Adrenocortical carcinoma overview
|
Adrenocortical carcinoma Microchapters |
|
Differentiating Adrenocortical carcinoma from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Study |
|
Adrenocortical carcinoma overview On the Web |
|
American Roentgen Ray Society Images of Adrenocortical carcinoma overview |
|
Risk calculators and risk factors for Adrenocortical carcinoma overview |
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. 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 androgen, glucocorticoid, mineralocorticoid, or estrogen excess.
Physical Examination
Common physical examination findings of adrenocortical carcinoma include, high blood pressure, weakness, gynecommastia 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
- ↑ National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq#section/_1