From September 1980 to November 1989, eight patients with adrenal tumors had been admitted to our Department of Urology, Chonbuk National University hospital. Nine tumors consisted of 5 adrenal adenomas, 2 pheochromocytomas. a neuroblasloma and adrenocortical cancer. A clinicopathological study was made and the results were obtained as follows : 1. The 8 patients in these series included 2 men and 6 women. Age was ranged from 12 years to 47 years at the lime of presentation (average ; 27.5 years). 2. Four tumors occurred in the right adrenal gland and 5 in the left including a case of bilateral pheochromocytoma. 3. In nonfunctioning tumor (a case of adrenal denoma), the main clinical manifestations were localizing symptoms and signs such as abdominal pain, palpable mass and nonspecific laboratory findings. The others were functioning tumors (2 cases of Cushing`s syndrome due to adrenal adenoma and adrenocortical carcinoma, 2 cases of primary aldosteronism due to drenal demoma, a case of neuroblastoma and 2 cases of pheochromocytoma). There were unique generalized symptoms, signs and laboratory findings due to overproduction of hormones by the tumors 4. Nine adrenalectomies in 8 patients were performed. The weight of resected adrenal mass was 330gm in nonfunctioning adrenal adenoma, 190gm in adrenocortical carcinoma, 5.2gm in Cushing`s syndrome due to derenal adenoma, 2.5gm and 3.2gm in aldosteronism due to adrenal adenoma, 960gm in neurobladstoma, and 85gm, 20gm and 75gm in phepchromocytoma. 5. Results of hormonal assays which were checked 2 days and 2 weeks postoperatively returned to almost normal levels 6. Final characreristic of adrenal tumors could be made mostly by clinical diagnostic procedures, but some of underlying diseases should be differentiated by pathologic confirmation.
Abdominal Pain ; Adenoma ; Adrenal Cortex Neoplasms ; Adrenal Glands ; Adrenalectomy ; Adrenocortical Carcinoma ; Female ; Humans ; Hyperaldosteronism ; Jeollabuk-do ; Male ; Neuroblastoma ; Pheochromocytoma ; Urology

We report a case of an adenoma arising from an ectopic adrenal gland mimicking a hepatocellular carcinoma in a heavy alcohol abuser. A MDCT showed a 2.7 low-attenuating nodule in segment VII of the liver through all CT phases. Compared to a precontrast image, however, a subtle enhancement was noted on the arterial phase CT image. On T1 weighted in- and opposed-phase MR images, an abundant fat component within the lesion was seen. Dynamic contrast-enhanced MR images after administration of gadolinium ethoxybenzyl diethylenetriaminepentaacetic acid (Gd-EOB-DTPA) more clearly depicted hypervascularity and wash-out of the lesion on arterial and portal phases, respectively. On delayed hepatobiliary phase MR images, obtained 20 minutes after Gd-EOB-DTPA administration, subtle uptake or retention of the contrast agent by the lesion was suspected. A tumorectomy was performed and adrenal adenoma from an ectopic adrenal gland within the liver was confirmed.
Adrenal Cortex Neoplasms/*diagnosis ; Adrenal Glands ; Adrenocortical Adenoma/*diagnosis ; Carcinoma, Hepatocellular/diagnosis ; Choristoma/*diagnosis ; *Contrast Media ; Diagnosis, Differential ; Gadolinium DTPA/*diagnostic use ; Humans ; Liver Neoplasms/*diagnosis ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; *Tomography, X-Ray Computed

Adrenal Cortex Neoplasms ; complications ; Adrenal Gland Neoplasms ; complications ; Adrenal Glands ; pathology ; Adrenocortical Adenoma ; complications ; Humans ; Pheochromocytoma ; complications

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

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

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of adrenal cortical carcinoma (ACC) in children. Methods: Twenty-five children with ACC diagnosed in the Department of Pathology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China from March 2014 to August 2022 were retrospectively analyzed. The related literature was reviewed. Results: A total of 25 children with ACC were collected, including 11 males and 14 females, with a male to female ratio of 1.0∶1.3. The patient ages ranged from 8 months to 14 years (median, 4 years). Eighteen cases with clinical data had functional tumors (18/22, 81.8%) presenting with virilization or precocious puberty (15/18), symptoms related to hypercortisolism (8/18) or endocrine symptoms mixed with both (5/18), while 3 cases (3/22, 13.6%) had unknown clinical data. The clinical manifestations of four patients with nonfunctional tumors were an abdominal mass and/or abdominal pain, walking instability and others. Grossly, the average maximum diameter of the tumor was 9.4 cm. Most of the tumors were nodular and partially encapsuled. The cut surfaces were gray or gray brown, soft with hemorrhage. Histologically, the tumor cells were diffusely distributed, separated by a vascular-rich network. The tumor cells were large, with distinct nucleoli, abundant eosinophilic or clear cytoplasm, and round or oval nuclei. The mitotic index was high, and atypical mitoses were common. Necrosis, calcification, capsule invasion or/and venous invasion were present. In some cases, the tumor invaded the surrounding soft tissues or kidneys. Immunohistochemically, the tumor cells were diffusely positive for syn and SF1 and focally positive for α-inhibin, Melan A and Calretinin, but negative for CgA. Ki-67 proliferation index ranged from 2%-90%. TP53 gene status was examined in 7 cases, in which mutations were detected in 4 cases. Follow-up data was obtained in 21 patients, among whom 18 received chemotherapy and 3 received radiotherapy. Distant metastasis occurred in 13 patients. Median progression-free survival (PFS) was 11.2 months and median overall survival (OS) was 54.7 months. Patients aged less than 5 years had a better prognosis for OS (P<0.05) than the older ones (≥5 years), but a similar PFS (P>0.05). Male patients and Ki-67 proliferation index <15% had a better prognosis tendency for OS, but there was no statistically significant difference (P>0.05). Conclusions: ACC in children is a rare, often functional tumor associated with Li-Fraumeni genetic syndrome and has a poor prognosis. Diagnosis and differential diagnosis require a combination of morphological, phenotypic and clinical analysis.
Child ; Humans ; Male ; Female ; Infant ; Adrenocortical Carcinoma ; Retrospective Studies ; Ki-67 Antigen ; China ; Adrenal Cortex Neoplasms

Adenoma, Oxyphilic/surgery* ; Adrenal Cortex Neoplasms/surgery* ; Adrenocortical Adenoma/surgery* ; Humans

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

Adrenal Cortex Neoplasms ; diagnosis ; pathology ; Adrenocortical Adenoma ; diagnosis ; pathology ; Aged ; Humans ; Immunohistochemistry ; Male ; Myxoma ; diagnosis ; pathology

OBJECTIVETo investigate the expression of telomerase in various lesions of adrenal cortex.

METHODSBy autoradiography-based telomeric repeat amplification protocol, telomerase expression was detected in 36 samples of adrenocortical lesions, including 29 cases adrenocortical adenoma (8 Cushing's syndrome, 17 aldosteronism and 4 nonfunctional adenomas), 5 cases of hyperplasia of adrenal cortex (presented with Chushing' syndrome), 2 cases adrenocortical carcinoma, and 4 samples of normal adrenal cortex.

RESULTSOf the 40 samples, 2 cases of adrenocortical carcinomas had telomerase expression, and the others had no telomerase expression detected.

CONCLUSIONSNo significant telomerase expression was found among different endocrine functional benign adrenocortical lesions. Telomerase expression may be used as an important marker of malignant adrenocortical tumor.

Adrenal Cortex ; enzymology ; Adrenal Cortex Neoplasms ; enzymology ; Adrenocortical Adenoma ; enzymology ; Biomarkers, Tumor ; analysis ; Cushing Syndrome ; enzymology ; Humans ; Telomerase ; analysis ; biosynthesis ; genetics