Adrenocortical carcinoma overview

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Adrenocortical carcinoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

MRI

CT

Ultrasound

Other Imaging Studies

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Radiation Therapy

Cost-Effectiveness of Therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Ahmad Al Maradni, M.D. [3]

Overview

Adrenocortical carcinoma, also adrenal cortical carcinoma (ACC) and adrenal cortex cancer, is an aggressive cancer originating in the cortex (steroid hormone-producing tissue) of the adrenal gland. 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%. There are no established causes for adrenocortical carcinoma. On gross pathology, a large tan-yellow surface with areas of hemorrhage and necrosis is a characteristic finding of adrenocortical carcinoma. On microscopic histopathological analysis, sheets of atypical cells with some resemblance to the cells of the normal adrenal cortex are a characteristic finding of adrenocortical carcinoma. Adrenal CT scan may be helpful in the diagnosis of adrenocortical carcinoma. Surgery is the mainstay of treatment for adrenocortical carcinoma. Chemotherapy and radiation may be required in treatment of adrenocortical carcinoma.

Classification

Adrenocortical carcinoma is classified according to hormone production and histological appearance.

Pathophysiology

On gross pathology, a large tan-yellow surface with areas of hemorrhage and necrosis is a characteristic finding of adrenocortical carcinoma. On microscopic histopathological analysis, sheets of atypical cells with some resemblance to the cells of the normal adrenal cortex are a characteristic finding of adrenocortical carcinoma.

Causes

There are no established causes for adrenocortical carcinoma.

Differentiating Adrenal Carcinoma from other Diseases

Adrenocortical carcinoma must be differentiated from other diseases such as adrenocortical adenoma, renal cell carcinoma, adrenal medullary tumors and hepatocellular carcinoma.

Epidemiology and Demographics

Adrenocortical carcinoma is a rare cancer that tends to affect children and young adults.

Risk Factors

The most potent risk factors in the development of adrenocortical cancer are TP53 mutation, Beckwith-Wiedemann syndrome and Carney complex.[1]

Screening

Natural History, Complication and Prognosis

Prognosis is generally poor, and the 5-year survival rate of patients with adrenocortical carcinoma stage I-III is approximately 30%.

Diagnosis

Staging

According to the TNM staging system, there are four stages of adrenocortical cancer based on the tumor size, lymph nodes, and distant metastasis. Each stage is assigned a number and letter that designates the number of lymph nodes involved and presence/absence of distant metastasis.

History and Symptoms

Symptoms of adrenocortical carcinoma include symptoms of either androgen, glucocorticoid, mineralocorticoid, or estrogen excess.

Physical Examination

Common physical examination findings of adrenocortical carcinoma include, high blood pressure, weakness, gynecomastia and acne.

Laboratory Findings

Some patients with adrenocortical carcinoma may have elevated concentration of serum cortisol, aldosterone, testosterone or estrogen and reduced concentration of plasma renin and potassium.

MRI

MRI scans are helpful in differentiating between adrenal adenoma, carcinoma and metastatic lesions.

CT

Adrenal CT scan may be helpful in the diagnosis of adrenocortical carcinoma

Other Imaging Studies

Adrenal angiography,venography, positron emission tomography and MIBG may be used in the diagnosis of adrenocortical carcinoma.

Biopsy

Adrenocortical carcinomas are often not biopsied prior to surgery.

Treatment

Medical Therapy

Chemotherapy, hormonal therapy and radiation may be required in treatment of adrenocortical carcinoma.

Surgery

Surgery is the mainstay of treatment for adrenocortical carcinoma.

References

  1. National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq#section/_1

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