On the Prevalence of Adrenocortical Adenomas in an Autopsy Material in Relation to Hypertension and Diabetes

176

114

Context: Adrenal and extra-adrenal phaeochromocytoma are chromaffin cell-derived tumours that are discovered due to classical symptom triad with headache, sweating and palpitations combined with persistent or paroxysmal hypertension. However, an increasing proportion of phaeochromocytoma seems to be discovered incidentally upon abdominal imaging. Objective: To specify the exact circumstances of discovery of adrenal and extra-adrenal phaeochromocytoma. Design and patients: Four German endocrine centres participated in this retrospective study. Medical records of 201 patients with adrenal and extra-adrenal phaeochromocytoma who were diagnosed between 1973 and 2007 were analyzed. Results: The typical triad of symptoms was found only in 10% of cases. Ten percent of patients presented were without clinical symptoms and 6.1% were normotensive. Documented blood pressure peaks occurred in 44.1% of cases. In 24 patients (12.2%), phaeochromocytoma was malignant. Before 1985, !10% of cases were incidentally discovered, whereas thereafter the frequency was O25% (29.4% of the total study population). Patients with incidentally detected phaeochromocytoma were significantly older (53.1G1.9 vs 47.0G1.3 years; P!0.05) and often had less blood pressure peaks (37.0 vs 70.7%; P!0.001) than patients in whom the diagnosis was suspected on clinical grounds. Of phaeochromocytomas 94.4% were intra-adrenal tumours, of which 12.9% were bilateral. Bilateral tumours were significantly smaller than unilateral tumours (36.6G14.7 vs 52.5G34.3 mm; P!0.05), whereas extra-adrenal tumours had a mean diameter of 52.6G28.7 mm. Conclusions: Owing to better availability and accessibility of imaging procedures, the number of incidentally discovered phaeochromocytoma is increasing and reaches nearly 30% in our study population. Every adrenal incidentaloma should be investigated for the presence of phaeochromocytoma.

Section: Discussionmentioning

confidence: 99%

Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma

Kopetschke¹,

Slisko²,

Kilisli³

et al. 2009

176

114

“…(19) In a review of a large number of studies, the etiology of incidentaloma was: adenoma 41%, metastasis 19%, adrenocortical carcinoma 10%, myolipoma 9%, and pheochromocytoma 8%, with the remainder being mostly benign lesions such as cysts (20).…”

Section: Glucocorticoids Pheochromocytoma and The Incidental Adrenamentioning

confidence: 99%

Pheochromocytoma crisis induced by glucocorticoids: a report of four cases and review of the literature

Rosas

Kasperlik-Załuska

Papierska

et al. 2008

Context: Pheochromocytoma crisis (PC) is a rare life-threatening endocrine emergency that may present spontaneously or can be unmasked by 'triggers', including certain medications that provoke the release of catecholamines by tumors. Several isolated cases of PC have been reported after administration of exogenous glucocorticoids; evidence that these drugs cause adverse events in patients with pheochromocytoma is mainly anecdotal. Patients: We report four cases of PC most likely induced by glucocorticoids and review seven previous reports in the literature linking steroid administration to the development of PC.

“…Adrenocortical tumours have a relatively high prevalence in the general population of up to 9% in autopsy studies (1,2). However, malignancy is rare, the yearly incidence being 0.5-2 per million people, but with only 12 months mean survival time for patients with nonresectable cancers (3)(4)(5)(6)(7).…”

Section: Introductionmentioning

confidence: 99%

Transcriptional profiling enables molecular classification of adrenocortical tumours

Laurell

Velázquez-Fernández

Lindsten

et al. 2009

Objective: Tumours in the adrenocortex are common human tumours. Malignancy is however, rare, the yearly incidence being 0.5-2 per million inhabitants, but associated with a very aggressive behaviour. Adrenocortical tumours are often associated with altered hormone production with a variety of clinical symptoms. The aggressiveness of carcinomas together with the high frequency of adenomas calls for a deeper understanding of the underlying biological mechanisms and an improvement of the diagnostic possibilities. Methods: Microarray gene expression analysis was performed in tumours of adrenocortex with emphasis on malignancy as well as hormonal activity. The sample set consisted of 17 adenomas, 11 carcinomas and 4 histological normal adrenocortexes. RNA from these was hybridised according to a reference design on microarrays harbouring 29 760 human cDNA clones. Confirmation was performed with quantitative real time-PCR and western blot analysis. Results: Unsupervised clustering to reveal relationships between samples based on the entire gene expression profile resulted in two subclusters; carcinomas and non-cancer specimens. A large number of genes were accordingly found to be differentially expressed comparing carcinomas to adenomas. Among these were IGF2, FGFR1 and FGFR4 in growth factor signalling the most predominant and also the USP4, UBE2C and UFD1L in the ubiquitin-proteasome pathway. Moreover, two subgroups of carcinomas were identified with different survival outcome, suggesting that survival prediction can be made on the basis of gene expression profiles. Regarding adenomas with aldosterone overproduction, OSBP and VEGFB were among the most up-regulated genes compared with the other samples. Conclusions: Adrenocortical carcinomas are associated with a distinct molecular signature apparent in their gene expression profiles. Differentially expressed genes were identified associated with malignancy, survival as well as hormonal activity providing a resource of candidate genes for an exploration of possible drug targets and diagnostic and prognostic markers.