Name = Adrenocortical carcinoma
Caption = Metastasis of an adrenocortical carcinoma to the lung
ICD10 = ICD10|C|74|0|c|73
ICD9 = ICD9|194
ICDO = 8370/3
eMedicineSubj = ped
eMedicineTopic = 41
MeshID = D018268
Adrenocortical carcinoma, also adrenal cortical carcinoma (ACC) and adrenal cortex cancer, is an aggressive
canceroriginating in the cortex ( steroid hormone-producing tissue) of the adrenal gland. Adrenocortical carcinoma is a rare tumor, with incidence of 1-2 per million population annually.cite book |author=edited by Vincent T. DeVita, Samuel Hellman, Steven A. Rosenberg |title=Cancer: principles & practice of oncology |publisher=Lippincott-Raven |location=Philadelphia |year=2005 |pages= |isbn=0-7817-4865-8 |oclc= |doi=] cite web | last =Savarese | first =Diane MF | coauthors =Lynnette K Nieman| title =Clinical presentation and evaluation of adrenocortical tumors| work =UpToDate Online v. 15.1| publisher =UpToDate| date = 2006-08-08| url =http://www.uptodateonline.com/utd/content/topic.do?topicKey=adrenal/17707&type=A&selectedTitle=1~16 | accessdate =2007-06-05] Adrenocortical carcinoma has a bimodal distribution by age, with cases clustering in children under 6, and in adults 30-40 years old. Adenocortical carcinoma is remarkable for the many hormonal syndromes which can occur in patients with steroid hormone-producing ("functional") tumors, including Cushing's syndrome, Conn syndrome, virilization, and feminization. Adrenocortical carcinoma has often invaded nearby tissues or metastasized to distant organs at the time of diagnosis, and the overall 5-year survival rate is only 20-35%.
Signs and Symptoms
Adrenocortical carcinoma may present differently in children and adults. Most tumors in children are functional, and
virilizationis by far the most common presenting symptom, followed by Cushing's syndromeand precocious puberty. Among adults presenting with hormonal syndromes, Cushing's syndrome alone is most common, followed by mixed Cushing's and virilization ( glucocorticoidand androgenoverproduction). Feminizationand Conn syndrome( mineralcorticoidexcess) occur in less than 10% of cases. Rarely, pheochromocytoma-like hypersecretion of catecholamines has been reported in adrenocortical cancers.cite book |author=Richard Cote, Saul Suster, Lawrence Weiss, Noel Weidner (Editor) |title=Modern Surgical Pathology (2 Volume Set) |publisher=W B Saunders |location=London |year= |pages= |isbn=0-7216-7253-1 |oclc= |doi=] Non-functional tumors (about 40%, authorities vary) usually present with abdominal or flank pain, or they may be asymptomatic and detected incidentally.
All patients with suspected adrenocortical carcinoma should be carefully evaluated for signs and symptoms of hormonal syndromes. For Cushing's syndrome (
glucocorticoidexcess) these include weight gain, muscle wasting, purple lines on the abdomen, a fatty "buffalo hump" on the neck, a "moonlike" face, and thinning, fragile skin. Virilism ( androgenexcess) is most obvious in women, and may produce excess facial and body hair, acne, enlargement of the clitoris, deepening of the voice, coarsening of facial features, and cessation of menstruation. Conn syndrome( mineralcorticoidexcess) is marked by high blood pressure, which can result in headache, and hypokalemia(low serum potassium), which can produce muscle weakness, confusion, and palpitations. low plasma reninactivity, and high serum aldosterone. Feminization( estrogenexcess) is most readily noted in men, and includes breast enlargement, decreased libidoand impotence. [Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL. "Harrison's Principles of Internal Medicine". New York: McGraw-Hill, 2005. ISBN 0-07-139140-1]
Hormonal syndromes should be confirmed with laboratory testing. Laboratory findings in Cushing syndrome include increased
serum glucose(blood sugar) and increased urine cortisol. Adrenal virilism is confirmed by the finding of an excess of serum androstenedioneand dehydroepiandrosterone. Findings in Conn syndromeinclude low serum potassium, low plasma reninactivity, and high serum aldosterone. Feminization is confirmed with the finding of excess serum estrogen
Radiological studies of the
abdomen, such as CT scans and magnetic resonance imagingare useful for identifying the site of the tumor, differentiating it from other diseases, such as adrenocortical adenoma, and determining the extent of invasion of the tumor into surrounding organs and tissues. CT scans of the chestand bone scans are routinely performed to look for metastases to the lungs and bones respectively. These studies are critical in determining whether or not the tumor can be surgically removed, the only potential cureat this time.
Adrenal tumors are often not biopsied prior to surgery, so diagnosis is confirmed on examination of the surgical specimen by a pathologist. Grossly, adrenocortical carcinomas are often large, with a tan-yellow cut surface, and areas of
hemorrhageand necrosis. On microscopic examination, the tumor usually displays sheets of atypical cells with some resemblance to the cells of the normal adrenal cortex. The presence of invasionand mitotic activity help differentiate small cancers from adrenocortical adenomas.cite book |author=Richard Cote, Saul Suster, Lawrence Weiss, Noel Weidner (Editor) |title=Modern Surgical Pathology (2 Volume Set) |publisher=W B Saunders |location=London |year= |pages= |isbn=0-7216-7253-1 |oclc= |doi=] There are several relatively rare variants of adrenal cortical carcinoma: Oncocytic adrenal cortical carcinoma, Myxoid adrenal cortical carcinoma, Carcinosarcoma, Adenosquamous adrenocortical carcinoma, Clear cell adrenal cortical carcinoma.
Differential diagnosis includes:
Adrenocortical adenoma, Renal cell carcinoma, Adrenal medullary tumors, Hepatocellular carcinoma.
The only curative treatment is complete surgical excision of the tumor, which can be performed even in the case of invasion into large blood vessels, such as the
renal veinor inferior vena cava. The 5-year survival rate after successful surgery is 50-60%, but unfortunately, a large percentage of patients are not surgical candidates. Radiation therapyand radiofrequency ablationmay be used for palliation in patients who are not surgical candidates. Chemotherapyregimens typically include the drug mitotane, an inhibitor of steroidsynthesis which is toxic to cells of the adrenal cortex,cite book |author=Laurence L. Brunton, editor-in-chief;John S. Lazo and Keith L. Parker, Associate Editors |title=Goodman & Gilman's The Pharmacological Basis of Therapeutics, 11th Edition |publisher=The McGraw-Hill Companies, Inc. |location=United States of America |year=2006 |pages= |isbn=0-07-142280-3 |oclc= |doi= ] as well as standard cytotoxic drugs. One widely used regimen consists of cisplatin, doxorubicin, etoposide) and mitotane. The endocrine cell toxin streptozotocinhas also been included in some treatment protocols. Chemotherapy may be given to patients with unresectable disease, to shrink the tumor prior to surgery ( neoadjuvant chemotherapy), or in an attempt to eliminate microscopic residual disease after surgery ( adjuvant chemotherapy).
Hormonal therapy with steroid synthesis inhibitors such as
aminoglutethimidemay be used in a palliative manner to reduce the symptoms of hormonal syndromes.
ACC, generally, carries a poor prognosiscite journal | author = Allolio B, Fassnacht M | title = Clinical review: Adrenocortical carcinoma: clinical update | journal = J Clin Endocrinol Metab | volume = 91 | issue = 6 | pages = 2027–37 | year = 2006 | pmid = 16551738 | doi = 10.1210/jc.2005-2639 [http://jcem.endojournals.org/cgi/content/full/91/6/2027 Free Full Text] .] and is unlike most tumours of the adrenal cortex, which are
benign( adenomas) and only occasionally cause Cushing's syndrome. Five-year disease-free survival for a complete resection of a stage I-III ACC is approximately 30%.The most important prognostic factors are age of the patient and stage of the tumor.Poor prognostic factors: mitotic activity, venous invasion, weight of 50g+; diameter of 6.5 cm+, Ki-67/MIB1 labeling index of 4%+, p53+.
* [http://www.endotext.org/adrenal/adrenal22/adrenalframe22.htm www.endotext.org article]
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