<unk>  <unk>  is a rare endocrine malignancy, often with an unfavorable prognosis. It originates from the adrenal cortex. In 1893, Grawitz et al was the first one who described<unk>  and falsely assumed it to be a hypernephroma. By 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<unk> .<unk>  may be classified<unk> ording to hormone production and histological appearance.<unk> s are typically large tumors upon clinical presentation, often measuring more than 6 cm in diameter. They are bilateral in 2% to 10% of cases. Genetic basis of<unk>  depends on genomic aberrations that contribute to neoplastic transformation of<unk>  cells such as clonality, gene expression arrays, microRNAs, gene mutations, chromosomal aberrations, epigenetic changes. Intracellular signaling depends on suggested three pathways: IGF pathway, WNT signaling pathway, Vascular endothelial growth factor pathway. On gross pathology, a large tan-yellow surface with areas of hemorrhage and necrosis is a characteristic finding of<unk> . On microscopic histopathological analysis, sheets of atypical cells with some resemblance to the cells of the normal adrenal cortex are a characteristic finding of<unk> .<unk>  may be associated with other neoplastic syndromes such as Lynch syndrome, Beckwith-Wiedemann syndrome (BWS), Carney complex, Neurofibromatosis type 1. There are no established causes for<unk> .<unk>  must be differentiated from other diseases such as<unk>  adenoma, adrenal metastasis, adrenal medullary tumors, and Cushing's syndrome. The incidence of<unk>  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 the sixth decade. There is a predilection for the female gender. The relatively increased incidence in childhood is mainly explained by germline TP53 mutations, which are the underlying genetic cause of<unk>  in >50% to 80% of children. The most potent risk factors in the development of<unk>  cancer are Lynch syndrome, Beckwith-Wiedemann syndrome, Carney complex, Neurofibromatosis type 1, Multiple endocrine neoplasia type 1 (MEN1), and Caney Complex. Screening is not recommended for<unk> . If left untreated, patients with<unk>  may progress to develop hyperglycemia, osteoporosis, delayed wound healing, hypertension, Cerebrovascular disease, and local or distant metastasis. Prognosis is generally poor, and the 5-year survival rate of patients with<unk>  stage I-III is approximately 30%. Complications may include metastasis, Conn's syndrome and Cushing'a syndrome.<unk> ording to the TNM staging system, there are four stages of<unk>  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. Symptoms of<unk>  include symptoms of androgen, glucocorticoid, mineralocorticoid, or estrogen excess. Symptoms of glucocorticoid excess include weight gain, acne, irritability. Symptoms of androgen excess symptoms include hirsutism, acne, and deepening of the voice.  Symptoms of mineralocorticoid excess include headache, muscle weakness, confusion, palpitations. Common physical examination findings of<unk>  include Cushing's syndrome findings such as hypertension, weakness, gynecomastia, and acne. Hyperandrogenic cases may show findings such as clitoromegaly and hirsutism. Some patients with<unk>  may have elevated concentrations of serum cortisol, aldosterone, testosterone or estrogen and reduced concentration of plasma renin and potassium. There are no findings associated with<unk> . Adrenal CT scan may be helpful in the diagnosis of<unk>  <unk>  and differentiating it from other diseases, such as<unk>  adenoma. Signs such as Internal hemorrhage, calcifications, CT density > 10 HU or necrosis increase the chances of<unk> . Contrast-enhanced CT scan is a reliable method of disease staging, identifying common metastatic sites such as regional and para-aortic lymph nodes, lungs, liver, and bones.CT imaging of the chest, liver, and bone scan are used for staging workup to detect metastasis. 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. 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. Inferior vena cava invasion has been reported in 9% to 19% of cases at presentation. Intraoperative and intravascular ultrasound may be used for metastatic deposits recognition. Adrenal angiography, venography, positron emission tomography and MIBG may be used in the diagnosis of<unk> . The sensitivity of FDG PET/CT was 90% for the diagnosis of metastases as compared with 88% for diagnostic CT. FDG PET/CT is a useful modality for staging<unk>  and evaluating local recurrence. FNA cytology cannot distinguish a benign adrenal mass from adrenal carcinoma. Overexpression of TP53, IGF-2, and cyclin E are found in<unk>  but not a conclusive procedure. Chemotherapy and hormonal therapy may be required in the treatment of<unk> . Mitotane is the only approved drug in the U.S. until now. Mitotane causes a destruction of the inner zones of the adrenal cortex, the zona fasciculata, and zona reticularis. Other drugs such as ketoconazole, metyrapone, aminoglutethimide, etomidate, and mifepristone can be used also. Target therapy such as sunitinib is IGF-1R antagonists that also may be effective. Surgery is the mainstay of treatment for<unk> . Appropriate preoperative evaluation and operative planning are the most important to assure the best outcome. Lymph nodes should be removed as part of the en bloc resection.  Recurrence in the peritoneum outside the tumor bed having the worst survival. Surgery is indicated in those patients with disease confined to one site or organ. Radiation therapyand radiofrequency ablation may be used for palliation in patients who are not surgical candidates. Recurrence is lower in the patient with adjuvant radiotherapy than in patients without radiotherapy.<unk>  with metastasis to bone experienced adequate pain relief after radiotherapy.
Adrenocortical carcinoma
Adrenal myelolipoma
Adrenal carcinoma
Renal oncocytoma
Metanephric adenoma
Adrenolipoma
Oncocytoma
Pineocytoma