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

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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] Mohammed Abdelwahed M.D[4]

Overview

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, often with an unfavorable prognosis. It originates from the adrenal cortex. ACCs may be functional and secret many hormones causing specific syndromes include Cushing's syndrome, Conn's syndrome, virilization, and feminization. Adrenocortical carcinoma invades nearby tissues or metastasize to distant organs at the time of diagnosis. The overall 5-year survival rate is only 20-35%. On gross pathology, a large tan-yellow surface with areas of hemorrhage and necrosis is a characteristic finding of adrenocortical carcinoma. On microscopic 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 (ACC) and differentiating it from other diseases, such as adrenocortical adenoma. Signs such as Internal hemorrhagecalcifications. MRI scans are helpful in differentiating between adrenal adenoma, carcinoma, and metastatic lesions. Due to the multiplanar capability of MRI, direct invasion of adjacent organs may be better shown. Surgery is the mainstay of treatment for adrenocortical carcinoma. Chemotherapy and hormonal therapy may be required in treatment of adrenocortical carcinoma.

Historical perspective

In 1893, Grawitz et al was the first one described ACC and falsely assumed it a hypernephroma. ByBy 1938, the Mayo group had removed tumors successfully from 16 consecutive patients, most of whom had Cushing’s syndrome. In 1960, mitotane was first used clinically to treat inoperable or recurrent ACC.

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. The relatively increased incidence in childhood is mainly explained by germline TP53 mutations, which are the underlying genetic cause of ACC in >50% to 80% of children.

Differentiating Adrenal Carcinoma from other Diseases

Adrenocortical carcinoma must be differentiated from other diseases such as adrenocortical adenoma, adrenal metastasisadrenal medullary tumors, and Cushing's syndrome.

Epidemiology and Demographics

The incidence of adrenocortical carcinoma is believed to be 0.72 per million cases per year leading to 0.2% of all cancer deaths in the United States and 0.2 to 0.3 per million children per year worldwide but valid data are lacking. A bimodal distribution was observed, the first one in pediatrics and the second one in the fifth to sixth decade. There is a predilection for the female gender.

Risk Factors

The most potent risk factors in the development of adrenocortical cancer are Lynch syndrome, Beckwith-Wiedemann syndrome, Carney complex, Neurofibromatosis type 1, Multiple endocrine neoplasia type 1 (MEN1), and Carney complex.

Screening

Screening is not recommended for adrenocortical carcinoma.

Natural History, Complication and Prognosis

If left untreated, patients with adrenocortical carcinoma may progress to develop hyperglycemia, osteoporosis, delayed wound healing, hypertension, Cerebrovascular disease, and local or distant metastasisPrognosis is generally poor, and the 5-year survival rate of patients with adrenocortical carcinoma stage I-III is approximately 30%. Complications may include metastasisConn's syndrome and Cushing'a syndrome.

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 androgenglucocorticoidmineralocorticoid, or estrogen excess.

Physical Examination

Common physical examination findings of Adrenocortical carcinoma include hypertensionweakness, gynecomastia, and acne.

Laboratory Findings

Some patients with adrenocortical carcinoma may have elevated concentration of serum cortisolaldosteronetestosterone or estrogen and reduced concentration of plasma renin and potassium.

X-ray

There are no findings associated with adrenocortical carcinoma.

MRI

MRI scans are helpful in differentiating between adrenal adenoma, carcinoma, and metastatic lesions. Due to the multiplanar capability of MRI, direct invasion of adjacent organs may be better shown.

CT

Adrenal CT scan may be helpful in the diagnosis of Adrenocortical carcinoma (ACC) and differentiating it from other diseases, such as adrenocortical adenoma. Signs such as Internal hemorrhagecalcifications, CT density > 10 HU or necrosis increase the chances of ACC.

Other Imaging Studies

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

Biopsy

FNA cytology cannot distinguish a benign adrenal mass from adrenal carcinoma. Overexpression of TP53, IGF-2, and cyclin E are found in ACC but not a conclusive procedure.

Treatment

Medical Therapy

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

Surgery

Surgery is the mainstay of treatment for adrenocortical carcinoma.

References

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