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Angiomyelglipoma Associated with Bilateral Adrenocortical Hyperplasia and Hypertension
0022-E:3£17 /85/1334-0655$02,00/0
Vol. 133,
THE JOURNAL OF TJRGLOGY
Copyright© 1985 by The \/Villiarn.s & 'vVilkins Co,
Printed in
ANGIOMYELOLIPOIVIA ASSOCIATED WITH BILATERAL ADRENOCORTICAL HYPERPLASIA AND HYPERTENSION F. ESCUIN, P. GOMEZ, I. MARTINEZ, M. PEREZ-FONTAN, R SELGAS
AND
L. SANCHEZ-SICILIA
From the Nephrology Service, Ciudad Sanitaria La Paz, Medical School of Uniuersidad Autonoma of Madrid, Madrid, Spain
ABSTRACT
We report a case of adrenal myelolipoma associated with adrenocortical hyperplasia and hypertension. Although several endocrine derangements have been described in association with myelolipomas, we have found no reports of its association with adrenocortical hyperplasia. Myelolipoma is a benign neoplasm of the adrenal gland that is constituted by fatty and hematopoietic tissue. 1 First described in 1905 by Gierke 2 myelolipoma received its present designation in 1929 from Oberling. 3 It is usually a necropsy finding and creates no problems owing to its slight growth rate,4 for which few cases have required surgical intervention. An operation is performed only when excessive tumor growth mimics other pathological conditions, such as calcified adrenal cysts,5 retroperitoneal malignancy, 6 macroscopic hematuria, 7 acute appendicitis 8 or even a casual finding. 9 We report a case of myelolipoma associated with adrenocortical hyperplasia, severe
satisfactorily with 400 mg. spironolactone daily. An abdominal computerized tomography (CT) scan revealed only a slight increase in the size of the right adrenal gland that was not considered significant. An adrenal scan with iodocholesterol showed an increased uptake of the tracer in the right adrenal gland, with no evidence of suppression bilaterally after the administration of dexamethasone (fig. 1). Exploratory laparotomy and right adrenalectomy were performed. The patient remained hypertensive postoperatively and spironolactone controlled the blood pressure. The surgical specimen labeled right adrenal gland was 5 x 4
FIG. l. Iodocholesterol adrenal scan. A, bilatem! uptake with more intensity on right side. B, no evidence of suppression after administration of dexamethasone.
hypertension and biochemical features of steronism.
hyperaldo-
CASE REPORT
A 12-year-old white girl was referred to our hospital for evaluation of severe hypertension. Blood pressure was 170/130 mm. Hg and serum potassium was 2.5 mmol./1. without diuretic therapy. Catecholamines and vanillyl mandelic acid in a 24hour urine specimen were normal. Excretory urography was normal. Plasma renin activity was markedly suppressed under basal conditions and after 3 hours of ambulation (see table). Plasma and urine (14 µ,g. per 24 hours, normal 5.5 to 13) aldosterone levels were high. The blood pressure was controlled Accepted for publication December 3, 1984. 655
X 4 cm. in size. The macroscopic sections showed that most of the parenchyma was replaced by hematic material. A solid structure with abundant fatty tissue and abundant islets of extramedullary hematopoiesis was noted microscopically and interspersed with the hemorrhagic zone (fig. 2). Hyperplasia of the adrenal cortex was demonstrated primarily at the zona glomerulosa.
DISCUSSION
Myelolipomas usually are necropsy findings, with a reported incidence ranging from 0.087 to 0.2 per cent. 10 The etiology of these neoplasms is still obscure, and various hypotheses of the pathogenesis include bone marrow embolism,4 extramedullary hematopoiesis 11 and bone marrow metaplasia. 4 •12 The tumor is associated frequently with hypertension. 6 •7 • 13
656
ESCUIN AND ASSOCIATES
FIG. 2. Adrenal myelolipoma. A, adipose tissue and hematopoietic islets occupy adrenal medulla. H & E, reduced from X25. B, glomerular type adrenal cortex cells in relation to osseous trabecula and hematopoietic islets. Preoperative and postoperative plasma renin and aldosterone levels during 100 mM. sodium diet Plasma renin activity (ng./ml./hr.): Basal After 3 hrs. ambulation Plasma aldosterone (pg./ml.): Basal After 3 hrs. ambulation
Preop.
Postop.
0.066 0.089
0.092 0.120
259 223
212 228
Normal Range 0.62 ± 0.13 2.40 ± 0.27 67.0 ± 12.5 184 ± 28
Plaut reported associated endocrine derangements in 9 of 100 patients with myelolipoma, including Addison's disease, Cushing's disease, hermaphroditism, obesity, virilism and multiple endocrinopathies. 4 In our patient, who to our knowledge is the youngest subject to be described, the tumor was associated with adrenocortical hyperplasia, hypertension and biochemical data suggesting primary hyperaldosteronism.
REFERENCES
1. Dyckman, J. and Freedman, D.: Myelolipoma of the adrenal with clinical features and surgical excision. J. Mt. Sinai Hosp., 24: 793, 1957. 2. Gierke, E.: Uber Knochenmarksgewebe in der Nebennierre. Beitr. Path. Anat., suppl., 7: 311, 1905. 3. Oberling, G.: Les formations myelo-lipomateuses. Bull. Cancer (Paris), 18: 234, 1929. 4. Plaut, A.: Myelolipoma in the adrenal cortex (myeloadipose structures). Amer. J. Path., 34: 487, 1958. 5. Whittaker, L. D.: Myelolipoma of the adrenal gland. Surgical removal. Arch. Surg., 97: 628, 1968. 6. Fernandez-Sanz, J., Galera, H., Garcia-Donas, A., GonzalezCampora, R., Llamas, R. and Matilla, A.: Adrenal myelolipoma simulating a retroperitoneal malignant neoplasm. J. Urol., 126: 780, 1981. 7. Olsson, C. A., Krane, R. J., Klugo, R. C. and Selikowitz, S. M.: Adrenal myelolipoma. Surgery, 73: 665, 1973.
ANGIOMYELOUPOMA, ADRENOCORTICAL HYPERPLASIA AND HYPERTENSION 80 Ishika-\iv.a I-I., Tachibanai JVL, Hata, IVL, Tazaki, H.i ~"''""u~,,, and Iri, H.: Myelolipoma of the adrenal gland. J. Urol., 777, 1981. 9. Braendstrup, 0., Gerdes, U., Lange, J. and Str0m, P. F.: Myelolipoma of the adrenal gland. Scand. J. Urol. Nephrol., 15: 343, 1981. 10. McDonnel, W. V.: Myelolipoma of the adrenal. Arch. Path., 61: 1
657
416, 1956. 11. Holliday, T. D. S.: Massive bone-marrow heterotopia in both adrenal glands. J. Path. Bact., 70: 239, 1955. 12. Nicod, J. L.: Adenoma, lipome et myelolipome de la cortico-surrenale. Bull. Ass. Franc Cancer, 50: 109, 1963. 13. Tulcinsky, D. E., Deutsch, V. and Bubis, J. J.: Myelolipoma of the adrenal gland. Brit. J. Surg., 57: 465, 1970.
Vol. 133,
THE JOURNAL OF TJRGLOGY
Copyright© 1985 by The \/Villiarn.s & 'vVilkins Co,
Printed in
ANGIOMYELOLIPOIVIA ASSOCIATED WITH BILATERAL ADRENOCORTICAL HYPERPLASIA AND HYPERTENSION F. ESCUIN, P. GOMEZ, I. MARTINEZ, M. PEREZ-FONTAN, R SELGAS
AND
L. SANCHEZ-SICILIA
From the Nephrology Service, Ciudad Sanitaria La Paz, Medical School of Uniuersidad Autonoma of Madrid, Madrid, Spain
ABSTRACT
We report a case of adrenal myelolipoma associated with adrenocortical hyperplasia and hypertension. Although several endocrine derangements have been described in association with myelolipomas, we have found no reports of its association with adrenocortical hyperplasia. Myelolipoma is a benign neoplasm of the adrenal gland that is constituted by fatty and hematopoietic tissue. 1 First described in 1905 by Gierke 2 myelolipoma received its present designation in 1929 from Oberling. 3 It is usually a necropsy finding and creates no problems owing to its slight growth rate,4 for which few cases have required surgical intervention. An operation is performed only when excessive tumor growth mimics other pathological conditions, such as calcified adrenal cysts,5 retroperitoneal malignancy, 6 macroscopic hematuria, 7 acute appendicitis 8 or even a casual finding. 9 We report a case of myelolipoma associated with adrenocortical hyperplasia, severe
satisfactorily with 400 mg. spironolactone daily. An abdominal computerized tomography (CT) scan revealed only a slight increase in the size of the right adrenal gland that was not considered significant. An adrenal scan with iodocholesterol showed an increased uptake of the tracer in the right adrenal gland, with no evidence of suppression bilaterally after the administration of dexamethasone (fig. 1). Exploratory laparotomy and right adrenalectomy were performed. The patient remained hypertensive postoperatively and spironolactone controlled the blood pressure. The surgical specimen labeled right adrenal gland was 5 x 4
FIG. l. Iodocholesterol adrenal scan. A, bilatem! uptake with more intensity on right side. B, no evidence of suppression after administration of dexamethasone.
hypertension and biochemical features of steronism.
hyperaldo-
CASE REPORT
A 12-year-old white girl was referred to our hospital for evaluation of severe hypertension. Blood pressure was 170/130 mm. Hg and serum potassium was 2.5 mmol./1. without diuretic therapy. Catecholamines and vanillyl mandelic acid in a 24hour urine specimen were normal. Excretory urography was normal. Plasma renin activity was markedly suppressed under basal conditions and after 3 hours of ambulation (see table). Plasma and urine (14 µ,g. per 24 hours, normal 5.5 to 13) aldosterone levels were high. The blood pressure was controlled Accepted for publication December 3, 1984. 655
X 4 cm. in size. The macroscopic sections showed that most of the parenchyma was replaced by hematic material. A solid structure with abundant fatty tissue and abundant islets of extramedullary hematopoiesis was noted microscopically and interspersed with the hemorrhagic zone (fig. 2). Hyperplasia of the adrenal cortex was demonstrated primarily at the zona glomerulosa.
DISCUSSION
Myelolipomas usually are necropsy findings, with a reported incidence ranging from 0.087 to 0.2 per cent. 10 The etiology of these neoplasms is still obscure, and various hypotheses of the pathogenesis include bone marrow embolism,4 extramedullary hematopoiesis 11 and bone marrow metaplasia. 4 •12 The tumor is associated frequently with hypertension. 6 •7 • 13
656
ESCUIN AND ASSOCIATES
FIG. 2. Adrenal myelolipoma. A, adipose tissue and hematopoietic islets occupy adrenal medulla. H & E, reduced from X25. B, glomerular type adrenal cortex cells in relation to osseous trabecula and hematopoietic islets. Preoperative and postoperative plasma renin and aldosterone levels during 100 mM. sodium diet Plasma renin activity (ng./ml./hr.): Basal After 3 hrs. ambulation Plasma aldosterone (pg./ml.): Basal After 3 hrs. ambulation
Preop.
Postop.
0.066 0.089
0.092 0.120
259 223
212 228
Normal Range 0.62 ± 0.13 2.40 ± 0.27 67.0 ± 12.5 184 ± 28
Plaut reported associated endocrine derangements in 9 of 100 patients with myelolipoma, including Addison's disease, Cushing's disease, hermaphroditism, obesity, virilism and multiple endocrinopathies. 4 In our patient, who to our knowledge is the youngest subject to be described, the tumor was associated with adrenocortical hyperplasia, hypertension and biochemical data suggesting primary hyperaldosteronism.
REFERENCES
1. Dyckman, J. and Freedman, D.: Myelolipoma of the adrenal with clinical features and surgical excision. J. Mt. Sinai Hosp., 24: 793, 1957. 2. Gierke, E.: Uber Knochenmarksgewebe in der Nebennierre. Beitr. Path. Anat., suppl., 7: 311, 1905. 3. Oberling, G.: Les formations myelo-lipomateuses. Bull. Cancer (Paris), 18: 234, 1929. 4. Plaut, A.: Myelolipoma in the adrenal cortex (myeloadipose structures). Amer. J. Path., 34: 487, 1958. 5. Whittaker, L. D.: Myelolipoma of the adrenal gland. Surgical removal. Arch. Surg., 97: 628, 1968. 6. Fernandez-Sanz, J., Galera, H., Garcia-Donas, A., GonzalezCampora, R., Llamas, R. and Matilla, A.: Adrenal myelolipoma simulating a retroperitoneal malignant neoplasm. J. Urol., 126: 780, 1981. 7. Olsson, C. A., Krane, R. J., Klugo, R. C. and Selikowitz, S. M.: Adrenal myelolipoma. Surgery, 73: 665, 1973.
ANGIOMYELOUPOMA, ADRENOCORTICAL HYPERPLASIA AND HYPERTENSION 80 Ishika-\iv.a I-I., Tachibanai JVL, Hata, IVL, Tazaki, H.i ~"''""u~,,, and Iri, H.: Myelolipoma of the adrenal gland. J. Urol., 777, 1981. 9. Braendstrup, 0., Gerdes, U., Lange, J. and Str0m, P. F.: Myelolipoma of the adrenal gland. Scand. J. Urol. Nephrol., 15: 343, 1981. 10. McDonnel, W. V.: Myelolipoma of the adrenal. Arch. Path., 61: 1
657
416, 1956. 11. Holliday, T. D. S.: Massive bone-marrow heterotopia in both adrenal glands. J. Path. Bact., 70: 239, 1955. 12. Nicod, J. L.: Adenoma, lipome et myelolipome de la cortico-surrenale. Bull. Ass. Franc Cancer, 50: 109, 1963. 13. Tulcinsky, D. E., Deutsch, V. and Bubis, J. J.: Myelolipoma of the adrenal gland. Brit. J. Surg., 57: 465, 1970.