Adrenocortical carcinoma: Difference between revisions
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Revision as of 20:19, 23 January 2012
| Adrenocortical carcinoma | |
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| Metastasis of an adrenocortical carcinoma to the lung | |
| ICD-10 | C74.0 |
| ICD-9 | 194 |
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Adrenocortical carcinoma Microchapters |
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Differentiating Adrenocortical carcinoma from other Diseases |
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Diagnosis |
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Treatment |
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Case Study |
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Adrenocortical carcinoma On the Web |
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American Roentgen Ray Society Images of Adrenocortical carcinoma |
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Risk calculators and risk factors for Adrenocortical carcinoma |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Signs and Symptoms
Diagnosis
Radiology
Differential Diagnosis
- Adrenocortical adenoma
- Renal cell carcinoma
- Adrenal medullary tumors
- Hepatocellular carcinoma
Treatment
The only curative treatment is complete surgical excision of the tumor, which can be performed even in the case of invasion into large blood vessells, such as the renal vein or 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 therapy and radiofrequency ablation may be used for palliation in patients who are not surgical candidates.[1]
Chemotherapy regimens typically include the drug mitotane, an inhibitor of steroid synthesis which is toxic to cells of the adrenal cortex,[2] as well as standard cytotoxic drugs. One widely used regimen consists of cisplatin, doxorubicin, etoposide) and mitotane. The endocrine cell toxin streptozotocin has 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).[1]
Hormonal therapy with steroid synthesis inhibitors such as aminoglutethimide may be used in a palliative manner to reduce the symptoms of hormonal syndromes.[1]
Prognosis
ACC, generally, carries a poor prognosis[3] 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%.[3] 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+.
References
- ↑ 1.0 1.1 1.2
- ↑ Laurence L. Brunton, editor-in-chief;
John S. Lazo and Keith L. Parker, Associate Editors (2006). Goodman & Gilman's The Pharmacological Basis of Therapeutics, 11th Edition. United States of America: The McGraw-Hill Companies, Inc. ISBN 0-07-142280-3. line feed character in
|author=at position 38 (help) - ↑ 3.0 3.1 Allolio B, Fassnacht M (2006). "Clinical review: Adrenocortical carcinoma: clinical update". J Clin Endocrinol Metab. 91 (6): 2027–37. PMID 16551738. Free Full Text.