal pelvis cause tremendous dilatation of the kidney, almost filling the entire abdomen as do at times the hemorrhagic cysts. There may be considerable functionating parenchyma present, and the phthalein return may be only slightly reduced on the affected side. The X-ray offers our best
110
A.
J.
SCHOLL
aid in diagnosis. The large rounded outline of th!:! ,cyst may readily be seen, especially if the cyst is at the lower pole of the kidney, as it is most frequently . Cysts of the upper pole may be obscured by upper abdominal organs, the liver and the spleen. The pyelogram may be negative, though, as Braasch has pointed out, the deformity usually seen is an abbreviation of the adjacent calices, compression and fla,ttening of the adjacent portion of the renal pelvis together with a change in the position and axis of the kidney as a result of the weight of the mass. Stirling noted that there was a marked deviation of the ureter if the cyst involved the lower pole. The pyelogram in Lamson's case demonstrated the right pelvis displaced 10 cm. to the left of the mid-line. In a case reported by Neff a pyelogram of the right kidney was not abnormal, the left ureter was pushed over to the right, and the left renal pelvis was displaced by a hemorrhagic cyst 6 cm. to the right of the right renal pelvis and 12 cm. to the right of the mid-line .. In my own case the pyelogram showed the left kidney displaced to the left. Treatment . Begg, in writing on hemorrhagic cysts, summed up the situation when he stated, "The diagnosis is obscure and the treatment total nephrectomy." Resection of the cyst has been suggested by several, but, except in the case of a small cyst, technically this would be difficult, as the cyst is usually an integral part of the kidney itself, and generally very firm adhesions have been. formed between the renal mass and the surrounding tissues. In most cases there would not be enough functioning renal tissue left to justify resection, and this as well as the frequency of associated malignant changes would decidedly indicate nephrectomy if the condition of the opposite kidney was satisfactory. REFERENCES BARNEY : Hemorrhagic cyst of kidney. J. Urol., 36: 602, 1936. BEGG: Solitary hemorrhagic cysts of kidney, etc. Brit. J. Surg., 13: 649, 1926. CRABTREE : Personal communication. HEPLER: Solitary cysts of kidney. Surg., Gynec. and Obst., 60: 668, 1930. JUDD AND SIMON : Hemorrhagic cysts of kidney. Surg. , Gynec. and Obst., 44: 601, 1927. LAMSON : Solitary cyst of kidney. Northwest. Med., 29: 206, 1930. LANCEREAUX: Bull. Soc. Anat. de Paris, 33: 205, 1858. LEOPOLD: Arch f. Gynaek, 19: 129, 1882. LE COMTE: Subcutaneous kidney ruptures. Urol. and Cutan. Rev., 39: 13, 1935. NEFF: Massive hemorrhagic cyst in Wilms' tumor in adult. J . Urol., 28: 65, 1932. SouuGoux AND GouGET: Contribution a l'Etude des· grandes Kystees hemaliques simples du Rein. Arch. Gen. de Med., 11: 833, 1882. STIRLING: Large solitary hemorrhagic renal cyst with review of literature. J . Urol., 26: 213, 1931.