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Cavernous hemangioma of the adrenal gland
Cavernous Hemangioma
of the Adrenal Gland
RICHARD J. CHODOFF, M.D., J. WINSLOW SMITH, M.D., AND NORTON HERING, M.D.,
Havertown, Pennsylvania
From Haverford Hospital,
Havertown,
3. Cystic adenomas. These are also uncommon, resembling pseudocysts but containing associated adenomatous elements. 4. Endothelial cysts. Outside of the angiowhich are extremely rare, matous variety, these are the second most common type. They are usually multiloculated with thin fibrous septa and a flattened endothelial lining, and contain a clear or milky fluid. 5. Pseudocysts. These are the common variety and result from organizational resorption of hemorrhage in or about a normal or diseased adrenal gland. The original hemorrhage may be due to any of the usual causes, such as injuries, infarcts, and ruptured small aneurysms. This type of cyst is usually unilocular and spherical and has small compressed foci of adrenal cortical substance peripherally. The contents may be clear or bloody. One third of the reported pseudocysts contain calcium, usually seen on roentgenogram as a curvilinear density about the wall of the cyst. The case we are reporting here was diagnosed by our pathologist as a true cavernous hemangioma. Sections were submitted to several other pathologists who all concurred in the diagnosis. Since this is apparently one of the very few surgically treated cysts of this extremely rare type, it is being added to the literature.
Pennsylvania.
HE MAJORITY of reported benign adrenal have been found at autopsy, the total number of cases reported as having been discovered at surgery being extremely small. A review of the world literature in 1959 by Abeshouse, Goldstein, and Abeshouse [l] revealed only 155 cases, of which three were their own. Of these, eighty-eight were discovered at autopsy and sixty-seven at operation. Martin and Amerson [Z] reviewed the American literature on adrenal cysts in 1963 and found sixty-one cases, to which they added two. Apparently the rarest type of adrenal cyst is the benign cavernous hemangioma. In 1955 Johnson and Jeppesen [3] found only six cases of this type of tumor in the world literature, all discovered at autopsy, quoted another case found at autopsy found by Herbut [4] and recorded the first case treated surgically. Their patient was a forty-nine year old female with hypertension in whom intravenous pyelography had revealed a small mass in the region of the right adrenal gland. Diagnostic studies for pheochromocytoma gave negative results. At operation a solid mass assuring 4 cm. in diameter was found attached to the right adrenal gland. This was removed and found to be a cavernous heman$orr~ of the adrenal with adenomatous hyperof the adrenal cortex. ‘&e classification of adrenal cysts most commonly used is that of Terrier and Lecine [5], quoted in an article by Gregorie, Bradham, and Chapman [6] in 1962, in which they reviewed the subject and reported two cases. A summary of this classification follows : 1. Parasitic cysts which are uncommon and usually due to echinococcus infestation. 2. Retention or congenital glandular cysts which are also rare.
T cysts
Vol. 111, A@4
1966
CASE REPORT The patient, S. P., a seventy-six year old white woman, was admitted to Haverford Hospital on June 1, 1959 because of a large abdominal mass, which the patient had first noticed about six months prior to admission; the mass had increased in size during this time. Her only complaints were discomfort in the region of the mass, slight weakness, and moderate weight loss. The patient gave no history of any operations or major illnesses. The significant finding on examination was a mass about the size of a football in the upper left abdominal quadrant. This 595
uas some\vltat movable and did not seem to arise from eitltcr the spleen or the left kidney. Complete l)lood count. urinalysis. blood sugar, and blood urea nitrogen were within normal limits. .kt x-ray film of the chest revealed no abnormalities except for aortic calcification and left T.entricular hypertroph;-. A survey lilm taken prior to intravenous pyelography showed a large 5oft tissue mass. with some calcification. in the left upper quadrant, The splenic shadow appeared normal. No abnormalities of the collecting svstcm of eitlter kidnel- were found nor was there ciisl,lacetttcnt of the leit kidncy. .-\ barium enema carried out on June 1 showed no intrinsic cola&c abnortnalitiea. but revealed marked depression and anterior displacetncnt of the splenic flexure and transverse colon by the soft tissue mass. (Fig. 1.) On June 8 an upper gastrointestinal series showed displacctncnt of the stomach anteriorly and to the right bv the mass. (Fig. 2. j It was the radiologist’s impr&ion that the tnass arose in the retroperitorreal space, probably from the tail of the pancreas. After rel-iewittg all studies a prcoI)erati\-e diagnosis of llcttign Ijancreatic cyst was made and the patient was opcratrd upon on June 1.5. The ahdomen was o~lencd through a long, transverse supraun&&&l incision. X large, thick-walled cyst was foun&,$$& ing from the rctroperitoneal space in the regiba of the tail of the pancreas and presenting between the stomach and transverse colon. producing the lmssttrc deformities and displacements previously noted in the s-ray studies. The gastrocolic ligament wab opened and the cvst dissected free from the stomach and colon. The pkdicle was freed from the tail of the pancreas and the splenic and left renal vessels, and the c\-st was excisccl. The abdomen was closed in layers without drainage. After an uneventful course -4~,re~icanJourmzl
oj- .Szwji<~y
Cavernous
Hemangioma
the patient was discharged on the tenth postopcrative dav. The -pathologist’s report revealed that on gross examination the specimen consisted of an ovoid mass measuring IS I)!- II\ cm. (Fig. 3.) On sectioning, it was well encapsulated, the capsule having two yellowish areas. The cut surface was mottled pinkish yellow to gray and hemorrhage was present in the peripheral areas. The central area was soft and full of friable, chocolate-colored clot. Miscroscopic examination of the cyst revealed a rim of adrenal cortex with some evidence of pressure atrophy of the inner zone. The medulla appeared to be replaced 1,~. enclothelially-lined spaces and engorgement with red blood cells and areas of rup ture with hemorrhage. There was neither evidenct of remaining cells of the adrenal medulla nor evidence of malignancy. (Fig. -1.J The diagnosis given was cavernous hemangioma of the adrenal medulla.
of the Adrenal
Gland
typical effects of pressure upon colon and stomach are well illustrated in this case. However, this patient did not have depression of the kidney, which has been noted as a frequent finding. The presence of a curved rim of calcification around a portion of the mass may lead to the suspicion that one is dealing with an adrenal pseudocyst. However, it is interesting that in this case calcification was present but the cyst was not a pseudocyst. The treatment of adrenal cysts is by surgical excision. The approach must be transperitoneal for obvious reasons, that is, the size of the mass and the uncertainty as to the exact anatomic and pathologic diagnosis. There were no technical problems encountered in removing this cyst; this has apparently been the experience in most of the other reported cases. SUMMAR?
COMMENTS
There are no symptoms characteristic of adrenal cysts. When they become large enough to exert pressure, the usual complaints are discomfort in the area, vague gastrointestinal symptoms, and, of course, the presence of a palpable mass. Detectable changes in adrenal hormone production are practically never present. Only in the rare case arising from hemorrhage into a pheochromocytoma can the adrenal be suspected as the organ of origin in the cyst [7]. Although Elliott and associates [8] state that the diagnosis of adrenal cyst may be suspected from the fact that it can be felt separately from the kidney, liver, and colon during deep inspiration, the fact is that seldom has the correct preoperative diagnosis been made. In most of the reported cases? as in ours, the preoperative impression was benign cyst, probably pancreatic, but possibly omental, mesenteric or splenic. Significant roentgenographic findings result when the cyst becomes large enough to produce extrinsic pressure deformities or displacement of adjacent organs, primarily the stomach, colon, and kidney. The
Vol. 111, Afwil 1966
597
1. The
literature
on benign adrenal cysts is reviewed. 2. A case is reported of the surgical removal of an extremely rare type of cyst, the benign cavernous hemangioma. REFERENCES 1. ABESHOUSE, G. A., GOLDSTEIN, R. B., and ABESHOUSE, B. S. Adrenal cysts: review of the literature and report of three cases. J. Ural., 81: 711, 1959. 2. MARTIN, J. D., JR. and AMERSON, J. R. Adrenal cysts. Am. Surgeon, 29: 52, 1963. 3. JOHNSON, C. C. and JEPPESEN, F. B. Hemangioma of the adrenal. J. Ural., 74: 573, 1955. 4. HERBUT, P. Cited by Johnson, C. C. and Jeppesen, F. B. [3]. 5. TERRIER, F. and LECINE, P. Cited by Gregorie, H. B., Jr., Bradham, R. R., and Chapman, W. W. [6]. 6. GREGORIE, H. B., JR., BRADHAM, R. R., and CHAPMAN, W. W. Adrenal cysts. Surgery, 52: 860, 1962. 7. ELLIS. F. H.. TR.. DAWE. C. T.. and CLAGETT. 0. T. Cysts of the adrenal piand:. ‘Ann. Surg., 136: 217,
1952. 8. ELLIOTT, G. B., WALKER, R. H., WRIGHT, A. S., and ELLIOTT, K. A. Adrenal giant cyst: hemagioma of medulla with osmotic pseudocyst formation. Ann.
Szug.,
159: 275, 1964.
of the Adrenal Gland
RICHARD J. CHODOFF, M.D., J. WINSLOW SMITH, M.D., AND NORTON HERING, M.D.,
Havertown, Pennsylvania
From Haverford Hospital,
Havertown,
3. Cystic adenomas. These are also uncommon, resembling pseudocysts but containing associated adenomatous elements. 4. Endothelial cysts. Outside of the angiowhich are extremely rare, matous variety, these are the second most common type. They are usually multiloculated with thin fibrous septa and a flattened endothelial lining, and contain a clear or milky fluid. 5. Pseudocysts. These are the common variety and result from organizational resorption of hemorrhage in or about a normal or diseased adrenal gland. The original hemorrhage may be due to any of the usual causes, such as injuries, infarcts, and ruptured small aneurysms. This type of cyst is usually unilocular and spherical and has small compressed foci of adrenal cortical substance peripherally. The contents may be clear or bloody. One third of the reported pseudocysts contain calcium, usually seen on roentgenogram as a curvilinear density about the wall of the cyst. The case we are reporting here was diagnosed by our pathologist as a true cavernous hemangioma. Sections were submitted to several other pathologists who all concurred in the diagnosis. Since this is apparently one of the very few surgically treated cysts of this extremely rare type, it is being added to the literature.
Pennsylvania.
HE MAJORITY of reported benign adrenal have been found at autopsy, the total number of cases reported as having been discovered at surgery being extremely small. A review of the world literature in 1959 by Abeshouse, Goldstein, and Abeshouse [l] revealed only 155 cases, of which three were their own. Of these, eighty-eight were discovered at autopsy and sixty-seven at operation. Martin and Amerson [Z] reviewed the American literature on adrenal cysts in 1963 and found sixty-one cases, to which they added two. Apparently the rarest type of adrenal cyst is the benign cavernous hemangioma. In 1955 Johnson and Jeppesen [3] found only six cases of this type of tumor in the world literature, all discovered at autopsy, quoted another case found at autopsy found by Herbut [4] and recorded the first case treated surgically. Their patient was a forty-nine year old female with hypertension in whom intravenous pyelography had revealed a small mass in the region of the right adrenal gland. Diagnostic studies for pheochromocytoma gave negative results. At operation a solid mass assuring 4 cm. in diameter was found attached to the right adrenal gland. This was removed and found to be a cavernous heman$orr~ of the adrenal with adenomatous hyperof the adrenal cortex. ‘&e classification of adrenal cysts most commonly used is that of Terrier and Lecine [5], quoted in an article by Gregorie, Bradham, and Chapman [6] in 1962, in which they reviewed the subject and reported two cases. A summary of this classification follows : 1. Parasitic cysts which are uncommon and usually due to echinococcus infestation. 2. Retention or congenital glandular cysts which are also rare.
T cysts
Vol. 111, A@4
1966
CASE REPORT The patient, S. P., a seventy-six year old white woman, was admitted to Haverford Hospital on June 1, 1959 because of a large abdominal mass, which the patient had first noticed about six months prior to admission; the mass had increased in size during this time. Her only complaints were discomfort in the region of the mass, slight weakness, and moderate weight loss. The patient gave no history of any operations or major illnesses. The significant finding on examination was a mass about the size of a football in the upper left abdominal quadrant. This 595
uas some\vltat movable and did not seem to arise from eitltcr the spleen or the left kidney. Complete l)lood count. urinalysis. blood sugar, and blood urea nitrogen were within normal limits. .kt x-ray film of the chest revealed no abnormalities except for aortic calcification and left T.entricular hypertroph;-. A survey lilm taken prior to intravenous pyelography showed a large 5oft tissue mass. with some calcification. in the left upper quadrant, The splenic shadow appeared normal. No abnormalities of the collecting svstcm of eitlter kidnel- were found nor was there ciisl,lacetttcnt of the leit kidncy. .-\ barium enema carried out on June 1 showed no intrinsic cola&c abnortnalitiea. but revealed marked depression and anterior displacetncnt of the splenic flexure and transverse colon by the soft tissue mass. (Fig. 1.) On June 8 an upper gastrointestinal series showed displacctncnt of the stomach anteriorly and to the right bv the mass. (Fig. 2. j It was the radiologist’s impr&ion that the tnass arose in the retroperitorreal space, probably from the tail of the pancreas. After rel-iewittg all studies a prcoI)erati\-e diagnosis of llcttign Ijancreatic cyst was made and the patient was opcratrd upon on June 1.5. The ahdomen was o~lencd through a long, transverse supraun&&&l incision. X large, thick-walled cyst was foun&,$$& ing from the rctroperitoneal space in the regiba of the tail of the pancreas and presenting between the stomach and transverse colon. producing the lmssttrc deformities and displacements previously noted in the s-ray studies. The gastrocolic ligament wab opened and the cvst dissected free from the stomach and colon. The pkdicle was freed from the tail of the pancreas and the splenic and left renal vessels, and the c\-st was excisccl. The abdomen was closed in layers without drainage. After an uneventful course -4~,re~icanJourmzl
oj- .Szwji<~y
Cavernous
Hemangioma
the patient was discharged on the tenth postopcrative dav. The -pathologist’s report revealed that on gross examination the specimen consisted of an ovoid mass measuring IS I)!- II\ cm. (Fig. 3.) On sectioning, it was well encapsulated, the capsule having two yellowish areas. The cut surface was mottled pinkish yellow to gray and hemorrhage was present in the peripheral areas. The central area was soft and full of friable, chocolate-colored clot. Miscroscopic examination of the cyst revealed a rim of adrenal cortex with some evidence of pressure atrophy of the inner zone. The medulla appeared to be replaced 1,~. enclothelially-lined spaces and engorgement with red blood cells and areas of rup ture with hemorrhage. There was neither evidenct of remaining cells of the adrenal medulla nor evidence of malignancy. (Fig. -1.J The diagnosis given was cavernous hemangioma of the adrenal medulla.
of the Adrenal
Gland
typical effects of pressure upon colon and stomach are well illustrated in this case. However, this patient did not have depression of the kidney, which has been noted as a frequent finding. The presence of a curved rim of calcification around a portion of the mass may lead to the suspicion that one is dealing with an adrenal pseudocyst. However, it is interesting that in this case calcification was present but the cyst was not a pseudocyst. The treatment of adrenal cysts is by surgical excision. The approach must be transperitoneal for obvious reasons, that is, the size of the mass and the uncertainty as to the exact anatomic and pathologic diagnosis. There were no technical problems encountered in removing this cyst; this has apparently been the experience in most of the other reported cases. SUMMAR?
COMMENTS
There are no symptoms characteristic of adrenal cysts. When they become large enough to exert pressure, the usual complaints are discomfort in the area, vague gastrointestinal symptoms, and, of course, the presence of a palpable mass. Detectable changes in adrenal hormone production are practically never present. Only in the rare case arising from hemorrhage into a pheochromocytoma can the adrenal be suspected as the organ of origin in the cyst [7]. Although Elliott and associates [8] state that the diagnosis of adrenal cyst may be suspected from the fact that it can be felt separately from the kidney, liver, and colon during deep inspiration, the fact is that seldom has the correct preoperative diagnosis been made. In most of the reported cases? as in ours, the preoperative impression was benign cyst, probably pancreatic, but possibly omental, mesenteric or splenic. Significant roentgenographic findings result when the cyst becomes large enough to produce extrinsic pressure deformities or displacement of adjacent organs, primarily the stomach, colon, and kidney. The
Vol. 111, Afwil 1966
597
1. The
literature
on benign adrenal cysts is reviewed. 2. A case is reported of the surgical removal of an extremely rare type of cyst, the benign cavernous hemangioma. REFERENCES 1. ABESHOUSE, G. A., GOLDSTEIN, R. B., and ABESHOUSE, B. S. Adrenal cysts: review of the literature and report of three cases. J. Ural., 81: 711, 1959. 2. MARTIN, J. D., JR. and AMERSON, J. R. Adrenal cysts. Am. Surgeon, 29: 52, 1963. 3. JOHNSON, C. C. and JEPPESEN, F. B. Hemangioma of the adrenal. J. Ural., 74: 573, 1955. 4. HERBUT, P. Cited by Johnson, C. C. and Jeppesen, F. B. [3]. 5. TERRIER, F. and LECINE, P. Cited by Gregorie, H. B., Jr., Bradham, R. R., and Chapman, W. W. [6]. 6. GREGORIE, H. B., JR., BRADHAM, R. R., and CHAPMAN, W. W. Adrenal cysts. Surgery, 52: 860, 1962. 7. ELLIS. F. H.. TR.. DAWE. C. T.. and CLAGETT. 0. T. Cysts of the adrenal piand:. ‘Ann. Surg., 136: 217,
1952. 8. ELLIOTT, G. B., WALKER, R. H., WRIGHT, A. S., and ELLIOTT, K. A. Adrenal giant cyst: hemagioma of medulla with osmotic pseudocyst formation. Ann.
Szug.,
159: 275, 1964.