Adrenocortical carcinoma: Difference between revisions

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===Radiology===
===Radiology===


Radiological studies of the [[abdomen]], such as [[CT scan]]s and [[magnetic resonance imaging]] are 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 [[chest]] and [[bone scan]]s are routinely performed to look for [[metastasis|metastases]] to the [[lung]]s and [[bone]]s respectively. These studies are critical in determining whether or not the tumor can be [[surgery|surgically]] removed, the only potential cure at this time.<ref name="UTDACC"/>
====MRI====
===Pathology===
===Pathology===



Revision as of 20:18, 23 January 2012

Adrenocortical carcinoma
Metastasis of an adrenocortical carcinoma to the lung
ICD-10 C74.0
ICD-9 194

Adrenocortical carcinoma Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Signs and Symptoms

Diagnosis

Radiology

Pathology

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 hemorrhage and 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 invasion and mitotic activity help differentiate small cancers from adrenocortical adenomas.[1] 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

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.[2]

Chemotherapy regimens typically include the drug mitotane, an inhibitor of steroid synthesis which is toxic to cells of the adrenal cortex,[3] 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).[2]

Hormonal therapy with steroid synthesis inhibitors such as aminoglutethimide may be used in a palliative manner to reduce the symptoms of hormonal syndromes.[2]

Prognosis

ACC, generally, carries a poor prognosis[4] 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%.[4] 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. Richard Cote, Saul Suster, Lawrence Weiss, Noel Weidner (Editor). Modern Surgical Pathology (2 Volume Set). London: W B Saunders. ISBN 0-7216-7253-1.
  2. 2.0 2.1 2.2
  3. 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)
  4. 4.0 4.1 Allolio B, Fassnacht M (2006). "Clinical review: Adrenocortical carcinoma: clinical update". J Clin Endocrinol Metab. 91 (6): 2027–37. PMID 16551738. Free Full Text.

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