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Adrenal Cortical Adenoma

Adenoma Adrenocortical

Adrenal cortical adenoma is often hormonally silent and discovered incidentally. In symptomatic patients, the resultant adrenal disease will reflect which hormone(s) is/are produced abundantly. There are specialized biochemical tests and imaging studies to make the diagnosis.


Presentation

Adrenal cortical adenomas are benign tumors that stem from the cortex of the adrenal gland. These adenomas are frequently discovered as adrenal incidentalomas, adrenal tumors confirmed on imaging during a workup for unrelated causes. Nearly 85% of adrenal incidentalomas are benign and nonfunctional [1] [2]. Functioning adrenal cortical adenomas may cause hypertension, orthostatic hypotension, and tachycardia. A physical exam may reveal central obesity, hirsutism, striae, and gynecomastia. An ophthalmologic exam may reveal retinopathy secondary to chronic hypertension. Endocrine diseases resulting from functioning adrenal cortical adenomas reflect the excess production of adrenal gland hormones such as aldosterone (primary aldosteronism), cortisol (Cushing's syndrome), and sex hormones.

Primary aldosteronism is not characterized by a specific clinical presentation. These patients may have unprovoked hypokalemia and hypertension although potassium levels can be normal [3]. As a result of hypernatremia, hypokalemic alkalosis, and other related mechanisms, patients may develop fatigue, weakness, headaches, tetany, and paresthesias. Also, polyuria and polydipsia could result from hypokalemia-induced nephrogenic diabetes insipidus. Complications secondary to chronic hypertension include renal, cardiac, neurologic, and ophthalmologic manifestations.

Cushing's syndrome is characterized by weakness, bruising, weight gain, and depression. Affected individuals may also develop osteoporosis and metabolic syndrome [4].

Finally, androgen or estrogen-secreting tumors may cause virilization in females and feminization in males.

Leg Pain
  • The authors report a primary spinal intramedullary adrenal cortical adenoma in a patient with spinal dysraphism presenting with bilateral leg pain and urinary frequency.[ncbi.nlm.nih.gov]
  • The tumor, which was located at the L2 level, manifested itself clinically by a short history of bilateral leg pain. It was well encapsulated; therefore, total surgical removal was accomplished.[ncbi.nlm.nih.gov]
  • The tumor, which was located at the L2 level, manifested itself clinically by a short history of bilateral leg pain. It was well encapsulated: therefore, total surgical removal was accomplished.[journals.lww.com]
Hirsutism
  • A physical exam may reveal central obesity, hirsutism, striae, and gynecomastia. An ophthalmologic exam may reveal retinopathy secondary to chronic hypertension.[symptoma.com]
  • These androgen secreting tumours frequently present in women with increased facial hair growth (hirsutism), irregular periods and infertility sometimes with masculinization. It is a very rare cause of polycytic ovary syndrome.[ensat.org]
  • Physical examination: truncal and centripetal obesity, thin extremities with muscle atrophy, plethoric moon face, echymoses and hirsutism.[endocrine-abstracts.org]
  • […] tumors, including cancers, are rare in patients with 21-hydroxylase deficiency but have been reported in patients with large adrenal glands and presumably inadequate cortisol therapy. 2 A 16-year-old girl with 21-hydroxylase deficiency was evaluated for hirsutism[nejm.org]
  • Conn's syndrome: hypertension, proximal muscle weakness, headache, polyuria, tachycardia with/without palpitation, hypokalemia, hypocalcemia Hypercortisolism/Cushing's syndrome: central obesity, moon facies, plethora, striae, thin skin, easy bruising, hirsutism[pathologyoutlines.com]
Recurrent Headache
  • In 1995, she was referred to our clinic again because of recurrent headache and palpitation, and enlargement of the left adrenal tumor.[ncbi.nlm.nih.gov]
Paresthesia
  • As a result of hypernatremia, hypokalemic alkalosis, and other related mechanisms, patients may develop fatigue, weakness, headaches, tetany, and paresthesias.[symptoma.com]

Workup

All individuals suspected to have adrenal cortical adenoma, whether based on symptoms or an incidental finding on imaging, need a detailed assessment. The workup should include a personal and family history, a thorough physical exam, and appropriate studies.

In the evaluation of adrenal incidentalomas, it is very important to consider the possibility of pheochromocytoma [5]. To identify a pheochromocytoma, the first-line test is the measurement of plasma fractionated metanephrines and normetanephrines and/or 24-hour urine collection of metanephrines and catecholamines [6].

Primary aldosteronism is confirmed with the ratio of plasma aldosterone concentration to plasma renin activity [7]. Moreover, the diagnosis is likely when the aldosterone–to–renin ratio is above 30 and the plasma aldosterone level is elevated (>0.5 nmol/L) [8]. Also, an electrolyte panel should be obtained in these patients.

An important test in the diagnosis of Cushing's syndrome is an overnight dexamethasone suppression test [9]. Other considerations in these patients include screening for metabolic syndrome with a lipid panel and glucose tolerance test.

Patients with virilization or feminization should be tested for androgens and estrogens respectively.

On unenhanced computed tomography (CT) scan, adrenal cortical adenoma is characterized by a low attenuation which reflects the presence of an abundant intracytoplasmatic fat [10]. Also, CT and magnetic resonance imaging (MRI) are both effective in discriminating between benign and malignant masses. Additionally, positron-emission tomography (PET) with 18F-fluorodeoxyglucose (18F-FDG) demonstrates excellent sensitivity for confirming adrenal malignancy [11].

Fine-needle aspiration has limited value and is reserved for cases in which all other findings are inconclusive [12].

Erythroblast
  • The adrenal cortical adenoma centrally included well-demarcated adipose tissue admixed with scattered islands of myelopoietic elements: erythroblasts, myeloid cell series and lymphocytic cells, which was eventually recognized as myelolipoma.[ncbi.nlm.nih.gov]

Treatment

  • Literature review revealed the disadvantages of different treatments in this period.[ncbi.nlm.nih.gov]
  • Our preliminary results suggest that percutaneous acetic acid injection is a safe and effective alternative for treatment of small functional adrenal cortical adenoma.[ncbi.nlm.nih.gov]
  • Five years after treatment of the Cushing's syndrome by removal of the tumor, asymptomatic mild hypercalcemia was incidentally noticed in both patients, which suggested the occurrence of primary hyperparathyroidism.[ncbi.nlm.nih.gov]
  • Early diagnosis and treatment of adrenal adenoma causing Cushing syndrome in pregnancy reduces maternal and fetal morbidity and mortality.[ncbi.nlm.nih.gov]
  • Treatment of small hepatocellular carcinoma in cirrhotic patients: a cohort study comparing surgical resection and percutaneous ethanol injection. Hepatology 1993 ; 18:1121-1126.[pubs.rsna.org]

Prognosis

  • The small size and weight of the tumor, the low mitotic rate, and the absence of necrosis and vascular and capsular invasion portend a good prognosis. The patient is currently free of disease 1 year after extirpation of the tumor.[ncbi.nlm.nih.gov]
  • […] other servers: Neurosecretory dense granules Molecular / cytogenetics description Tumorigenesis not well understood Outside of immunohistochemistry for diagnosis, adjunct molecular studies not currently utilized for clinical purposes (i.e. treatment, prognosis[pathologyoutlines.com]
  • Prognosis Extremely poor prognosis, with a survival rate at 20% at five years for stage I-II disease.Poor prognostic indicators include: age at diagnosis, tumor size, distant metastases, invasion of vessels, capsule, or adjacent organs, and tumor necrosis[atlasgeneticsoncology.org]
  • Treatment and prognosis Small adrenal masses with manifestations of hormonal excess require resection, as do large ( 3-5 cm) non-functioning adrenal mass lesions, as they are considered potentially malignant (see adrenal carcinoma ).[radiopaedia.org]
  • Prognosis The usual prognosis of malignant pheochromocytomas is about 50%/5-year survival. Some patients may have indolent disease with a life expectancy of more than 20 years.[nature.com]

Etiology

  • We discuss the etiology of myelolipoma and suggest that myelolipoma can develop in the course of endocrine hyperfunction.[ncbi.nlm.nih.gov]
  • An explanation of the etiology of the tumor is suggested by the unusual clinical course. Past History A. A., a Caucasian infant was born September 8, 1950, to a 31-year-old mother.[pediatrics.aappublications.org]
  • […] hormone (ACTH), blood glucose Virilization: DHEA, DHEA-sulfate (DHEA-S), androstenedione, testosterone, DHT urinary 17-ketosteroids (metabolic product) Feminization: androgens aromatization estrogen, estradiol urinary 17-ketosteroids (metabolic product) Etiology[pathologyoutlines.com]

Epidemiology

  • General Epidemiology: Often an incidental finding. Pathologic/clinical: May be hormonally active.[librepathology.org]
  • Each entity is extensively discussed with information on clinicopathological, epidemiological, immunophenotypic and genetic aspects of these diseases. The book is an authoritative, concise reference, prepared by 150 authors from 20 countries.[books.google.de]
  • Terminology Adrenal cortical adenoma (ACA) Incidentaloma: small adenoma discovered incidentally during workup of other conditions ( Mod Pathol 2011;24:S58 ) Black (pigmented) adenoma: diffusely pigmented, brown-black ACA presumably due to lipofuscin Epidemiology[pathologyoutlines.com]
  • Epidemiology Adrenal cancer is a rare malignancy representing only 0.05% and 0.2% of all cancers worldwide. Women tend to develop functional adrenal cortical carcinomas more commonly than men.[atlasgeneticsoncology.org]
Sex distribution
Age distribution

Pathophysiology

  • A review of the relevant literature and discussion of the pathophysiology and clinical implications is provided.[ncbi.nlm.nih.gov]
  • More common in adults, 5th - 7th decade Equal predilection for right and left adrenal glands True incidence unknown because many are not functional, estimates include 8.7% in autopsy series and 4% in radiology series ( Mol Cell Endocrinol 2014;386:67 ) Pathophysiology[pathologyoutlines.com]
  • ., : Pheochromocytoma, in, (eds): Hypertension: Pathophysiology, Diagnosis, and Management Raven Press, New York, 1990, pp 1639 – 1659. 9., : Management of pheochromocytoma.[academic.oup.com]
  • Evolving concepts in the pathophysiology, diagnosis and treatment of pheochromocytoma. Endocr Rev 1994; 15 :356–368. 43. Shapiro B, Sisson JC, Lloyd R, et al. Malignant pheochromocytoma: clinical, biochemical and scintigraphic characterization.[nature.com]
  • Evolving concepts in the pathophysiology, diagnosis and treatment of pheochromocytoma . Endocr Rev 1994; 15 :356–368. 43. Shapiro B , Sisson JC , Lloyd R , et al .[nature.com]

Prevention

  • Evaluation should be performed on this co-occurrence to prevent perioperative complications from resection of an unexpected secretory cortical neoplasm.[ncbi.nlm.nih.gov]
  • […] staining for MELAN-A, Syn (46 of 50 cases of ACA), NSE (44 of 50 cases of ACA), Vim (42 of 50 cases of ACA) and Ki-67 Conclusion: Prediction of endocrine syndrome in functional ACA was possible based on its structure and morphologic features, which could prevent[dovepress.com]
  • Benign adrenal tumor care at Mayo Clinic Clinical trials Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this disease.[mayoclinic.org]
  • How can I prevent adrenal cancer? There are no preventable risk factors associated with adrenal cancer so there is no way to prevent adrenal cancer.[oncolink.org]

References

Article

  1. Cawood TJ, Hunt PJ, O'Shea D, Cole D, Soule S. Recommended evaluation of adrenal incidentalomas is costly, has high false-positive rates and confers a risk of fatal cancer that is similar to the risk of the adrenal lesion becoming malignant; time for a rethink? Eur J Endocrinol. 2009;161(4):513-527.
  2. Nieman LK. Approach to the patient with an adrenal incidentaloma. J Clin Endocrinol Metab. 2010;95(9):4106-4113.
  3. Cruz DN, Perazella MA. Hypertension and hypokalemia: unusual syndromes. Conn Med. 1997;61(2):67-75.
  4. Chiodini I, Morelli V, Masserini B, et al. Bone mineral density, prevalence of vertebral fractures, and bone quality in patients with adrenal incidentalomas with and without subclinical hypercortisolism: an Italian multicenter study. J Clin Endocrinol Metab. 2009;94(9):3207-3214.
  5. Terzolo M, Bovio S, Pia A, Reimondo G, Angeli A. Management of adrenal incidentaloma. Best Pract Res Clin Endocrinol Metab. 2009;23(2):233-243.
  6. Zeiger MA, Thompson GB, Duh QY. The American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons medical guidelines for the management of adrenal incidentalomas.Endocr Pract. 2009;15(Suppl1):1-20.
  7. Young WF Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med. 2007;356(6):601-610.
  8. Toniato A, Merante-Boschin I, Opocher G, et al. Surgical versus conservative management for subclinical Cushing syndrome in adrenal incidentalomas: a prospective randomized study. Ann Surg. 2009;249(3):388-391.
  9. Emral R, Uysal AR, Asik M, et al. Prevalence of subclinical Cushing's syndrome in 70 patients with adrenal incidentaloma: clinical, biochemical and surgical outcomes. Endocr J. 2003;50(4):399-408.
  10. Szolar DH, Korobkin M, Reittner P, et al. Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT. Radiology. 2005;234(2):479-485.
  11. Yun M, Kim W, Alnafisi N, et al. 18F-FDG PET in characterizing adrenal lesions detected on CT or MRI. J Nucl Med. 2001;42(12):1795-1799.
  12. Mazzaglia PJ, Monchik JM. Limited value of adrenal biopsy in the evaluation of adrenal neoplasm: a decade of experience. Arch Surg. 2009;144(5):465-470.

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Last updated: 2019-06-28 10:19