Recommended screening for all incidentalomas Test Cushings syndrome Cushings syndrome 1 mg overnight dexamethasone suppression test Pheochromocytoma Pheochromocytoma 24hour urine collection for fractionated metanephrines and catecholamines ID: 915588
Download Presentation The PPT/PDF document "Recommended screening for all incidental..." is the property of its rightful owner. Permission is granted to download and print the materials on this web site for personal, non-commercial use only, and to display it on your personal computer provided you do not modify the materials and that you retain all copyright notices contained in the materials. By downloading content from our website, you accept the terms of this agreement.
Slide1
Recommended screening for all incidentalomas
Recommended screening for all incidentalomas
Test
Cushing's syndromeCushing's syndrome1 mg overnight dexamethasone suppression testPheochromocytomaPheochromocytoma24-hour urine collection for fractionated metanephrines and catecholaminesPrimary aldosteronism (screen only in hypertensive patients)Primary aldosteronism (screen only in hypertensive patients)Plasma aldosterone to plasma renin activity ratio
Table 1
Recommended screening tests in adrenal incidentalomas. Additional analyses in bilateral incidentalomas listed below will depend on the clinical presentation and family history.
Slide3Recommended screening for all incidentalomas
Test
Cushing's syndrome
1 mg overnight dexamethasone suppression testPheochromocytoma24-hour urine collection for fractionated metanephrines and catecholaminesPrimary aldosteronism (screen only in hypertensive patients)Plasma aldosterone to plasma renin activity ratio
Slide4Additional screening recommended for bilateral incidentalomas
Tes
t
Adrenal insufficiencyMorning cortisol and ACTH (or corticotrophin stimulation test)MEN2RET gene mutation analysis, evaluation for hyperparathyroidism, medullary thyroid cancer, or mucosal neuromasVon Hippel-Lindau syndromeVHL gene mutation analysis and evaluation for additional tumors (such as renal, retinal, or nervous system)Neurofibromatosis type 1NF1 gene mutation analysis
Pheochromocytoma-paraganglioma syndrome
SDHB/SDHD
gene mutation analysis
Additional screening recommended for bilateral incidentaloma
s
Test
Adrenal insufficiency
Morning cortisol and ACTH (or corticotrophin stimulation test)
MEN2
RET
gene mutation analysis, evaluation for hyperparathyroidism, medullary thyroid cancer, or mucosal neuromas
Von Hippel-Lindau syndrome
VHL
gene mutation analysis and evaluation for additional tumors (such as renal, retinal, or nervous system)
Neurofibromatosis type 1
NF1
gene mutation analysis
Pheochromocytoma-paraganglioma syndrome
SDHB/SDHD
gene mutation analysis
Slide5Slide6Slide7Slide8Conn’s syndrome
(adrenal aldosterone-producing adenoma) and
bilateral
adrenal hyperplasia are the most common causes of primary aldosteronism. The treatment of choice for patients with aldosterone-producing adenoma is unilateral total adrenalectomy. Recurrence after adequate surgery is exceptional.
Slide9A 62-year-old
woman was admitted to the
hospital
because of severe hypokalemia and poorly controlled hypertension. She underwent adenomectomy for primary aldosteronism due to right APA 9 years ago. The pathological examination revealed a 2-cm diameter ad-enoma without other abnormalities. After surgery, she had been asymptomatic with normotension and normokalemia without pharmacologic treatment
Slide10In the
follow-up
repeated
plasma renin activity and serum aldosterone levels were normal. Six months before admission, hypertension resistant to antihypertensive poly therapy, weakness and paraesthesias developed. At entry, her medications comprised fosinopril (20 mg daily),amlodipine (10 mg daily) and hydrochloro-iazide (25 mg daily). There was no family history
of hypertension.
Slide11A physical
examination
showed
only moderate obesity. The blood pressure was 160/110 mmHg. Blood chemistry showed: urea 4.3 nmol/l, creatinine 52 μmol/l, sodium 143 mmol/l, potassium 2.4 mmol/l and chloride 96 mmol/l. Haematology, chest X-ray and electrocardiogram were normal.
Slide12On free dietary
sodium intake
, measured
supine at 08.00 h before she arose, plasma aldosterone concentration was3575 pmol/l (normal range 41–412 pmol/l)and plasma renin activity was undetectable.Upright at 08.00 h, plasma aldosterone con-centration and plasma renin activity were 2550pmol/l (137–825 pmol/l) and undetectable,respectively. Plasma aldosterone concentration before and after saline loading was 3230 pmol/land 3055 pmol/l, respectively.
Slide13The
24-hour urinary
free cortisol
was 120 nmol (75–250nmol). The 08.00 h basal cortisol was 223nmol/l (140–690 nmol/l). The 08.00 h cortisol after dexamethasone suppression (1 mg at23.00 h) was 47 nmol/l. The 24-hour urinary free catecholamines, dopamine, epinephrine and norepinephrine were normal.
Slide14Slide15Slide16Adrenal adenomas can be
di
ff
erentiated from metastases by adrenal MRI. A hyperintense rim in T1- andT2-weighted sequences is characteristic for adenomas,as in our case.
Slide17We describe the recurrence of an adrenal
APA 9
years after adenomectomy, which is
extremely rare; to our knowledge, only two such cases have previously been reported in the literature.
Slide18The resected adrenal gland sometimes
contains
macro- or micro-nodular lesions
in addition to APA.These nodules are multiple and generally bilateral and lead to a more complex diffrential diagnosis than the presence of a unilateral solitary mass. This bilateral nodularity is not synonymous with hyperplasia, and adrenal venous sampling is required to detect cases of surgically correctable APA.The clinical significance of these nodular lesions in patients with APA is not fully clear. They are not thought to contribute to recurrence but to result from intractable hypertension.
Slide19Slide20Autopsy studies
suggest a prevalence of incidentalomas of around
2% (range 1.0–8.7%),
increasing with age. Radiological studies report a frequency close to 3% in patients below the age of 50 years, increasing up to 10% in the elderly. Childhood incidentalomas are extremely rare.
Slide21Rationale
Bilateral adrenal masses usually represent
benign adenomas, macronodular hyperplasia or distinct bilateral nodules. In the relevant clinical setting, metastases (especially in patients with known malignancy), lymphoma or pheochromocytomas should also be considered. Each lesion should be evaluated individually as bilateral adrenal masses can represent co-occurrence of different lesions.
Slide22Slide23IMPORTANCE
Adrenal
incidentalomas are found in 1% to 5% of abdominal cross-sectional imaging studies. Although the workup and management of unilateral lesions are well established, limited information exists for bilateral incidentalomas.OBJECTIVETo compare the natural history of patients having bilateral incidentalomas with those having unilateral incidentalomas.DESIGN, SETTING, AND PARTICIPANTS Retrospective analysis of a prospective database of consecutive patients referred to an academic multidisciplinary adrenal conference. The setting was a tertiary care university hospital among a cohort of 500 patients with adrenal lesions between July 1, 2009, and July 1, 2014
Slide24RESULTS
23
patients with bilateral incidentalomas and 112 patients with unilateral lincidentalomas were identified. The mean age at diagnosis of bilateral lesions was 58.7 years.The mean lesion size was 2.4 cm on the right side and 2.8 cm on the left side. Bilateral incidentalomas were associated with a significantly higher prevalence of subclinical Cushing syndrome (21.7%[5 of 23] vs 6.2%[7 of 112]) (P = .009) and a significantly lower prevalence of pheochromocytoma (4.3%[1 of 23] vs 19.6%[22 of 112]) (P = .003) compared with unilateral lesions, while rates of hyperaldosteronism
were similar in both groups (4.3%[1
of23
] vs 5.4%[6 of 112]) (P > .99). Only one patient with bilateral incidentalomas underwent unilateral resection. The mean follow-up was 4 years (range, 1.2-13.0 years). There were no
occult adrenocortical carcinomas.
Slide25Slide26Abstract
A 50-year-old male
patient with a
15-year history of hypertension was referred to our hospital for evaluation of bilateral adrenal tumors. No Cushingoid features were observed. Computed tomographic scan showed 10-mm masses in each adrenal gland. Preoperative endocrinological examinations revealed autonomous cortisol and aldosterone secretion in this patient. The results of a subsequent adrenal venous catheterization study were consistent with the presence of a left cortisol-producing tumor and a right aldosterone-producing tumor. A left partial adrenalectomy was performed initially, but cortisol and aldosterone over-secretion persisted. Accordingly, the patient underwent a right adrenalectomy. Pathological examination of the resected specimens, including immunohistochemical analysis, demonstrated that both adenomas possibly produced cortisol and aldosterone. This is an extremely rare case of bilateral adrenal tumors, in which the left adrenocortical tumor produced and secreted cortisol or both cortisol and aldosterone and the right one produced and secreted both aldosterone and cortisol, as confirmed by clinical findings and pathological studies using immunohistochemical analysis.
Slide27Approximately
15% of adrenal incidentalomas occur
bilaterally
. Whereas most unilateral masses are benign or nonfunctional, the bilateral adrenal mass is more likely metastatic disease, hemorrhage, infiltrative disease, congenital adrenal hyperplasia, macronodular Cushing's syndrome, or bilateral cortical adenomas . A Bilaterl pheochromocytomas are also possible, especially as part of a familial syndrome.
Slide28Primary bilateral macronodular adrenal hyperplasia
is a rare cause of Cushing's
syndrome and is
more often diagnosed as bilateral adrenal incidentalomas with subclinical cortisol production.