Find information on thousands of medical conditions and prescription drugs.

Adrenocortical carcinoma

Adrenocortical carcinoma is a carcinoma of the cortex (outer layer) of the adrenal gland. While most tumors of the adrenal cortex are benign (adenomas) and only occasionally cause Cushing's syndrome, the malignant form makes up about 3% of all cortical tumors and requires surgery and sometimes chemotherapy. Excess cortisol production may require suppression with ketoconazole or metyrapone. Production of aldosterone or androgens by carcinomas is extremely rare. Adrenocortical carcinomas are also sometimes referred to as adrenal cortical carcinomas, ACC, or adrenal cortex cancers. more...

Home
Diseases
A
Aagenaes syndrome
Aarskog Ose Pande syndrome
Aarskog syndrome
Aase Smith syndrome
Aase syndrome
ABCD syndrome
Abdallat Davis Farrage...
Abdominal aortic aneurysm
Abdominal cystic...
Abdominal defects
Ablutophobia
Absence of Gluteal muscle
Acalvaria
Acanthocheilonemiasis
Acanthocytosis
Acarophobia
Acatalasemia
Accessory pancreas
Achalasia
Achard syndrome
Achard-Thiers syndrome
Acheiropodia
Achondrogenesis
Achondrogenesis type 1A
Achondrogenesis type 1B
Achondroplasia
Achondroplastic dwarfism
Achromatopsia
Acid maltase deficiency
Ackerman syndrome
Acne
Acne rosacea
Acoustic neuroma
Acquired ichthyosis
Acquired syphilis
Acrofacial dysostosis,...
Acromegaly
Acrophobia
Acrospiroma
Actinomycosis
Activated protein C...
Acute febrile...
Acute intermittent porphyria
Acute lymphoblastic leukemia
Acute lymphocytic leukemia
Acute mountain sickness
Acute myelocytic leukemia
Acute myelogenous leukemia
Acute necrotizing...
Acute promyelocytic leukemia
Acute renal failure
Acute respiratory...
Acute tubular necrosis
Adams Nance syndrome
Adams-Oliver syndrome
Addison's disease
Adducted thumb syndrome...
Adenoid cystic carcinoma
Adenoma
Adenomyosis
Adenosine deaminase...
Adenosine monophosphate...
Adie syndrome
Adrenal incidentaloma
Adrenal insufficiency
Adrenocortical carcinoma
Adrenogenital syndrome
Adrenoleukodystrophy
Aerophobia
Agoraphobia
Agrizoophobia
Agyrophobia
Aicardi syndrome
Aichmophobia
AIDS
AIDS Dementia Complex
Ainhum
Albinism
Albright's hereditary...
Albuminurophobia
Alcaptonuria
Alcohol fetopathy
Alcoholic hepatitis
Alcoholic liver cirrhosis
Alektorophobia
Alexander disease
Alien hand syndrome
Alkaptonuria
Alliumphobia
Alopecia
Alopecia areata
Alopecia totalis
Alopecia universalis
Alpers disease
Alpha 1-antitrypsin...
Alpha-mannosidosis
Alport syndrome
Alternating hemiplegia
Alzheimer's disease
Amaurosis
Amblyopia
Ambras syndrome
Amelogenesis imperfecta
Amenorrhea
American trypanosomiasis
Amoebiasis
Amyloidosis
Amyotrophic lateral...
Anaphylaxis
Androgen insensitivity...
Anemia
Anemia, Diamond-Blackfan
Anemia, Pernicious
Anemia, Sideroblastic
Anemophobia
Anencephaly
Aneurysm
Aneurysm
Aneurysm of sinus of...
Angelman syndrome
Anguillulosis
Aniridia
Anisakiasis
Ankylosing spondylitis
Ankylostomiasis
Annular pancreas
Anorchidism
Anorexia nervosa
Anosmia
Anotia
Anthophobia
Anthrax disease
Antiphospholipid syndrome
Antisocial personality...
Antithrombin deficiency,...
Anton's syndrome
Aortic aneurysm
Aortic coarctation
Aortic dissection
Aortic valve stenosis
Apert syndrome
Aphthous stomatitis
Apiphobia
Aplastic anemia
Appendicitis
Apraxia
Arachnoiditis
Argininosuccinate...
Argininosuccinic aciduria
Argyria
Arnold-Chiari malformation
Arrhythmogenic right...
Arteriovenous malformation
Arteritis
Arthritis
Arthritis, Juvenile
Arthrogryposis
Arthrogryposis multiplex...
Asbestosis
Ascariasis
Aseptic meningitis
Asherman's syndrome
Aspartylglycosaminuria
Aspergillosis
Asphyxia neonatorum
Asthenia
Asthenia
Asthenophobia
Asthma
Astrocytoma
Ataxia telangiectasia
Atelectasis
Atelosteogenesis, type II
Atherosclerosis
Athetosis
Atopic Dermatitis
Atrial septal defect
Atrioventricular septal...
Atrophy
Attention Deficit...
Autoimmune hepatitis
Autoimmune...
Automysophobia
Autonomic dysfunction
Familial Alzheimer disease
Senescence
B
C
D
E
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Medicines

Complete surgical excision (if possible) is the primary treatment used for these neoplasms.

Read more at Wikipedia.org


[List your site here Free!]


Pathologic quiz case: An adrenal mass in a 54-year-old man--Pathologic diagnosis: Adrenocortical adenoma with spironolactone bodies
From Archives of Pathology & Laboratory Medicine, 7/1/03 by Stephany, Joshua D

An Adrenal Mass in a 54-Year-Old Man

A 54-year-old, 112-kg black man presented with primary hyperaldosteronism. His medical history was significant for multiple myeloma diagnosed 10 years earlier, difficult-to-control hypertension, glaucoma, hypothyroidism diagnosed 1.5 years earlier, and an intramuscular lipoma. His current medications included methadone 10 mg by mouth twice daily (PO BID), celecoxib 100 mg PO BID, gabapentin 300 mg PO once daily (QD), minoxidil 10 mg PO QD, atenolol 50 mg PO QD, levothyroxine 0.25 mg PO QD, and spironolactone 100 mg PO QD. On admission, he had a sodium (Na+) concentration of 135 mEq/L, a potassium (K+) concentration of 4.0 mEq/L, a chloride (Cl-) concentration of 94 mEq/L, and a bicarbonate (HCO-) concentration of 27 mEq/L. A computed tomography scan of the abdomen showed a modestly enlarged right adrenal gland with a 1.2-cm nodule. The patient underwent a right laparoscopic adrenalectomy. Intraoperative findings were severe perihepatitis and a right adrenal cortical nodule.

Grossly, the specimen was an 11.5-g, 7.0 X 3.0 X 2.0-- cm adrenal gland containing a 1.5 x 1.3 X 1.2-cm wellcircumscribed golden yellow nodule. Histologically, under low-power magnification, the lesion was well circumscribed with an alveolar or nesting pattern separated by fine microvasculature (Figure 1). On higher power magnification, populations of cells with variable morphology were identified. The more common populations were large cells that had a pale-staining cytoplasm and were admixed with smaller cells with less abundant but more eosinophilic cytoplasm. The most striking feature was the numerous concentric eosinophilic cytoplasmic inclusions throughout the lesion (Figure 2).

What is your diagnosis?

Pathologic Diagnosis: Adrenocortical Adenoma With Spironolactone Bodies

Primary hyperaldosteronism is usually caused by an adrenocortical adenoma (65%-88%) and, rarely, by an adrenocortical carcinoma. Patients are usually women in their fourth to fifth decades of life.1 Patients present with hypertension, averaging 200 mm Hg systolic and 120 mm Hg diastolic; weakness; hypokalemia; and lassitude.2 The diagnosis is made by clinical evaluations and laboratory criteria. Initial presentation will show hypokalemia with a potassium concentration of less than 4 mEq/L, a normal serum sodium concentration, low plasma renin activity, and elevated plasma and/or urine aldosterone concentrations. Once primary hyperaldosteronism is suspected, a computed tomography scan will differentiate between hyperplasia and an adenoma. The treatment of choice for a primary hyperaldosteronism due to a single nodule is a unilateral adrenalectomy, most commonly via laparoscopy.3

Grossly, the tumor is a yellow-orange, solitary, well-circumscribed nodule averaging 1.5 cm in diameter and is unencapsulated or incompletely encapsulated. Histologically, the tumor may have variable cell morphology, with the most common population of cells being large cells with pale cytoplasm and vesicular chromatin resembling cells from the zona fasciculata. Other cell populations may include smaller cells with eosinophilic cytoplasm and more condensed chromatin resembling cells from the zona reticularis.3

Patients treated with the aldosterone agonist spironolactone may have spironolactone bodies. Spironolactone is a potassium-sparing diuretic that acts on the distal tubules and collecting ducts. It competitively binds the aldosterone receptor, thus blocking the reabsorption of sodium.4 Spironolactone bodies are concentric laminated eosinophilic inclusions within the endoplasmic reticulum of the tumor cells or the adjacent zona glomerulosa. They range in size from 2 to 12 (mu)m, are periodic acid-Schiff positive and diastase resistant, and stain dark blue with Luxol fast blue They are seen while the patient is taking the medication and have no apparent correlation with dosage or duration of treatment. However, they are unlikely to be present when the medication is stopped for more than a day. Their origin is not known, but they react positively for aldosterone by immunohistochemistry.6

Although a rare neoplasm, a differential diagnosis of an adrenocortical carcinoma must be considered. It has a bimodal peak in age incidence occurring in the first decade of life and in the forth to fifth decades of life.7 Unlike the adrenal cortical adenoma, the average weight of the adrenal gland can range from 510 to 1200 g and measure from 12.0 to 16.0 cm.8 There are usually areas of focal hemorrhage and necrosis. The most common histologic pattern is that of broad anastomosing trabeculae with sinusoids. Nuclear pleomorphism, high mitotic activity, and atypical mitoses also help differentiate adrenocortical carcinoma from adrenocortical adenoma.7,8

References

1. Conn JW, Knopf RF, Nesbit RM. Clinical characteristics of primary aldosteronism from analysis of 14 cases. Am J Surg. 1964;107:159-172.

2. Neville AM, Symington T. Pathology of primary aldosteronism. Cancer. 1966;19:1854-1868.

3. Shen WT, Lim RC, Siperstein AE, et al. Laparoscopic vs open adrenalectomy for the treatment of primary hyperaldosteronism. Arch Surg. 1999;134:628-632.

4. Brater DC. Pharmacology of diuretics. Am J Med Sci. 2000;319:38-51.

5. Wenig BM, Heffess CS, Adair CF. Atlas of Endocrine Pathology. Philadelphia, Pa: WB Saunders Co; 1997.

6. Hsu S-M, Raine L, Martin HE Spironolactone bodies: an immunoperoxidase study with biochemical correlation. Am J Clin Pathol. 1981;75:92-95.

7. Lack EE, Mulvihill JJ, Travis WD, Kozakewich HPW. Adrenal cortical neoplasms in the pediatric and adolescent age group: clinicopathologic study of 30 cases with emphasis on epidemiological and prognostic factors. Pathol Annu. 1992;27:1-53.

8. Lack EE. Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia. Washington, DC: Armed Forces Institute of Pathology; 1997. Atlas of Tumor Pathology, 3rd series, fascicle 19.

Joshua D. Stephany, MD; Gary S. Pearl, MD, PhD

Accepted for publication November 21, 2002.

From the Department of Pathology, Orlando Regional Health Care, Orlando, Fla.

Corresponding author: Joshua D. Stephany, MD, 1414 Kuhl Ave, Orlando Regional Health Care, Department of Pathology, Orlando, FL 32806 (e-mail: jdsmd2@yahoo.com).

Reprints not available from the author.

Copyright College of American Pathologists Jul 2003
Provided by ProQuest Information and Learning Company. All rights Reserved

Return to Adrenocortical carcinoma
Home Contact Resources Exchange Links ebay