Adrenocortical carcinoma
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| ICD-10 | C74.0 |
|---|---|
| ICD-9 | 194 |
| eMedicine | ped/41 |
Adrenocortical carcinoma, also adrenal cortical carcinoma (ACC) and adrenal cortex cancer, is a carcinoma of the cortex (outer layer) of the adrenal gland.
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[edit] Prognosis
ACC, generally, carries a poor prognosis[1] 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%.[1]
[edit] Prevalance
The malignant form makes up about 3% of all cortical tumors.
[edit] Treatment
The only curative treatment is complete surgical excision and often done with adjuvant chemotherapy. The role of radiation therapy has not been established.
Chemotherapy regimes include the Italian protocol (cisplatin, doxorubicin, etoposide) plus mitotane and streptozotocin plus mitotane.
[edit] Pathophysiology
Excess cortisol production may require suppression with ketoconazole or metyrapone. Production of aldosterone or androgens by carcinomas is extremely rare.
[edit] References
- ^ a b Allolio B, Fassnacht M (2006). "Clippnical review: Adrenocortical carcinoma: clinical update.". J Clin Endocrinol Metab 91 (6): 2027-37. PMID 16551738. Free Full Text.
[edit] External links
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