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Cavernous Hemangioma of the Adrenal Gland
0022-5347/92/1471-0110$03.00/0 THE JOURNAL OF UROLOGY Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Vol. 147, 110-112, January 1992 Printed in U.S.A.
Case Reports CAVERNOUS HEMANGIOMA OF THE ADRENAL GLAND R. SALUP, R. FINEGOLD, D. BOROCHOVITZ, M. BOEHNKE AND M. POSNER From the Departments of Surgery, Pathology and Radiology, University of Pittsburgh, Montefiore University Hospital, Pittsburgh, Pennsylvania
ABSTRACT Cavernous hemangiomas of the adrenal gland are rare. We report on a patient with a left adrenal hemangioma successfully treated by radical excision. The literature pertaining to this interesting pathological condition is reviewed. KEY WORDS:
adrenal gland diseases; hemangioma, cavernous
Hemangiomas of the adrenal gland are rare. The first sur gically treated adrenal hemangioma was described in 1955. 1 Since then 12 cases have been reported. 2 We report a case of adrenal hemangioma unsuspected preoperatively. The clinical presentation and management are described, and the pertinent literature is reviewed. CASE REPORT
A 74-year-old black woman was hospitalized after an episode of syncope. On routine physical examination a large, palpable, left upper quadrant mass was noted. The medical history was unremarkable. All vital signs were normal. Laboratory values were all within normal limits. Urinalysis revealed microscopic hematuria and a urine culture was sterile. An abdominal plain film showed no calcifications and chest radiography was unremarkable. Upper abdominal sonography showed a normal right kidney, liver and gallbladder. There was a spherical left upper quadrant mass, approximately 15 cm. in diameter, above the upper pole of the left kidney. The mass appeared to be solid on sonography with a large hypoechoic central area. Computerized tomography (CT) confirmed the finding of a nonenhancing, noncystic, rounded, clearly demar cated, solid mass with a large central necrotic area displacing the left kidney inferiorly (fig. 1). Abdominal aortic and selective
FIG. 2. Selective left renal arteriogram does not indicate neovascu lar process.
FIG. 1. CT scan shows left upper quadrant mass originating from left adrenal gland. Accepted for publication May 17, 1991.
left renal arteriography showed a hypovascular left suprarenal mass with a vascular supply separate from that of the left renal pedicle (fig. 2). A small vascular blush appeared in the area of the superior pole of the left kidney. After mechanical and antibiotic bowel preparation the pa tient underwent exploratory laparotomy through a midline
110
CAVERNOUS HEMANGIOMA OF ADRENAL GLAND
inc1s10n o A large, left upper quadrant retrocolic tumor was found o The mass was densely adherent to the tail of the pan creas, spleen and upper pole of the left kidney o With adequate mobilization en bloc left adrenalectomy, distal pancreatectomy, splenectomy and left radical nephrectomy were performed o No enlarged regional lymph nodes or other abdominal masses were found o Convalescence was uneventful and the patient was dis charged from the hospital 17 days postoperativelyo The surgical specimen consisted of the left kidney, spleen and distal pancreas received en bloc o There was a large mass approximately 15 cm o in diameter at the upper pole of the left
111
kidney and displacing the spleen (figo 3)0 Although the mass was adherent to the kidney an easy plane of dissection could be developed between the mass, the kidney inferiorly and the spleen laterally o The mass was well circumscribed and showed a somewhat firm, white, fibrous, thickened walL The surface showed central replacement of the mass by friable, reddish granular material with focal areas of hemorrhageo Multiple sections prepared from the wall of the lesion showed a thick fibrous wall with a periphery of prominent cavernous vessels and central hemorrhage o The lining of these cavernous spaces was confirmed as endothelium by immunoperoxidase stain uniformly positive for factor VIII (figo 4, A)o Small islands of typical adrenocortical cells were identified in some of the pe ripheral sections o A small cavernous hemangioma was also detected grossly in the peripelvic fat of the left kidney and confirmed microscopically (fig o 4, B)o The spleen and pancreas showed no pathological changeo DISCUSSION
F!Go 30 Left adrenal and kidney show large well circumscribed mass completely replacing adrenal glando
Adrenal hemangiomas are rare tumors of unknown etiology, although some physicians suggest a possible relationship with prior traumai3-5 The first 7 cases reported were diagnosed at autopsy o 6 In 1955 Johnson and Jeppesen reported the first clinical case o 1 Since then 12 other clinical cases have been described, all with similar pathological features o 3-12 Histologi cally, hemangiomas are classified as cavernous, capillary, scle rosing or hemangiopericytoma o 13 Cavernous hemangiomas are uncommon and usually occur in children o They frequently are located in the skin and liver but they also can present in the cerebellum and eye grounds as part of the von Hippel-Lindau syndrome, in conjunction with cystic lesions of the pancreas o The term cavernous is used to describe the large vascular channels present in these neoplasms as opposed to the capillary type of hemangiomas o 12 Factor VIII immunoperoxidase stain demonstrates an endothelial cell lining of the cavernous space o Most adrenal hemangiomas are cavernouso Patient age ranged between 25 and 79 years in the reported cases with a strong predilection for women o The tumors ranged between 2 and 22 cm o in diameter with the majority being greater than 10 cm Bilateral involvement has been reported only twice o 6 There are no characteristic symptoms of adrenal hemangioma unless the tumor reaches a size large enough to exert pressure o The usual complaints are of abdominal discomfort, vague gastrointestinal symptoms and a palpable masso Changes in adrenal hormone production have not been described in patients with adrenal hemangioma in contrast to those with adrenal carcinoma and adenoma o Preoperative diagnosis fre quently is not adrenal hemangioma but instead a benign cyst of either pancreatic, omental, mesenteric or splenic origin o As
F!G o 4 0 A, factor VIII immunoperoxidase stain, counterstained with hematoxylino Note central necrosis with surrounding large cavernous vessels consistent with cavernous hemangioma o B, section of hilus of kidney shows separate cavernous hemangioma (H) closely approximating pelvis (P) of kidney o H & E, reduced from xlO o
112
KOIDE AND ASSOCIATES
a result of improved radiological imaging methods adrenal hemangiomas, historically identified post mortem, are currently frequently diagnosed clinically. Plain abdominal films showing a curved ring of calcification3 • may lead to the impression of an adrenal pseudocyst. However, 30% of all adrenal carcinomas show calcifications, and they also are seen occasionally in cases of pheochromocytoma, Addison's disease and adrenal adenoma. Abdominal sonography usually shows a large mass with a heterogeneous, nonspecific structural pattern. 1 An unenhanced CT scan occasionally demonstrates a calcified lesion with a hypodense center and a thick periphery. 1 Only the peripheral dense area enhances markedly upon injection of contrast ma terial,11 while the central necrotic area is hypoperfused as in our patient. Angiography usually will show extensive neovas cularity within the mass6 but depending on the amount of necrosis it may be nondiagnostic. Patients can also have asso ciated hemangiomas in other intraabdominal organs as noted in our patient. Hemangiomas of the liver, ovaries or uterus have previously been described. 4 Finally, 2 cases of malignant adrenal hemangioblastoma have been described. 3 Because current visualization methods lack specificity, all solid or partially cystic adrenal masses should be considered malignant until proved otherwise by tissue di agnosis. Therefore, the treatment of choice for adrenal heman gioma is surgical excision through a transperitoneal approach. Adrenalectomy with clear tumor margins or, if necessary, en bloc resection with partial or total excision of involved adjacent organs is the goal at laparotomy. 4
2
2
REFERENCES
L Johnson, C, C. and Jeppesen, F. B,: Hemangioma of the adrenal. J, UroL, 74: 573, 1955. 2. Travis, W. D,, Oertel, J. E, and Lack, E. E.: Miscellaneous tumors and tumefactive lesions of the adrenal gland. In: Pathology of the Adrenal Glands. Edited by E, E. Lack. New York: Churchill Livingstone, pp. 362-364, 1990. 3. Weiss, J. M. and Schulte, J. W.: Adrenal hemangioma: a case report. J, UroL, 95: 604, 1966, 4. Chodoff, RJ., Smith, J, W. and Hering, N.: Cavernous heman gioma of the adrenal gland. Amer. J. Surg., 111: 595, 1966. 5. Goren, E., Bensal, D., Reif, R M. and Eidelman, A.: Cavernous hemangioma of the adrenal gland. J. UroL, 135: 341, 1986. 6, Vargas, A. D.: Adrenal hemangioma, Urology, 16: 389, 1980, 7. Nagatani, M., Harada, T. and Takeda, T,: Case of successfully removed giant hemangioma of the adrenal gland.. Naika, 24: 383, 1969.- 8. Ruebel, A. A.: Adrenal hemangioma. Urology, 2: 289, 1973. 9, Rothberg, M., Bastidas, J., Mattey, W. E. and Bernas, E.: Adrenal hemangiomas: angiographic appearance of a rare tumor. Radiol ogy, 126: 341, 1978, 10. Zeman, V. and Hruby, J.: Cavernous hemangioma of the adrenal gland, RozhL Chir., 58: 195, 1979, 11. Orringer, RD,, Lynch, J. A. and McDermott, W. V.: Cavernous hemangioma of the cdrenal gland. J. Surg, OncoL, 22: 106, 1983, 12. Derchi, L. E., Rapaccini, G. L., Banderali, A., Danza, F. M. and Grillo, F.: Ultrasound and CT findings in two cases of heman gioma of the adrenal gland. J. Comput, Assist. Tomogr,, 13: 659, 1989, 13. Sisson, J. A.: General Pathology, Philadelphia: J. B. Lippincott Co., 1974.
0022-5347/92/1471-0112$03.00/0 THE JOURNAL OF UROLOGY Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Vol. 147, 112-114, January 1992
Printed in U.S.A.
A STRATEGY OF CYSTINE STONE MANAGEMENT TAKUO KOIDE, TOSHIAKI YOSHIOKA, SEIJI YAMAGUCHI, MASATO UTSUNOMIYA AND TAKAO SONODA From the Department of Urology, Osaka University School of Medicine, Osaka, Japan
ABSTRACT
Simple mechanical disintegration of cystine calculi by extracorporeal shock wave lithotripsy and/ or percutaneous nephrolithotomy is being performed widely around the world. However, it cannot be denied that the cystine calculus is one of the most difficult stones for mechanical disintegration. We previously reported the oral medical chemolysis of cystine calculi in 1982 and a third of the patients became stone-free. In another third of the patients the cystine component was replaced by apatite during the medical treatment and apatite stones formed in this manner are easily disinte grated. In view of the complications of mechanical disintegration, oral medical treatment for chemolysis should be recommended before simple destruction of cystine calculi. KEY WORDS: urinary calculi, extracorporeal shockwave lithotripsy, cystine
Since extracorporeal shock wave lithotripsy (ESWL*) and percutaneous techniques of stone destruction are widely per formed worldwide, most renal stones are treated successfully by ESWL with or without endourological techniques. The cystine calculus, however, is hard and is one of the most difficult stones for mechanical disintegration. 1• In the case of ESWL monotherapy for cystine calculus more than 2 to 3 times the usual shocks are needed to destroy the calculus even when the high power ESWL machine is used. 2 Endourological tech niques, such as percutaneous nephrolithotomy, often require preoperative or postoperative ESWL for problem stones,1 such as cystine calculi. Moreover, direct disintegration of cystine 2
Accepted for publication May 17, 1991. * Dornier Medical Systems, Inc,, Marietta, Georgia,
calculi by percutaneous nephrolithotomy is not easy because of the hardness of the stone and the tendency for staghorn stones. 3 For management of cystine calculi several medical treat ments have been proposed. We previously reported the effec tiveness of medical treatment with a-mercaptopropionylglycine (a-MPG) on stone prophylaxis and stone dissolution. 4 Further more, if a-MPG therapy fails to achieve complete stone disso lution the stone composition often changes from cystine to apatite, which is much more fragile than cystine for mechanical disintegration. We present 3 cases whose cystine stones were replaced by apatite and those stones were disintegrated without difficulty. We recommend medical treatment of cystine calculi before the decision is made for simple mechanical stone disintegration.
Vol. 147, 110-112, January 1992 Printed in U.S.A.
Case Reports CAVERNOUS HEMANGIOMA OF THE ADRENAL GLAND R. SALUP, R. FINEGOLD, D. BOROCHOVITZ, M. BOEHNKE AND M. POSNER From the Departments of Surgery, Pathology and Radiology, University of Pittsburgh, Montefiore University Hospital, Pittsburgh, Pennsylvania
ABSTRACT Cavernous hemangiomas of the adrenal gland are rare. We report on a patient with a left adrenal hemangioma successfully treated by radical excision. The literature pertaining to this interesting pathological condition is reviewed. KEY WORDS:
adrenal gland diseases; hemangioma, cavernous
Hemangiomas of the adrenal gland are rare. The first sur gically treated adrenal hemangioma was described in 1955. 1 Since then 12 cases have been reported. 2 We report a case of adrenal hemangioma unsuspected preoperatively. The clinical presentation and management are described, and the pertinent literature is reviewed. CASE REPORT
A 74-year-old black woman was hospitalized after an episode of syncope. On routine physical examination a large, palpable, left upper quadrant mass was noted. The medical history was unremarkable. All vital signs were normal. Laboratory values were all within normal limits. Urinalysis revealed microscopic hematuria and a urine culture was sterile. An abdominal plain film showed no calcifications and chest radiography was unremarkable. Upper abdominal sonography showed a normal right kidney, liver and gallbladder. There was a spherical left upper quadrant mass, approximately 15 cm. in diameter, above the upper pole of the left kidney. The mass appeared to be solid on sonography with a large hypoechoic central area. Computerized tomography (CT) confirmed the finding of a nonenhancing, noncystic, rounded, clearly demar cated, solid mass with a large central necrotic area displacing the left kidney inferiorly (fig. 1). Abdominal aortic and selective
FIG. 2. Selective left renal arteriogram does not indicate neovascu lar process.
FIG. 1. CT scan shows left upper quadrant mass originating from left adrenal gland. Accepted for publication May 17, 1991.
left renal arteriography showed a hypovascular left suprarenal mass with a vascular supply separate from that of the left renal pedicle (fig. 2). A small vascular blush appeared in the area of the superior pole of the left kidney. After mechanical and antibiotic bowel preparation the pa tient underwent exploratory laparotomy through a midline
110
CAVERNOUS HEMANGIOMA OF ADRENAL GLAND
inc1s10n o A large, left upper quadrant retrocolic tumor was found o The mass was densely adherent to the tail of the pan creas, spleen and upper pole of the left kidney o With adequate mobilization en bloc left adrenalectomy, distal pancreatectomy, splenectomy and left radical nephrectomy were performed o No enlarged regional lymph nodes or other abdominal masses were found o Convalescence was uneventful and the patient was dis charged from the hospital 17 days postoperativelyo The surgical specimen consisted of the left kidney, spleen and distal pancreas received en bloc o There was a large mass approximately 15 cm o in diameter at the upper pole of the left
111
kidney and displacing the spleen (figo 3)0 Although the mass was adherent to the kidney an easy plane of dissection could be developed between the mass, the kidney inferiorly and the spleen laterally o The mass was well circumscribed and showed a somewhat firm, white, fibrous, thickened walL The surface showed central replacement of the mass by friable, reddish granular material with focal areas of hemorrhageo Multiple sections prepared from the wall of the lesion showed a thick fibrous wall with a periphery of prominent cavernous vessels and central hemorrhage o The lining of these cavernous spaces was confirmed as endothelium by immunoperoxidase stain uniformly positive for factor VIII (figo 4, A)o Small islands of typical adrenocortical cells were identified in some of the pe ripheral sections o A small cavernous hemangioma was also detected grossly in the peripelvic fat of the left kidney and confirmed microscopically (fig o 4, B)o The spleen and pancreas showed no pathological changeo DISCUSSION
F!Go 30 Left adrenal and kidney show large well circumscribed mass completely replacing adrenal glando
Adrenal hemangiomas are rare tumors of unknown etiology, although some physicians suggest a possible relationship with prior traumai3-5 The first 7 cases reported were diagnosed at autopsy o 6 In 1955 Johnson and Jeppesen reported the first clinical case o 1 Since then 12 other clinical cases have been described, all with similar pathological features o 3-12 Histologi cally, hemangiomas are classified as cavernous, capillary, scle rosing or hemangiopericytoma o 13 Cavernous hemangiomas are uncommon and usually occur in children o They frequently are located in the skin and liver but they also can present in the cerebellum and eye grounds as part of the von Hippel-Lindau syndrome, in conjunction with cystic lesions of the pancreas o The term cavernous is used to describe the large vascular channels present in these neoplasms as opposed to the capillary type of hemangiomas o 12 Factor VIII immunoperoxidase stain demonstrates an endothelial cell lining of the cavernous space o Most adrenal hemangiomas are cavernouso Patient age ranged between 25 and 79 years in the reported cases with a strong predilection for women o The tumors ranged between 2 and 22 cm o in diameter with the majority being greater than 10 cm Bilateral involvement has been reported only twice o 6 There are no characteristic symptoms of adrenal hemangioma unless the tumor reaches a size large enough to exert pressure o The usual complaints are of abdominal discomfort, vague gastrointestinal symptoms and a palpable masso Changes in adrenal hormone production have not been described in patients with adrenal hemangioma in contrast to those with adrenal carcinoma and adenoma o Preoperative diagnosis fre quently is not adrenal hemangioma but instead a benign cyst of either pancreatic, omental, mesenteric or splenic origin o As
F!G o 4 0 A, factor VIII immunoperoxidase stain, counterstained with hematoxylino Note central necrosis with surrounding large cavernous vessels consistent with cavernous hemangioma o B, section of hilus of kidney shows separate cavernous hemangioma (H) closely approximating pelvis (P) of kidney o H & E, reduced from xlO o
112
KOIDE AND ASSOCIATES
a result of improved radiological imaging methods adrenal hemangiomas, historically identified post mortem, are currently frequently diagnosed clinically. Plain abdominal films showing a curved ring of calcification3 • may lead to the impression of an adrenal pseudocyst. However, 30% of all adrenal carcinomas show calcifications, and they also are seen occasionally in cases of pheochromocytoma, Addison's disease and adrenal adenoma. Abdominal sonography usually shows a large mass with a heterogeneous, nonspecific structural pattern. 1 An unenhanced CT scan occasionally demonstrates a calcified lesion with a hypodense center and a thick periphery. 1 Only the peripheral dense area enhances markedly upon injection of contrast ma terial,11 while the central necrotic area is hypoperfused as in our patient. Angiography usually will show extensive neovas cularity within the mass6 but depending on the amount of necrosis it may be nondiagnostic. Patients can also have asso ciated hemangiomas in other intraabdominal organs as noted in our patient. Hemangiomas of the liver, ovaries or uterus have previously been described. 4 Finally, 2 cases of malignant adrenal hemangioblastoma have been described. 3 Because current visualization methods lack specificity, all solid or partially cystic adrenal masses should be considered malignant until proved otherwise by tissue di agnosis. Therefore, the treatment of choice for adrenal heman gioma is surgical excision through a transperitoneal approach. Adrenalectomy with clear tumor margins or, if necessary, en bloc resection with partial or total excision of involved adjacent organs is the goal at laparotomy. 4
2
2
REFERENCES
L Johnson, C, C. and Jeppesen, F. B,: Hemangioma of the adrenal. J, UroL, 74: 573, 1955. 2. Travis, W. D,, Oertel, J. E, and Lack, E. E.: Miscellaneous tumors and tumefactive lesions of the adrenal gland. In: Pathology of the Adrenal Glands. Edited by E, E. Lack. New York: Churchill Livingstone, pp. 362-364, 1990. 3. Weiss, J. M. and Schulte, J. W.: Adrenal hemangioma: a case report. J, UroL, 95: 604, 1966, 4. Chodoff, RJ., Smith, J, W. and Hering, N.: Cavernous heman gioma of the adrenal gland. Amer. J. Surg., 111: 595, 1966. 5. Goren, E., Bensal, D., Reif, R M. and Eidelman, A.: Cavernous hemangioma of the adrenal gland. J. UroL, 135: 341, 1986. 6, Vargas, A. D.: Adrenal hemangioma, Urology, 16: 389, 1980, 7. Nagatani, M., Harada, T. and Takeda, T,: Case of successfully removed giant hemangioma of the adrenal gland.. Naika, 24: 383, 1969.- 8. Ruebel, A. A.: Adrenal hemangioma. Urology, 2: 289, 1973. 9, Rothberg, M., Bastidas, J., Mattey, W. E. and Bernas, E.: Adrenal hemangiomas: angiographic appearance of a rare tumor. Radiol ogy, 126: 341, 1978, 10. Zeman, V. and Hruby, J.: Cavernous hemangioma of the adrenal gland, RozhL Chir., 58: 195, 1979, 11. Orringer, RD,, Lynch, J. A. and McDermott, W. V.: Cavernous hemangioma of the cdrenal gland. J. Surg, OncoL, 22: 106, 1983, 12. Derchi, L. E., Rapaccini, G. L., Banderali, A., Danza, F. M. and Grillo, F.: Ultrasound and CT findings in two cases of heman gioma of the adrenal gland. J. Comput, Assist. Tomogr,, 13: 659, 1989, 13. Sisson, J. A.: General Pathology, Philadelphia: J. B. Lippincott Co., 1974.
0022-5347/92/1471-0112$03.00/0 THE JOURNAL OF UROLOGY Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Vol. 147, 112-114, January 1992
Printed in U.S.A.
A STRATEGY OF CYSTINE STONE MANAGEMENT TAKUO KOIDE, TOSHIAKI YOSHIOKA, SEIJI YAMAGUCHI, MASATO UTSUNOMIYA AND TAKAO SONODA From the Department of Urology, Osaka University School of Medicine, Osaka, Japan
ABSTRACT
Simple mechanical disintegration of cystine calculi by extracorporeal shock wave lithotripsy and/ or percutaneous nephrolithotomy is being performed widely around the world. However, it cannot be denied that the cystine calculus is one of the most difficult stones for mechanical disintegration. We previously reported the oral medical chemolysis of cystine calculi in 1982 and a third of the patients became stone-free. In another third of the patients the cystine component was replaced by apatite during the medical treatment and apatite stones formed in this manner are easily disinte grated. In view of the complications of mechanical disintegration, oral medical treatment for chemolysis should be recommended before simple destruction of cystine calculi. KEY WORDS: urinary calculi, extracorporeal shockwave lithotripsy, cystine
Since extracorporeal shock wave lithotripsy (ESWL*) and percutaneous techniques of stone destruction are widely per formed worldwide, most renal stones are treated successfully by ESWL with or without endourological techniques. The cystine calculus, however, is hard and is one of the most difficult stones for mechanical disintegration. 1• In the case of ESWL monotherapy for cystine calculus more than 2 to 3 times the usual shocks are needed to destroy the calculus even when the high power ESWL machine is used. 2 Endourological tech niques, such as percutaneous nephrolithotomy, often require preoperative or postoperative ESWL for problem stones,1 such as cystine calculi. Moreover, direct disintegration of cystine 2
Accepted for publication May 17, 1991. * Dornier Medical Systems, Inc,, Marietta, Georgia,
calculi by percutaneous nephrolithotomy is not easy because of the hardness of the stone and the tendency for staghorn stones. 3 For management of cystine calculi several medical treat ments have been proposed. We previously reported the effec tiveness of medical treatment with a-mercaptopropionylglycine (a-MPG) on stone prophylaxis and stone dissolution. 4 Further more, if a-MPG therapy fails to achieve complete stone disso lution the stone composition often changes from cystine to apatite, which is much more fragile than cystine for mechanical disintegration. We present 3 cases whose cystine stones were replaced by apatite and those stones were disintegrated without difficulty. We recommend medical treatment of cystine calculi before the decision is made for simple mechanical stone disintegration.