Functioning adrenocortical oncocytomas are extremely rare and most reported patients are 40-60 yr of age. To our knowledge, only 2 cases of functioning adrenocortical oncocytomas have been reported in childhood. We report a case of functioning adrenocortical oncocytoma in a 14-yr-old female child presenting with virilization. She presented with deepening of the voice and excessive hair growth, and elevation of plasma testosterone and dehydroepiandrosterone sulfate. She had an adrenalectomy. The completely resected tumor composed predominantly of oncocytes without atypical mitosis and necrosis. A discussion of this case and a review of the literature on this entity are presented.
Adenoma, Oxyphilic/*complications/metabolism/pathology/surgery ; Adolescent ; Adrenal Cortex Neoplasms/*complications/metabolism/pathology/surgery ; Adrenalectomy ; Adult ; Female ; Humans ; Male ; Middle Aged ; Virilism/*etiology/pathology/surgery

BACKGROUNDHypertension often persists after adrenalectomy for primary aldosteronism (PA). Many studies have analyzed the outcomes of adrenalectomy for aldosterone-producing adenomas (APA) to identify predictive factors for persistent hypertension. However, differentially expressed genes in persistent postoperative hypertension remain unknown. Our aim was to describe gene expression profile of persistent postoperative hypertension patients with APA.

METHODSIn this study, we described and compared gene expression profiles in persistent postoperative hypertension and postoperative normotension in Chinese patients with APA using microarray analysis. Confirmation was performed with quantitative real time-polymerase chain reaction analysis. Bioinformatic analysis (gene ontology analysis, pathway analysis and network analysis) was used for further research.

RESULTSMicroarray analysis identified a total of 99 differentially expressed genes, including 18 up-regulated and 81 down-regulated genes. Among the dysregulated genes were fat atypical cadherin 1 as well as fatty acid binding protein 4 and other genes that have not been previously studied in persistent postoperative hypertension with APA. Bioinformatics analysis indicated that differentially expressed genes were associated with lipid metabolic process, metal ion binding, and cell differentiation. Pathway analysis determined that five pathways corresponded to the dysregulated transcripts. The mRNAs-ncRNAs co-expression network was composed of 49 network nodes and 72 connections between 18 coding genes and 31 noncoding genes.

CONCLUSIONSThis study revealed differentially expressed genes in persistent postoperative hypertension with APA and provided a resource of candidate genes for exploration of possible drug targets and prognostic markers.

Adenoma ; metabolism ; physiopathology ; surgery ; Adrenalectomy ; Aldosterone ; metabolism ; Blood Pressure ; physiology ; Gene Expression Profiling ; methods ; Humans ; Hyperaldosteronism ; metabolism ; physiopathology ; surgery ; Postoperative Complications ; Retrospective Studies

No abstract available.
Adrenal Cortex Function Tests ; *Adrenal Cortex Neoplasms/complications/diagnosis/metabolism/surgery ; *Adrenal Gland Neoplasms/complications/diagnosis/metabolism/surgery ; Adrenalectomy ; *Adrenocortical Adenoma/complications/diagnosis/metabolism/surgery ; Biopsy ; Cushing Syndrome/diagnosis/etiology ; Female ; Humans ; Immunohistochemistry ; *Incidental Findings ; Middle Aged ; *Neoplasms, Multiple Primary/complications/diagnosis/metabolism/surgery ; *Pheochromocytoma/complications/diagnosis/metabolism/surgery ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome ; Tumor Markers, Biological/metabolism

Adenocarcinoma, Clear Cell ; complications ; metabolism ; pathology ; surgery ; Adenoma ; pathology ; Adnexal Diseases ; pathology ; Adult ; Carcinoma, Renal Cell ; pathology ; secondary ; Cystadenoma, Papillary ; complications ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Fallopian Tube Neoplasms ; complications ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Keratin-7 ; metabolism ; Kidney Neoplasms ; pathology ; secondary ; Mucin-1 ; metabolism ; von Hippel-Lindau Disease ; complications ; metabolism ; pathology ; surgery

It has been suggested that the patients with Cushing's disease secondary to pituitary macroadenomas (>10 mm) have higher basal adrenocorticotropic hormone (ACTH) levels, which are less suppressible on high-dose dexamethasone suppression tests (HDDST). We compared the clinical and biochemical characteristics of patients with macroadenomas (N=7) and microadenomas (N=23) who were diagnosed at Samsung Medical Center in Korea between 1996 and 2006. Basal morning plasma ACTH levels were 101.5+/-23.2 pg/mL for macroadenoma patients and 83.6+/-11.1 pg/mL for microadenoma patients (mean+/-SEMs) (p=0.44). Morning serum cortisol levels were 26.8+/-3.2 microgram/dL for macroadenoma patients and 29.5 +/-2.9 microgram/dL for microadenoma patients (p=0.77). The proportion of patients who showed suppressibility on HDDST was almost identical in the two groups (71.4% [5/7] for macroadenoma patients vs. 72.7% [16/22] for microadenoma patients, p=1.00). Furthermore, the remission rate with trans-sphenoidal surgery was similar between the two groups (100% [5/5] for macroadenoma patients vs. 73.3% [11/ 15] for microadenoma patients, p=0.53). Thus, tumor size is not a major determinant of hormone secretion or clinical outcomes in patients with Cushing's disease.
Adenoma/complications/*metabolism/surgery ; Adolescent ; Adrenocorticotropic Hormone/blood/*secretion ; Adult ; Aged ; Dexamethasone/metabolism ; Female ; Humans ; Hydrocortisone/blood/*secretion ; Male ; Middle Aged ; Pituitary ACTH Hypersecretion/*diagnosis/etiology ; Pituitary Neoplasms/complications/*metabolism/surgery ; Retrospective Studies

Adenoma ; metabolism ; pathology ; Adult ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Ear Neoplasms ; complications ; genetics ; metabolism ; pathology ; surgery ; Endolymphatic Sac ; Female ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; Middle Aged ; Paraganglioma ; metabolism ; pathology ; Point Mutation ; Von Hippel-Lindau Tumor Suppressor Protein ; genetics ; von Hippel-Lindau Disease ; complications ; genetics ; metabolism

OBJECTIVETo study the clinicopathologic characteristics of parathyroid carcinoma (PTC).

METHODSEleven cases of PTC encountered during the period from 1994 to 2012 were enrolled into the study. Forty cases of parathyroid adenoma (PA) were also retrieved for comparison. The clinical manifestations, laboratory results and pathologic features were analyzed, with literature review.

RESULTSThe main clinical manifestations of PTC included neck mass (11/11), hypercalcemia (11/11) and hyperparathyroidism (11/11). Most patients also had osteoporosis (10/11). In contrast, PA often manifested as hypercalcemia (40/40) and hyperparathyroidism (40/40). Histologic examination of PTC showed that the tumor cells contained clear to eosinophilic cytoplasm and separated by dense bands of fibrosis. The tumor mass was surrounded by thick fibrous capsule. Foci of capsular invasion and vascular permeation were identified at the tumor periphery in all cases. Cellular atypia was not conspicuous but mitotic figures and coagulative necrosis were easily identified. On the other hand, PA were composed of tumor cells with clear to eosinophilic cytoplasm, forming glands, trabeculae or nests. Most of them (35/40) had intact fibrous capsule. Mitotic figures were rarely encountered and tumor necrosis was absent. Immunohistochemical study showed that the tumor cells in PTC were positive for CK19 (11/11), chromogranin A (9/11), synaptophysin (7/11) and parathyroid hormone (11/11). They were negative for thyroglobulin, TTF-1 and calcitonin. The Ki-67 index was less than 10% (range = 2% to 9%). In contrast, the tumor cells in PA were positive (40/40) for CK19, chromogranin A, synaptophysin and parathyroid hormone. They were negative for thyroglobulin, TTF-1 and calcitonin. The Ki-67 index was less than 3%. Follow up-data were available in 9 cases of PTC (duration of follow up = 11 months to 224 months) and 7 of the patients were still alive. Follow up of all PA cases showed no evidence of recurrence.

CONCLUSIONSPTC is a rare malignant endocrine tumor presenting as neck mass. Histologic features suggestive of malignant behavior include presence of coagulative tumor necrosis and capsular/vascular invasion. It needs to be distinguished from other entities such as parathyroid adenoma, papillary thyroid carcinoma and medullary thyroid carcinoma.

Adenoma ; metabolism ; pathology ; Adult ; Carcinoma ; metabolism ; pathology ; Carcinoma, Neuroendocrine ; Carcinoma, Papillary ; Chromogranin A ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hypercalcemia ; etiology ; Hyperparathyroidism ; etiology ; Immunohistochemistry ; Keratin-19 ; metabolism ; Male ; Middle Aged ; Osteoporosis ; etiology ; Parathyroid Hormone ; metabolism ; Parathyroid Neoplasms ; complications ; metabolism ; pathology ; surgery ; Synaptophysin ; metabolism ; Thyroid Neoplasms ; metabolism ; pathology