Non-neoplastic lesions were added in the 5th edition WHO classification of adrenal cortical tumor based on the recent update, including adrenal rests, adrenal cysts, congenital adrenal hyperplasia and adrenocortical nodular disease. A range of tumor concepts were updated or refined based on tumor cell origin, histopathology, oncology and molecular biology. The most significant nomenclature change in the field of adrenal cortical pathology involves the refined classification of adrenal cortical nodular disease, which now includes sporadic nodular adrenocortical disease, bilateral micronodular adrenal cortical disease, and bilateral macronodular adrenal cortical disease. The 5th edition WHO classification endorses the nomenclature of the HISTALDO classification to help the classification of aldosterone producing adrenal cortical lesions, which uses CYP11B2 immunohistochemistry to identify functional sites of aldosterone production. The 5th edition WHO classification does not change the Weiss and Lin-Weiss-Bisceglia histopathologic criteria for diagnosing adrenal cortical carcinomas, and underscores the diagnostic and prognostic impact of angioinvasion in these tumors. Reticulin algorithm and Helsinki scoring system were added to assist the differential diagnosis of adrenal cortical neoplasms in adults. Pediatric adrenal cortical neoplasms are assessed using the Wieneke system. The 5th edition WHO classification places an emphasis on an accurate assessment of tumor proliferation rate using both the mitotic count (mitoses per 10 mm²) and Ki-67 labeling index which play an essential role in the dynamic risk stratification of affected patients. This review highlights advances in knowledge of histological features, ancillary studies, and associated genetic findings that increase the understanding of the adrenal cortex pathologies in the 5th edition WHO classification.
Adult ; Humans ; Child ; Aldosterone ; Adrenal Cortex Neoplasms/chemistry* ; Adrenocortical Carcinoma/pathology* ; Adrenal Gland Neoplasms ; World Health Organization

OBJECTIVETo evaluate the effect of insulin-like growth factor (IGF) and its receptor-I antibody on the growth of human adrenocortical carcinoma SW-13 cell line in vitro.

METHODThe growth curves of SW-13 cell treated with IGF and its receptor-I antibody were obtained by means of MTT assay. The effects of the two agents, added either alone or in combination at different concentrations, on the cell growth were evaluated.

RESULTSIGF significantly promoted proliferation of SW-13 cells, and its effect was positively correlated with its concentrations (P<0.05). IGF receptor-I antibody inhibited the effect of insulin-like growth factor with direct inhibitory effect on proliferation of SW-13 cells (P<0.05).

CONCLUSIONIGF can promote the growth of human adrenocortical carcinoma SW-13 cells via its receptor-I. IGF receptor-I antibody can inhibit the effect of the growth factor, suggesting a possible role of this receptor in the treatment of adrenocortical carcinoma.

Adrenocortical Carcinoma ; pathology ; Antibodies ; pharmacology ; Cell Line, Tumor ; Cell Proliferation ; drug effects ; Dose-Response Relationship, Drug ; Humans ; Insulin-Like Growth Factor I ; pharmacology ; Receptor, IGF Type 1 ; immunology

Primary adrenocortical carcinoma (ACC) is a rare tumor and its usual sites of metastasis are the lung (71%), lymph node (68%), liver (42%), and bone (26%). However, intracaval invasion extending into the right atrium is very rare and spontaneous regression of tumor burden in adrenal carcinoma is also rare. We report a case of ACC with direct invasion of the inferior vena cava and right atrium. A 34-yr-old male patient presented with progressive dyspnea, weight loss, and poor oral intake over 3 months. Non-functioning ACC with direct invasion of the inferior vena cava and right atrium was confirmed by imaging, pathologic, and hormonal study. Chemo-radiotherapy was attempted. However, tumor burden was not changed, but rather toxic hepatitis and thrombocytopenia were developed. His subjective symptoms and general conditions were improved after 1 month of conservative management and the patient was discharged. During clinical follow-up, this tumor showed spontaneous regression.
Vena Cava, Inferior/*pathology ; Tomography, X-Ray Computed ; Remission Induction ; Neoplasm Metastasis ; Male ; Humans ; Heart Neoplasms/pathology/*secondary ; Heart Atria/*pathology ; Follow-Up Studies ; Echocardiography ; Biopsy ; Adult ; Adrenocortical Carcinoma/*diagnosis/*pathology ; Adrenal Cortex Neoplasms/*diagnosis/*pathology

OBJECTIVETo study the clinicopathologic features and expression of epidermal growth factor receptor (EGFR) and vascular endothelial growth factor (VEGF) in adrenocortical tumors.

METHODSForty-two cases of adrenocortical tumors operated at the Beijing Union Medical College Hospital during the period from July, 2001 to July, 2010 were retrospectively reviewed. Immunohistochemical study for EGFR and VEGF was carried out. The clinical information and follow-up data were analyzed.

RESULTSThe cases included 21 adrenocortical carcinomas (ACC) and 21 adrenocortical adenomas (ACA). Nine patients suffered from primary aldosterone syndrome, including 8 cases with ACA and 1 case with ACC. The average tumor size, tumor weight, and duration between disease onset and diagnosis in the 21 cases of ACC were 11.7 cm, 542 g and 8.5 months, respectively. This was in contrast to 3 cm, 9.8 g and 45.6 months, respectively in cases of ACA. Histologically, the WEISS score in all the 21 cases of ACA was ≤ 2 (average = 0.9). None of the ACC cases had score less than 4 (average = 6.6). The presence of sinus invasion correlated with tumor metastasis (P < 0.01). Immunohistochemical study showed that EGFR was expressed in 61.9% of ACC patients (13/21), whereas EGFR staining was mostly negative in ACA (except for weak staining in 5 cases and moderate staining in 1 case). The difference of EGFR expression between ACC and ACA was statistically significant (P = 0.030). On the other hand, the positive rate of VEGF in ACC was 71.4% (15/21), including 28.6% (6/21) with strong expression and 28.6% (6/21) with moderate expression. In contrast, the expression rate of VEGF in ACA was 30.0% (7/21), including 14.3% (3/21) with moderate expression. The difference of VEGF expression between ACC and ACA was statistically significant (P = 0.013). There was correlation between VEGF expression and venous invasion (P = 0.028). The average duration of survival in patients with ACC was shorter than that in ACA. The tumor weight in ACC also correlated with prognosis.

CONCLUSIONSTumor size, weight and presence of endocrine symptoms may help in the differential diagnosis between ACC and ACA. A WEISS score of ≥ 3 highly suggests ACC. The presence of sinus invasion is associated with metastasis. EGFR or VEGF expression may also be important in differentiating ACC from ACA.

Adolescent ; Adrenal Cortex Neoplasms ; metabolism ; pathology ; surgery ; Adrenocortical Adenoma ; metabolism ; pathology ; surgery ; Adrenocortical Carcinoma ; metabolism ; pathology ; surgery ; Adult ; Aged ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Receptor, Epidermal Growth Factor ; metabolism ; Retrospective Studies ; Survival Rate ; Tumor Burden ; Vascular Endothelial Growth Factor A ; metabolism ; Young Adult

Actins ; metabolism ; Adrenal Cortex Neoplasms ; metabolism ; pathology ; surgery ; Adrenocortical Adenoma ; metabolism ; pathology ; surgery ; Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; surgery ; Follow-Up Studies ; Hemangioma ; metabolism ; pathology ; surgery ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neoplasms, Multiple Primary ; metabolism ; pathology ; surgery ; Nephrectomy ; Pelvic Neoplasms ; metabolism ; pathology ; surgery