J. Endocrinol. Invest. 13: 581-585,1990
Adrenal macrotumors diagnosed by computed tomography J.M. Lopez, C. Fardella, E. Arteaga,P. Michaud, J.A. Rodriguez, and F. Cruz Departments of Endocrinology and Radiology, School of Medicine, Catholic University of Chile, Santiago, Chile
ABSTRACT. Adrenal tumors larger than 6 cm are unusual but show a greater incidence of malignant etiologies than smaller adrenal tumors. The scarce information about adrenal macrotumors (AMT) induced us to study prospectively all patients who were seen in our clinic during the period 19841988 and were diagnosed by computed tomography (CT) to have an adrenal mass ~ 6 cm. The clinical characteristics, including the main complaint, tumoral secretory activity, CT findings and histologic diagnosis from 18 patients are described; they represented a 0.3% of the total amount of abdominal CT studies performed. Seventy-two % of AMT resulted to be non cortisol or catecholamines secreting masses, and from them, a 380/0 corresponded to malignant etiologies characterized by marked and rapid weight loss. Four out of
five hormone secreting AMT corresponded to pheochromocytomas, while the fifth one resulted to be a bilateral macronodular hyperplasia secreting cortisol. In two cases AMT did not correspond to a genuine adrenal mass, resulting to be a malignant histocytoma in one case and a hydatidic cyst in other one. When performed in two occasions, a percutaneous needle biopsy was a valuable diagnostic tool, permitting to diagnose a lymphoma and a tuberculoma. In conclusion: 1) AMT correspond mainly to non cortisol or catecholamines secreting tumors, often malignant; 2) not all AMT diagnosed by CT correspond to true adrenal masses; 3) percutaneous needle biopsy can be a helpful procedure for diagnosing nonfunctioning solid AMT.
INTRODUCTION
(8 men and 10 women) were diagnosed as having adrenal macrotumor, defined by a diameter ~ than 6 cm. Seventeen patients consulted because of symptoms related to the tumor. The clinical characteristics of the study group are summarized on Tables 1 and 2. In 14 subjects computed tomography was performed using a Siemens' Somaton DR 512*512 matrix, with 8 mm sections. In 4 subjects a 320*320 EMI model was used, with 10 mm sections. ' Fasting blood samples were taken at 09:00 h for plasma sodium, potassium and cortisol determinations or catecholamines if required. A 24-h urine sample was obtained for 17 hydroxycorticosteroids (17-OHCS), 17 ketosteroids (17-KS), adrenaline and noradrenaline determinations. In those cases suspected of having a hormone-secreting adrenal tumor, the study was focused on confirming this condition. Sodium and potassium were determined by flame photometry and cortisol by a direct radioimmunoassay developed in our laboratory using an antibody kindly provided by Dr. Paul Vecsei, Heidelberg. The 17-OHCS were measured by the method of Porter and Silber (12), and 17-KS bythat
Computed tomography allows detection of adrenal masses as small as 1 cm in diameter (1-3). Copeland (4) focused the study of adrenal tumors according to its size recognizing a group larger and other smaller than 6 cm in diameter; those tumors larger than 6 cm have a higher probability of being malignant. Since the information available on adrenal tumors concerns mostly small masses (5-7), which are clearly more frequent than larger masses, as inferred from four autopsy studies (8-11), we report observations on 18 subjects who' were diagnosed as having large adrenal tumors.
SUBJECTS AND METHODS During the 1984-198a period, a total of 6031 abdominal computed tomographic studies were performed in our Center. In this population 18 subjects Key-words: Adrenal macrotumor, adrenomegaly, computed tomography. Correspondence: Jose M. Lopez, M.D., Laboratorio de Endocrinologfa, Hospital Cllnico, Universidad Catolica de Chile, Casilla 114-0, Santiago, Chile. Received July 4, 1989; accepted April 26, 1990.
581
J.M. Lopez, C. FardelIa, E. Aiteaga, et al.
Table 1 - Secreting adrenal macrotumors. Histologic Diagnosis
sex
Clinical features age complaint (yr)
size (cm)
CT findings side
density
Urine excretion Ep NE (JLg/24 h)
Pheochromocytoma
F
44
Hbp
7.5
R
solid
390
503
Pheochromocytoma
F
44
Hbp
6.4
R
solid
71
1720
Pheochromocytoma
F
47
Hbp
6.0
R
solid
83
2519
Pheochromocytoma
F
20
Hbp
8.0
R
solid
121
Macronodular Hp
F
45
Cushing
9.3&4.7
L&R
solid
1093 Cortisol
CT: computed tomography Ep : Epinephrine NE: Nor-epinephrine NV (I Ep + NE < 100 ~g/24 h)
Hp : hyperplasia Hbp: high blood pressure L : left R : right
of Zimmermann (13). Adrenaline and noradrenaline were determined in urine by a fluorometric technique (14). In two patients a percutaneous fine needle biopsy of the adrenal mass under ultrasound guidance was performed (15). All subjects, except one, underwent surgical exploration. The specimens obtained by either puncture or surgery were histologically examined.
Histological tindinqs were classified as follows: 5 pheochromocytomas (one of them necrosed), 3 cortical adenomas, 3 metastatic malignancies (2 lung cancers and one lymphoma), 1 primitive adrenal carcinoma, 1 macronodular adrenal hyperplasia (16), 1 adrenal tuberculoma, 1 lipoma and 1 adrenal hematoma with no detectable histologic abnormalities in the residual adrenal tissue. In 2 cases the normal adrenal gland closely adhered to a paraadrenal tumor. In one of these cases the mass resulted to be a hydatidic cyst (Fig. 1b) while in the other was a malignant histiocytoma, which extensively metastasized two years after diagnosis was made. Globally, 28% of the tumors corresponded to malignancies. Adrenal macrotumors were grouped as non cortisol
RESULTS In 17 cases an abdominal retroperitoneal macrotumor was confirmed bya surgical procedure; the
remaining patient was not operated on because a bilateral malignant lymphoma was certified by a percutaneous puncture (Fig. 1a). Table 2 - Non-secreting adrenal macrotumors. Histologic Diagnosis
sex
Clinical features age complaint (yr)
size
CT findings side
density
Glucocorticoid deficit
(ern)
Lymphoma
M
54
weight loss
9.0&8.0
L&R
solid
Yes
Hystiocytoma
M
80
pain
13.5
L
mixed
No
Metastatic cancer
M
60
weight loss
9.5
L
solid
No
Metastatic cancer
M
29
weight loss
6.0
R
solid
No
Adrenal cancer
Cortical adenoma
M F F F
Cortical adenoma Cortical adenoma
21
weight loss
10.5
L
mixed
No
78
fever
8.0
R
mixed
No
62
weight loss
8.0
L
mixed
No
63
Hbp
6.0
R
soHd
No
M
29
pain
6.0
R
mixed
No
M
44
Hbp
6.0
L
solid
No
Lipoma
F
63
pain
10.0
L
solid
No
Hematoma
M
35
pain
10.0
L
solid
No
Hydatidic Cyst
F
38
infertility
9.0
R
solid
No
Pheochromocytoma Tuberculosis
582
Adrenal macrotumors
Fig. 1 - Computed tomographic scans of abdomen showing: a) a bilateral adrenal lymphoma; b) a right para -adrenal hydatidic cyst.
or catecholamines secreting (NS) (72%) or secreting tumors (S) (28%). Clinical, radiological and histological characteristics are presented in Tables 1 and 2. The patient with a necrosed pheochromocytoma showed normal urinary epinephrine (2.8 M9/24 h) and norepinephrine (41 M9/24 h) excretion (normal values : ~ epinephrine and norepinephrine: < 100 119/24 h).
explain the observed febrile syndrome and the normalcy of blood pressure and catecholamine excretion. Tuberculosis is another rare cause of nonsecreting adrenal macrotumor (19-20); its reported rarity probably reflects the low frequency of this disease . In Chile, however , adrenal tuberculosis is relatively common, accounting for 88% of the cases of Addison's disease, according to a necropsy study of 25 patients (21). Because of indemnity of contralateral gland our patient with tuberculosis did not show any signs of adrenal dysfunction. Other 3 benign NS tumors corresponded to cortical adenomas, an etiology which was recognized in 1.9% of 12,000 cumulative necropsy studies from four different authors; however, cort ical adenomas larger than 6 em resulted to be only a 1.3% of the general adenoma group (4). In 2 out of 3 cortical adenomas high blood pressure was detected; in spite of hypertension did not remit after tumor excision and serum potassium levels were always normal a DOC secreting cortical adenoma cannot be fully ruled out. Other two masses corresponded to a lipoma and one adrenal hematoma ; the last patient did not have any of the usual conditions related to adrenal hemorrhage, like altered blood coagulation, sepsis, surgery or trauma; also the study of the remaining tissue show an histologically normal adrenal gland. The presence of 2 nongenuine adrenal macrotumors in our series, one hydatidic cyst and one malignant histiocytoma, demonstrated the limitations of computed tomography to differentiate adrenal from para-adrenal tumors. The hydatidic cyst was incidentally detected when the patient consulted
DISCUSSION Our study shows that adrenal macrotumors have a low incidence among patients selected for abdominal CT scan (0.3%); they are generally symptomatic and correspond to a wide spectrum of etiologies where NS tumors are predominant (72%). Among the group defined as NS masses malignancy has a high frequency (38%) and is clinically char acterized by marked weight loss and abdominal pain. One of the malignanc ies was a bilateral adrenal lymphoma , manifested by adrenal glucocorticoid insufficiency; hypoadrenalism related to lymphoma has been described only in very few cases (17-18). The other four malignancies corresponded to a primitive adrenal carcinoma and 2 cases of adrenal compromise by lung carcinoma, and a malignant histiocytoma. The remaining group of 9 NS tumors corresponded to benign masses generally associated with abdominal pain with no significant weight loss. As expected , the posterior clinical course of these patients was in accordance to their benign type of lesion. One of these tumors corresponded to an extensively necrosed pheochromocytoma, a finding which may
583
J.M. Lopez, C. Fardella, E. Arteaga, et al.
for infertility; the perfect roundness of the images and some small peripherical calcifications deserve attention, since that shape is commonly described in hydatidic cysts from other localizations. Among the total group of macrotumors, a 28% (5 cases) corresponded to secreting masses. In four cases the etiology resulted to be a chromaffine tumor, clinically characterized by the classical symptoms related to hypersecretion of catecholamines (Table 1). The remaining case was an adrenocorticotropin independent bilateral macronodular hyperplasia, manifested as Cushing's syndrome, an extremely rare etiology (22). In general secreting adrenal tumors did not offer major diagnostic difficulty because of their secretory pattern. A percutaneous fine needle biopsy as performed in 2 cases. In one of them, a lymphoma was recognized, and thus, surgical exploration was omitted; in the other case tuberculosis was diagnosed when caseous material was obtained, and specific therapy was initiated prior to the surgical drainage. Percutaneous puncture is considered a useful procedure for studying adrenal masses, because of its high sensitivity and specificity for detection of metastatic malignant adrenal lesions (23, 24). However, the indication given by Copeland (4) to puncture cystic adrenal masses must be cautiously weighed, considering the risk related to etiologies such as cystic or necrosed unsuspected pheochromocytomas (25, 26) and hydatidic cysts. We agree that adrenal puncture should not be performed in patients with actively secreting tumors, because the diagnosis is generally obvious and they must be operated on (4)..Thus the major indication for puncture should be solid nonsecreting macrotumors since the presence of metastatic cancer could avoid an unnecessary surgical treatment. In our series, computed tomography could not clearly discriminate benign from malignant lesions. Radiological signs, other than big size, as contrast enhancement and consistency, have been described as good predictive indices for malignancy (27); however, in our series they did not permit a clear discrimination among benign and malignant lesions. Magnetic resonance imaging of the adrenals provides tissue characterization, that is unattainable with computed tomography. According to the signal intensity adrenal masses can be divided in three groups: adenomas.adrenal metastases or primitive carcinomas, and pheochromocytomas, if that intensity is low, intermediate or high, respectively
(28-29); identification of chromaffin tumors has been highly successful. In summary: 1) adrenal macrotumors mainly correspond to non hormone-secreting tumors, often malignant, whose diagnosis can be suspected by the associated weigh loss; 2) not all adrenal macrotumors diagnosed by computed tomography correspond to true adrenal masses; they can also be juxtadrenal tumors; 3) computed tomography could not accurately differentiate benign from malignant adrenal macrotumors; 4) percutaneous fine needle biopsy of an adrenal mass under ultrasound guidance can be a helpful diagnostic procedure to avoid unnecessary surgery in non-functional tumors suspected of containing metastatic cancer.
REFERENCES 1. Korobkin M., White E.A., Kressel H.Y., Moss A.A., Montagne J.P. Computed tomography in the diagnosis of adrenal disease. Am. J. Roentgenol. 132: 231, 1979.
2. Prinz R.A., Brooks M.H., Churchill R., Graner J.L., Lawrence A.M., Paloyan E., Sparagana M. Incidental asymptomatic adrenal masses detected by computed tomographic scanning. Is operation required? JAMA 248: 701, 1982.
3. Adams J., Johnson R., Rickards D., Isherwood I. Computed tomography in adrenal disease. Clin. Radiol. 34: 39, 1983. 4. Copeland P. The incidentally discovered adrenal mass. Ann. Intern. Med. 98: 940, 1983. 5. Guerrero L.A. Diagnositic and therapeutic approach to incidental adrenal mass. Urology 26: 435, 1985. 6. Virkkala A., Valimaki M., Pelkonin R., Huikuri K., Kahri A., Kivisaari L., Korhonen T., Salmi J., Sepals P. Endocrine abnormalities in patients with adrenal tumours incidentally discovered on computed tomography. Acta Endocrinol. (Copenh.) 121: 67, 1989. 7. Thompron N.W., Cheung P.S.Y. Diagnosis and treatment of functioning and non functioning adrenocortical neoplasms including incidentalomas. Surg. Clin. NA. 67: 423, 1987.
8. Shamma A.H., Goddard J.W., Sommers S.C. A study of the adrenal status in hypertension. J. Chronic Dis. 8: 587, 1958.
584
Adrenal macrotumors
20. Billaud L., Benabed K., Requeda E., Guilhaume B., Louvel A., Chapuis I., Bonnin A., Luton J.P. Lesions tumorales et pseudo-tumorales de la loge surrenalienne a ne pas rneconnaitre, Presse Med. 16: 1405, 1987.
9. Russi S., Blumenthal H.T., Gray S.H. Small adenomas of the adrenal cortex in hypertension and diabetes. Arch. Intern. Med. 76: 284, 1945. 10. Kokko J.P., Brown T.C., Berman M.N. Adrenal adenoma and hypertension. Lancet 1: 468, 1967.
21. i.apez E., Rolando M., Donoso F., Jadresic A., Maira J., Lennon H., Gonzalez J., Guevara J. Etiologia y tratamiento de la enfermedad de Addison en Chile. Rev. Med. Chile. 102: 283, 1974.
11. Hedeland H., Osteberg G., Hokfelt B. On the prevalence of adrenocortical adenomas in an autopsy material in relation to hypertension and diabetes. Acta Med. Scand. 184: 211, 1968. 12. Silber R., Porter C. The determination of 17,21 hydroxi-20 ketosteroides in urine and plasma. J. BioI. Chem. 210: 923, 1954.
22. Malchoff C., Rosa J., Rowan De Bold C., Kozol R., Ramsby G., Page D., Malchoff D., Orth D. Adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia, an unusual cause of Cushing's syndrome. J. Clin. Endocrinol. Metab. 68: 855, 1989.
13. Zimmermann J. Eine farbreaktion dear sexual hormone und ihre an werendung zur quantitative colorimetrishen bentimung. Hopper Segler Z. Physical. Chem. 233: 257, 1935.
23. Montali G., Solbiati L., Bossi M., De Pra L., Di Donna A., Ravetto C. Sonographically guidedfine-needleaspiration biopsy of adrenal masses. AJR. 143:1081,1984.
14. Aaron H., Sayre D. A study of the factors affecting the aluminiunoxidetrihidrcxyindole. Procedure for the analyses of catecholarnlries. J. Pharm. 138: 360, 1962.
24. Orell S., Langlois S., Marshall V. Fine needle aspiration cytology in the diagnosis of solid renal and adrenal masses. Scand. J. Urol. Nephrol. 19: 211,1985.
15. Bernardino M.E., Walther M.M., Philips V.M. CT-Guided adrenal biopsy: accuracy, safety and indications. Am. J. Roentgenol. 144: 67,1985. 16. Arteaga E.U., Mahana D., Gonzalez R., Martinez P. Sindrome de Cushing por hiperplasia suprarrenal macronodular independiente de ACTH. Rev. Med. Chile 117: 1398, 1989. 17. Huminer D., Garty M., Lapidot M., Leiba S., Borohov H., Rosenfeld J.B. Lymphoma presenting with adrenal insufficiency. Adrenal enlargementon computed tomographic scanning as a clue to diagnosis. Am. J. Med. 84: 169, 1988. 18. Osei K., Falko J., Patcht E., Wall R., Goldberg R. Primary adrenal insufficiency manifesting as malignant lymphoma. Arch. Intern. Med. 143: 1791, 1983.
25. Mc Corkell S., Niles N. Fine-needle aspiration of catecholamine-producing adrenal masses: A possibly fatal mistake. AJR 145: 113, 1985. 26. Casola G., Nicolet V., Van Sonnenberg E., Withers C., Bretagnolle M., Saba R., Bret P. Unsuspected pheochromocytoma: Risk of bloodpressure alterations during percutaneous adrenal biopsy. Radiology 159: 733, 1986. 27. Hussain S., Belldegrun A., Seltzer S.E., Richie J.P., Gittes R.F., Abrams H.L. Differentiation of malignant from benign adrenal masses: predictive indices on computedtomography. AJR 144: 61, 1985. 28. Reinig J.W., Doppman J.L., Dwyer A.J., Frank J. MRI of indeterminateadrenal masses. AJR 147: 493, 1986. 29. Glazer G.M., Woolsey E.J., Borrello J., Francis I.R., Aisen A.M., Bookstein F., Amendola M.A., Gross M.D., Bree R.L., Martel W. Adrenal tissue characterization using MR imaging. Radiology 158: 73, 1986.
19. MartinezJ., Masa C., Perez R. Adrenal cold abscesses. Ann. Intern. Med. 99: 413, 1983.
585
Adrenal macrotumors diagnosed by computed tomography J.M. Lopez, C. Fardella, E. Arteaga,P. Michaud, J.A. Rodriguez, and F. Cruz Departments of Endocrinology and Radiology, School of Medicine, Catholic University of Chile, Santiago, Chile
ABSTRACT. Adrenal tumors larger than 6 cm are unusual but show a greater incidence of malignant etiologies than smaller adrenal tumors. The scarce information about adrenal macrotumors (AMT) induced us to study prospectively all patients who were seen in our clinic during the period 19841988 and were diagnosed by computed tomography (CT) to have an adrenal mass ~ 6 cm. The clinical characteristics, including the main complaint, tumoral secretory activity, CT findings and histologic diagnosis from 18 patients are described; they represented a 0.3% of the total amount of abdominal CT studies performed. Seventy-two % of AMT resulted to be non cortisol or catecholamines secreting masses, and from them, a 380/0 corresponded to malignant etiologies characterized by marked and rapid weight loss. Four out of
five hormone secreting AMT corresponded to pheochromocytomas, while the fifth one resulted to be a bilateral macronodular hyperplasia secreting cortisol. In two cases AMT did not correspond to a genuine adrenal mass, resulting to be a malignant histocytoma in one case and a hydatidic cyst in other one. When performed in two occasions, a percutaneous needle biopsy was a valuable diagnostic tool, permitting to diagnose a lymphoma and a tuberculoma. In conclusion: 1) AMT correspond mainly to non cortisol or catecholamines secreting tumors, often malignant; 2) not all AMT diagnosed by CT correspond to true adrenal masses; 3) percutaneous needle biopsy can be a helpful procedure for diagnosing nonfunctioning solid AMT.
INTRODUCTION
(8 men and 10 women) were diagnosed as having adrenal macrotumor, defined by a diameter ~ than 6 cm. Seventeen patients consulted because of symptoms related to the tumor. The clinical characteristics of the study group are summarized on Tables 1 and 2. In 14 subjects computed tomography was performed using a Siemens' Somaton DR 512*512 matrix, with 8 mm sections. In 4 subjects a 320*320 EMI model was used, with 10 mm sections. ' Fasting blood samples were taken at 09:00 h for plasma sodium, potassium and cortisol determinations or catecholamines if required. A 24-h urine sample was obtained for 17 hydroxycorticosteroids (17-OHCS), 17 ketosteroids (17-KS), adrenaline and noradrenaline determinations. In those cases suspected of having a hormone-secreting adrenal tumor, the study was focused on confirming this condition. Sodium and potassium were determined by flame photometry and cortisol by a direct radioimmunoassay developed in our laboratory using an antibody kindly provided by Dr. Paul Vecsei, Heidelberg. The 17-OHCS were measured by the method of Porter and Silber (12), and 17-KS bythat
Computed tomography allows detection of adrenal masses as small as 1 cm in diameter (1-3). Copeland (4) focused the study of adrenal tumors according to its size recognizing a group larger and other smaller than 6 cm in diameter; those tumors larger than 6 cm have a higher probability of being malignant. Since the information available on adrenal tumors concerns mostly small masses (5-7), which are clearly more frequent than larger masses, as inferred from four autopsy studies (8-11), we report observations on 18 subjects who' were diagnosed as having large adrenal tumors.
SUBJECTS AND METHODS During the 1984-198a period, a total of 6031 abdominal computed tomographic studies were performed in our Center. In this population 18 subjects Key-words: Adrenal macrotumor, adrenomegaly, computed tomography. Correspondence: Jose M. Lopez, M.D., Laboratorio de Endocrinologfa, Hospital Cllnico, Universidad Catolica de Chile, Casilla 114-0, Santiago, Chile. Received July 4, 1989; accepted April 26, 1990.
581
J.M. Lopez, C. FardelIa, E. Aiteaga, et al.
Table 1 - Secreting adrenal macrotumors. Histologic Diagnosis
sex
Clinical features age complaint (yr)
size (cm)
CT findings side
density
Urine excretion Ep NE (JLg/24 h)
Pheochromocytoma
F
44
Hbp
7.5
R
solid
390
503
Pheochromocytoma
F
44
Hbp
6.4
R
solid
71
1720
Pheochromocytoma
F
47
Hbp
6.0
R
solid
83
2519
Pheochromocytoma
F
20
Hbp
8.0
R
solid
121
Macronodular Hp
F
45
Cushing
9.3&4.7
L&R
solid
1093 Cortisol
CT: computed tomography Ep : Epinephrine NE: Nor-epinephrine NV (I Ep + NE < 100 ~g/24 h)
Hp : hyperplasia Hbp: high blood pressure L : left R : right
of Zimmermann (13). Adrenaline and noradrenaline were determined in urine by a fluorometric technique (14). In two patients a percutaneous fine needle biopsy of the adrenal mass under ultrasound guidance was performed (15). All subjects, except one, underwent surgical exploration. The specimens obtained by either puncture or surgery were histologically examined.
Histological tindinqs were classified as follows: 5 pheochromocytomas (one of them necrosed), 3 cortical adenomas, 3 metastatic malignancies (2 lung cancers and one lymphoma), 1 primitive adrenal carcinoma, 1 macronodular adrenal hyperplasia (16), 1 adrenal tuberculoma, 1 lipoma and 1 adrenal hematoma with no detectable histologic abnormalities in the residual adrenal tissue. In 2 cases the normal adrenal gland closely adhered to a paraadrenal tumor. In one of these cases the mass resulted to be a hydatidic cyst (Fig. 1b) while in the other was a malignant histiocytoma, which extensively metastasized two years after diagnosis was made. Globally, 28% of the tumors corresponded to malignancies. Adrenal macrotumors were grouped as non cortisol
RESULTS In 17 cases an abdominal retroperitoneal macrotumor was confirmed bya surgical procedure; the
remaining patient was not operated on because a bilateral malignant lymphoma was certified by a percutaneous puncture (Fig. 1a). Table 2 - Non-secreting adrenal macrotumors. Histologic Diagnosis
sex
Clinical features age complaint (yr)
size
CT findings side
density
Glucocorticoid deficit
(ern)
Lymphoma
M
54
weight loss
9.0&8.0
L&R
solid
Yes
Hystiocytoma
M
80
pain
13.5
L
mixed
No
Metastatic cancer
M
60
weight loss
9.5
L
solid
No
Metastatic cancer
M
29
weight loss
6.0
R
solid
No
Adrenal cancer
Cortical adenoma
M F F F
Cortical adenoma Cortical adenoma
21
weight loss
10.5
L
mixed
No
78
fever
8.0
R
mixed
No
62
weight loss
8.0
L
mixed
No
63
Hbp
6.0
R
soHd
No
M
29
pain
6.0
R
mixed
No
M
44
Hbp
6.0
L
solid
No
Lipoma
F
63
pain
10.0
L
solid
No
Hematoma
M
35
pain
10.0
L
solid
No
Hydatidic Cyst
F
38
infertility
9.0
R
solid
No
Pheochromocytoma Tuberculosis
582
Adrenal macrotumors
Fig. 1 - Computed tomographic scans of abdomen showing: a) a bilateral adrenal lymphoma; b) a right para -adrenal hydatidic cyst.
or catecholamines secreting (NS) (72%) or secreting tumors (S) (28%). Clinical, radiological and histological characteristics are presented in Tables 1 and 2. The patient with a necrosed pheochromocytoma showed normal urinary epinephrine (2.8 M9/24 h) and norepinephrine (41 M9/24 h) excretion (normal values : ~ epinephrine and norepinephrine: < 100 119/24 h).
explain the observed febrile syndrome and the normalcy of blood pressure and catecholamine excretion. Tuberculosis is another rare cause of nonsecreting adrenal macrotumor (19-20); its reported rarity probably reflects the low frequency of this disease . In Chile, however , adrenal tuberculosis is relatively common, accounting for 88% of the cases of Addison's disease, according to a necropsy study of 25 patients (21). Because of indemnity of contralateral gland our patient with tuberculosis did not show any signs of adrenal dysfunction. Other 3 benign NS tumors corresponded to cortical adenomas, an etiology which was recognized in 1.9% of 12,000 cumulative necropsy studies from four different authors; however, cort ical adenomas larger than 6 em resulted to be only a 1.3% of the general adenoma group (4). In 2 out of 3 cortical adenomas high blood pressure was detected; in spite of hypertension did not remit after tumor excision and serum potassium levels were always normal a DOC secreting cortical adenoma cannot be fully ruled out. Other two masses corresponded to a lipoma and one adrenal hematoma ; the last patient did not have any of the usual conditions related to adrenal hemorrhage, like altered blood coagulation, sepsis, surgery or trauma; also the study of the remaining tissue show an histologically normal adrenal gland. The presence of 2 nongenuine adrenal macrotumors in our series, one hydatidic cyst and one malignant histiocytoma, demonstrated the limitations of computed tomography to differentiate adrenal from para-adrenal tumors. The hydatidic cyst was incidentally detected when the patient consulted
DISCUSSION Our study shows that adrenal macrotumors have a low incidence among patients selected for abdominal CT scan (0.3%); they are generally symptomatic and correspond to a wide spectrum of etiologies where NS tumors are predominant (72%). Among the group defined as NS masses malignancy has a high frequency (38%) and is clinically char acterized by marked weight loss and abdominal pain. One of the malignanc ies was a bilateral adrenal lymphoma , manifested by adrenal glucocorticoid insufficiency; hypoadrenalism related to lymphoma has been described only in very few cases (17-18). The other four malignancies corresponded to a primitive adrenal carcinoma and 2 cases of adrenal compromise by lung carcinoma, and a malignant histiocytoma. The remaining group of 9 NS tumors corresponded to benign masses generally associated with abdominal pain with no significant weight loss. As expected , the posterior clinical course of these patients was in accordance to their benign type of lesion. One of these tumors corresponded to an extensively necrosed pheochromocytoma, a finding which may
583
J.M. Lopez, C. Fardella, E. Arteaga, et al.
for infertility; the perfect roundness of the images and some small peripherical calcifications deserve attention, since that shape is commonly described in hydatidic cysts from other localizations. Among the total group of macrotumors, a 28% (5 cases) corresponded to secreting masses. In four cases the etiology resulted to be a chromaffine tumor, clinically characterized by the classical symptoms related to hypersecretion of catecholamines (Table 1). The remaining case was an adrenocorticotropin independent bilateral macronodular hyperplasia, manifested as Cushing's syndrome, an extremely rare etiology (22). In general secreting adrenal tumors did not offer major diagnostic difficulty because of their secretory pattern. A percutaneous fine needle biopsy as performed in 2 cases. In one of them, a lymphoma was recognized, and thus, surgical exploration was omitted; in the other case tuberculosis was diagnosed when caseous material was obtained, and specific therapy was initiated prior to the surgical drainage. Percutaneous puncture is considered a useful procedure for studying adrenal masses, because of its high sensitivity and specificity for detection of metastatic malignant adrenal lesions (23, 24). However, the indication given by Copeland (4) to puncture cystic adrenal masses must be cautiously weighed, considering the risk related to etiologies such as cystic or necrosed unsuspected pheochromocytomas (25, 26) and hydatidic cysts. We agree that adrenal puncture should not be performed in patients with actively secreting tumors, because the diagnosis is generally obvious and they must be operated on (4)..Thus the major indication for puncture should be solid nonsecreting macrotumors since the presence of metastatic cancer could avoid an unnecessary surgical treatment. In our series, computed tomography could not clearly discriminate benign from malignant lesions. Radiological signs, other than big size, as contrast enhancement and consistency, have been described as good predictive indices for malignancy (27); however, in our series they did not permit a clear discrimination among benign and malignant lesions. Magnetic resonance imaging of the adrenals provides tissue characterization, that is unattainable with computed tomography. According to the signal intensity adrenal masses can be divided in three groups: adenomas.adrenal metastases or primitive carcinomas, and pheochromocytomas, if that intensity is low, intermediate or high, respectively
(28-29); identification of chromaffin tumors has been highly successful. In summary: 1) adrenal macrotumors mainly correspond to non hormone-secreting tumors, often malignant, whose diagnosis can be suspected by the associated weigh loss; 2) not all adrenal macrotumors diagnosed by computed tomography correspond to true adrenal masses; they can also be juxtadrenal tumors; 3) computed tomography could not accurately differentiate benign from malignant adrenal macrotumors; 4) percutaneous fine needle biopsy of an adrenal mass under ultrasound guidance can be a helpful diagnostic procedure to avoid unnecessary surgery in non-functional tumors suspected of containing metastatic cancer.
REFERENCES 1. Korobkin M., White E.A., Kressel H.Y., Moss A.A., Montagne J.P. Computed tomography in the diagnosis of adrenal disease. Am. J. Roentgenol. 132: 231, 1979.
2. Prinz R.A., Brooks M.H., Churchill R., Graner J.L., Lawrence A.M., Paloyan E., Sparagana M. Incidental asymptomatic adrenal masses detected by computed tomographic scanning. Is operation required? JAMA 248: 701, 1982.
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