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Intrarenal pheochromocytoma Preoperative angiographic diagnosis
INTRARENAL Preoperative LESLIE RICHARD BRUNON HOWARD
PHEOCHROMOCYTOMA Angiographic
PREGER,
F.R.C.R.,
E. GARDNER, 0.
KAWALA,
L. STEINBACH,
Diagnosis
F.F.R.R.C.S.
(Irel.)
M.D. M.B. M.D.
From the Departments of Radiology and Surgery, University of California School of Medicine, and French Hospital, and the Department of Internal Medicine, French Hospital, San Francisco, California
findings in a - This report documents the angiographic, urographic, and tomographic patient with a pheochromocytoma within the renal capsule and separate jkom a normal adrenal gland.
ABSTRACT
A fifty-one-year-old woman who had had severe hypertension for many years experienced a sudden episode of interscapular pain. Chest radiographs indicated a dissecting aneurysm of the descending aorta. The blood pressure was reduced with reserpine, and aortography ordered as an aid to surgical treatment of the aortic dissection. Urine samples were collected for assay of vanillylmandelic acid and catecholamines. A technical mishap made the urine assay impossible. The proximal portion of the left ureter showed notching, despite symmetry of renal lengths and excretion rates (Fig. 1A). The absence of the S-o’clock calyx of the left kidney was disregarded. On the tomograms a cyst was noted at the lower left edge of the left kidney (Fig. 1B). Tomograms also showed considerable peri-infundibular fat replacement. Because of the long-standing hypertension, selective renal arteriography was performed prior to completion of an arch aortogram. The right renal and adrenal vasculature was normal. A vascular mass was seen in the lower half of the left kidney (Fig. 2A). It opacified from the intrarenal branches of the renal artery and from a perirenal artery that had a tortuous course along the inferior hilus of the kidney. Although a suprarenal gland was identified at the upper pole of the left kidney
194
on the tomogram, opacification of an inferior adrenal artery was not seen on the selective left renal arteriogram. The arteries within the mass were beaded. Despite the marked hypervascularity of the mass, no early venous shunting was seen. During the venous phase the peripheral cyst was better seen, the mass appeared almost homogeneously opacified, and two large venous structures were noted (arrowheads, Fig. 2B). The more medial structure was a gonadal vein, the other was an abnormal vessel, apparently draining the upper part of the mass. It is this latter vessel that lay adjacent to the notched ureter and was presumed the cause of the notching. Biplane views confirmed the presence of a dissection beginning at the left subclavian artery and terminating at the origin of the celiac artery. Ultrasonography was performed after the arteriogram possibly justifying the appellation A. 0. K. (Academic Over-kill) syndrome. The area of the pheochromocytoma was mainly echo-free, despite its solid nature at the gain-setting used. The mass merged into the large peripheral cyst, which partly overlaid it laterally. The left adrenal gland and kidney were resected. The adrenal gland was found to be normal in size (6 by 0.9 by 1.2 cm.) and position, with a thin cortex and abundant fat. Its medullary tissue was
UROLOGY
/ AUGUST 1976 I
VOLUME
VIII,
NUMBER
2
FIGURE 1. (A) Excretory urogram at ten Jnitryltes. Beading of proximal portion of lc>ffuretcr-irir,/.ori,lir,cl~il isseen; S-o’clock calyx is not opacijed but it was fot1n(.1 normal in surgical specimen. (B) Tomogram rnatlc clirt-inc:c i i:retory urography showing normal-sized left adrc~nal Large peripheral cyst is visible between 3- and iSo‘, iock 1
FIGURE 2. Selective renal arteriogram. (A) A i-terial phase demonstrates hypervascular mass in lower ha‘ofleft kidney. Mass is fed directly by major intrarenal branches and also by smaller perirenal branch arisingfrorN( below there is no early venous shunting. (B ) \enous h&s. Vessels in tumor are beaded, but despiti, hypervascularity phase shows large gonadal vein (vnedial arrowlrrad) and a large tortuous vein (lateral arrowhead) which tl .iin the tumor. Presumably, this latter vein has cause( I’ureter-al notching.
UROLOGY
/ AUGUST1976
/ VOLUME
VIII,KlrMBER 2
195
unremarkable. Contained within the capsule of the left kidney was a 3.5-cm. yellow-brown lobulated tumor with a central, blue-gray, fibrous area, 1.5 cm. in diameter. In addition to the cyst seen radiographically were several smaller cysts. A normal calyx was present at the 5-o’clock position in the left kidney, despite the planographic The tumor was diagnosed as a appearance. pheochromocytoma during surgery (at the time of “frozen section”) and later by definitive examination. Seven months after surgery the patient was normotensive and without medication. Surgical treatment of the dissecting thoracic aortic aneurysm was not deemed necessary. Comment The mass seen on the selective renal arteriogram was diagnosed preoperatively as either an intrarenal pheochromocytoma or dermoid tumor on the basis of the beaded vessels and hypervascularity, but absence of early venous shunting. If early venous shunting had been present, a malignant tumor would have been more likely. It is known that a pheochromocytoma can arise outside the adrenals from chromafhn tissue1’2 and that adrenal glands themselves may lie within the renal capsule. 3 More frequently a left pheochromocytoma may lie over the left kidney and have some connection with a normally placed left adrenal gland.4 Our patient was unusual in that a
196
normal left adrenal gland was present, separate from the tumor. Therefore, we are not dealing with the condition of adrenal-renal heterotopia a condition that can be complete or partial, and is always bilateral. Ifthe heterotopia is complete, no medullary cells are found in the subcapsular adrenal, which consists of thin cortical tissue only.5 In our patient the normally sited left adrenal gland contained normal medullary cells. Department of Radiology, 380-M University of California School of Medicine San Francisco, California 94143 (DR. PREGER) ACKNOWLEDGMENT.
Beiers, M.D., studies.
To G.
A. Watson,M.D., andR. L.
who performed
the surgical,
histologic
References 1. FRIES, J. G., and CHAMBERLIN, J. A.: Extra-adrenal pheochromocytoma: literature review and report of a cervical pheochromocytoma, Surgery 63: 268 (1968). 2. STACKPOLE, R. H., MELICOW, M. M., and USON, A. c.: Pheochromocytoma in children: report of 9 cases and review of the first 190 published cases with follow-up studies, J. Pediatr. 63: 315 (1963). 3. PENGELLY, C. D. R.: Phaeochromocytoma within the renal capsule, Br. Med. J. 2: 477 (1959). 4. SHRAGO, G. G., MCKINNON, C., and CLARK, R.: Adrenal tumors simulating intrarenal lesions, Am. J. Roentgenol. 121: 518 (1974). 5. O’CRO~LEY, C. R., and MARTLAND, H. S.: Adrenal heterotopia, rests, and the so-called Grawitz tumor, J. Urol. 50: 756 (1943).
UROLOGY /
AUGUST 1976 / VOLUME VIII. NUMBER 2
PHEOCHROMOCYTOMA Angiographic
PREGER,
F.R.C.R.,
E. GARDNER, 0.
KAWALA,
L. STEINBACH,
Diagnosis
F.F.R.R.C.S.
(Irel.)
M.D. M.B. M.D.
From the Departments of Radiology and Surgery, University of California School of Medicine, and French Hospital, and the Department of Internal Medicine, French Hospital, San Francisco, California
findings in a - This report documents the angiographic, urographic, and tomographic patient with a pheochromocytoma within the renal capsule and separate jkom a normal adrenal gland.
ABSTRACT
A fifty-one-year-old woman who had had severe hypertension for many years experienced a sudden episode of interscapular pain. Chest radiographs indicated a dissecting aneurysm of the descending aorta. The blood pressure was reduced with reserpine, and aortography ordered as an aid to surgical treatment of the aortic dissection. Urine samples were collected for assay of vanillylmandelic acid and catecholamines. A technical mishap made the urine assay impossible. The proximal portion of the left ureter showed notching, despite symmetry of renal lengths and excretion rates (Fig. 1A). The absence of the S-o’clock calyx of the left kidney was disregarded. On the tomograms a cyst was noted at the lower left edge of the left kidney (Fig. 1B). Tomograms also showed considerable peri-infundibular fat replacement. Because of the long-standing hypertension, selective renal arteriography was performed prior to completion of an arch aortogram. The right renal and adrenal vasculature was normal. A vascular mass was seen in the lower half of the left kidney (Fig. 2A). It opacified from the intrarenal branches of the renal artery and from a perirenal artery that had a tortuous course along the inferior hilus of the kidney. Although a suprarenal gland was identified at the upper pole of the left kidney
194
on the tomogram, opacification of an inferior adrenal artery was not seen on the selective left renal arteriogram. The arteries within the mass were beaded. Despite the marked hypervascularity of the mass, no early venous shunting was seen. During the venous phase the peripheral cyst was better seen, the mass appeared almost homogeneously opacified, and two large venous structures were noted (arrowheads, Fig. 2B). The more medial structure was a gonadal vein, the other was an abnormal vessel, apparently draining the upper part of the mass. It is this latter vessel that lay adjacent to the notched ureter and was presumed the cause of the notching. Biplane views confirmed the presence of a dissection beginning at the left subclavian artery and terminating at the origin of the celiac artery. Ultrasonography was performed after the arteriogram possibly justifying the appellation A. 0. K. (Academic Over-kill) syndrome. The area of the pheochromocytoma was mainly echo-free, despite its solid nature at the gain-setting used. The mass merged into the large peripheral cyst, which partly overlaid it laterally. The left adrenal gland and kidney were resected. The adrenal gland was found to be normal in size (6 by 0.9 by 1.2 cm.) and position, with a thin cortex and abundant fat. Its medullary tissue was
UROLOGY
/ AUGUST 1976 I
VOLUME
VIII,
NUMBER
2
FIGURE 1. (A) Excretory urogram at ten Jnitryltes. Beading of proximal portion of lc>ffuretcr-irir,/.ori,lir,cl~il isseen; S-o’clock calyx is not opacijed but it was fot1n(.1 normal in surgical specimen. (B) Tomogram rnatlc clirt-inc:c i i:retory urography showing normal-sized left adrc~nal Large peripheral cyst is visible between 3- and iSo‘, iock 1
FIGURE 2. Selective renal arteriogram. (A) A i-terial phase demonstrates hypervascular mass in lower ha‘ofleft kidney. Mass is fed directly by major intrarenal branches and also by smaller perirenal branch arisingfrorN( below there is no early venous shunting. (B ) \enous h&s. Vessels in tumor are beaded, but despiti, hypervascularity phase shows large gonadal vein (vnedial arrowlrrad) and a large tortuous vein (lateral arrowhead) which tl .iin the tumor. Presumably, this latter vein has cause( I’ureter-al notching.
UROLOGY
/ AUGUST1976
/ VOLUME
VIII,KlrMBER 2
195
unremarkable. Contained within the capsule of the left kidney was a 3.5-cm. yellow-brown lobulated tumor with a central, blue-gray, fibrous area, 1.5 cm. in diameter. In addition to the cyst seen radiographically were several smaller cysts. A normal calyx was present at the 5-o’clock position in the left kidney, despite the planographic The tumor was diagnosed as a appearance. pheochromocytoma during surgery (at the time of “frozen section”) and later by definitive examination. Seven months after surgery the patient was normotensive and without medication. Surgical treatment of the dissecting thoracic aortic aneurysm was not deemed necessary. Comment The mass seen on the selective renal arteriogram was diagnosed preoperatively as either an intrarenal pheochromocytoma or dermoid tumor on the basis of the beaded vessels and hypervascularity, but absence of early venous shunting. If early venous shunting had been present, a malignant tumor would have been more likely. It is known that a pheochromocytoma can arise outside the adrenals from chromafhn tissue1’2 and that adrenal glands themselves may lie within the renal capsule. 3 More frequently a left pheochromocytoma may lie over the left kidney and have some connection with a normally placed left adrenal gland.4 Our patient was unusual in that a
196
normal left adrenal gland was present, separate from the tumor. Therefore, we are not dealing with the condition of adrenal-renal heterotopia a condition that can be complete or partial, and is always bilateral. Ifthe heterotopia is complete, no medullary cells are found in the subcapsular adrenal, which consists of thin cortical tissue only.5 In our patient the normally sited left adrenal gland contained normal medullary cells. Department of Radiology, 380-M University of California School of Medicine San Francisco, California 94143 (DR. PREGER) ACKNOWLEDGMENT.
Beiers, M.D., studies.
To G.
A. Watson,M.D., andR. L.
who performed
the surgical,
histologic
References 1. FRIES, J. G., and CHAMBERLIN, J. A.: Extra-adrenal pheochromocytoma: literature review and report of a cervical pheochromocytoma, Surgery 63: 268 (1968). 2. STACKPOLE, R. H., MELICOW, M. M., and USON, A. c.: Pheochromocytoma in children: report of 9 cases and review of the first 190 published cases with follow-up studies, J. Pediatr. 63: 315 (1963). 3. PENGELLY, C. D. R.: Phaeochromocytoma within the renal capsule, Br. Med. J. 2: 477 (1959). 4. SHRAGO, G. G., MCKINNON, C., and CLARK, R.: Adrenal tumors simulating intrarenal lesions, Am. J. Roentgenol. 121: 518 (1974). 5. O’CRO~LEY, C. R., and MARTLAND, H. S.: Adrenal heterotopia, rests, and the so-called Grawitz tumor, J. Urol. 50: 756 (1943).
UROLOGY /
AUGUST 1976 / VOLUME VIII. NUMBER 2