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

Clinical Endocrinology, Charité Campus Mitte, Charité University Medicine Berlin, Charitéplatz 1, D 10117 Berlin, Germany.
European Journal of Endocrinology (Impact Factor: 4.07). 07/2009; 161(2):355-61. DOI: 10.1530/EJE-09-0384
Source: PubMed


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.
To specify the exact circumstances of discovery of adrenal and extra-adrenal phaeochromocytoma.
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.
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 >25% (29.4% of the total study population). Patients with incidentally detected phaeochromocytoma were significantly older (53.1+/-1.9 vs 47.0+/-1.3 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 (36.6+/-14.7 vs 52.5+/-34.3 mm; P<0.05), whereas extra-adrenal tumours had a mean diameter of 52.6+/-28.7 mm.
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.

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    • "Incidental discovery of PPGLs on imaging is becoming an increasingly important mode of their diagnosis; for instance, the proportion of PCs detected incidentally is now 25–30% of all cases (Mannelli et al. 1999, Amar et al. 2005, Kopetschke et al. 2009, Shen et al. 2010). Specific imaging findings are discussed in more detail below. "
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    ABSTRACT: The paraganglioma (PGL) syndromes types 1-5 are autosomal dominant disorders characterized by familial predisposition to PGLs, phaeochromocytomas (PCs), renal cell cancers, gastrointestinal stromal tumours and, rarely, pituitary adenomas. Each syndrome is associated with mutation in a gene encoding a particular subunit (or assembly factor) of succinate dehydrogenase (SDHx). The clinical manifestations of these syndromes are protean: patients may present with features of catecholamine excess (including the classic triad of headache, sweating and palpitations), or with symptoms from local tumour mass, or increasingly as an incidental finding on imaging performed for some other purpose. As genetic testing for these syndromes becomes more widespread, presymptomatic diagnosis is also possible, although penetrance of disease in these syndromes is highly variable and tumour development does not clearly follow a predetermined pattern. PGL1 syndrome (SDHD) and PGL2 syndrome (SDHAF2) are notable for high frequency of multifocal tumour development and for parent-of-origin inheritance: disease is almost only ever manifest in subjects inheriting the defective allele from their father. PGL4 syndrome (SDHB) is notable for an increased risk of malignant PGL or PC. PGL3 syndrome (SDHC) and PGL5 syndrome (SDHA) are less common and appear to be associated with lower penetrance of tumour development. Although these syndromes are all associated with SDH deficiency, few genotype-phenotype relationships have yet been established, and indeed it is remarkable that such divergent phenotypes can arise from disruption of a common molecular pathway. This article reviews the clinical presentations of these syndromes, including their component tumours and underlying genetic basis. © 2015 The authors.
    Endocrine Related Cancer 08/2015; 22(4):T91-T103. DOI:10.1530/ERC-15-0268 · 4.81 Impact Factor
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    • "The relatively large size of our patient's tumor may be a cause, as well as a consequence, of the lack of symptoms, which is concordant with observations that pheochromocytomas are more likely to be incidentally diagnosed in older patients [6] [9]. Moreover, as the use of computed tomography scans and magnetic resonance imaging to evaluate abdominal symptoms has become more frequent, the incidence of pheochromocytomas which do not present with classical symptoms has increased [5] [9] [10]. Incidental pheochromocytomas are less likely to be associated with classical symptoms and are larger at diagnosis [5] [10]. "
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    ABSTRACT: Pheochromocytomas are thought to be uncommon in the elderly. However, the prevalence is likely to be higher than reported, as older patients are less likely to be diagnosed due to absence of classical symptoms of sympathetic overactivity and confounding effects of aging, comorbidities, and medications. We describe a hypertensive elderly patient with incidentally diagnosed pheochromocytoma complicated by recurrent urosepsis, cardiomyopathy, and fatal myocardial infarction. Our case demonstrates that, in older hypertensive patients without classical symptoms, orthostatic hypotension and urinary retention, which are common in the elderly, may indicate catecholamine excess and that the deleterious cardiovascular consequences of catecholamine excess in the elderly are not prevented by pharmacological α- and β-blockade.
    Case Reports in Endocrinology 09/2011; 2011:759523. DOI:10.1155/2011/759523
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    • "only ten of whom were diagnosed prior to 2000. Kopetschke, et al.8 reported that approximately 30% of pheochromocytomas are detected incidentally, and that this proportion is increasing. This increase in detection is likely due to increasing availability of imaging procedures. "
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    ABSTRACT: We report herein 119 patients with pheochromocytoma at our institute over the last 23 years. Between 1986 and 2009, 119 patients were diagnosed with pheochromocytoma at our institute. We reviewed the medical records of these patients. Of 119 patients, 45 were male and 74 were female, and mean age was 43.83 ± 13.49 years. Forty-three patients (36.1%) were diagnosed incidentally, and 8 patients (6.7%) were found to have familial pheochromocytoma. The mean dimension of the tumors was 5.89 ± 3.18 cm. 4 patients had bilateral tumors; three of these patients were found to have familial pheochromocytoma and 1 patient was diagnosed with malignant pheochromocytoma. A total of eight patients (6.7%) were found to have malignant pheochromocytoma. In 1 patient, metastasis to a lymph node was found at the time of diagnosis. Metastases were found at a mean of 49 ± 25.83 (6-75) months after surgery in the other seven patients. 6 patients died of malignant pheochromocytoma at a mean of 31 ± 28.71 months (1-81) after diagnosis, and the other 2 patients survived for 15 and 24 months, respectively. Approximately 35% of patients with pheochromocytoma are diagnosed incidentally, and the number of detected cases is increasing. Although familial pheochromocytoma was found only in 6.7% of the patients, genetic testing should be considered in all patients, especially in patients with a family history, young age, or multifocal, bilateral, extra-adrenal, or malignant tumors. Given that malignant pheochromocytomas are frequently diagnosed during the follow-up period, long-term follow-up is necessary to confirm the absence of recurrence or metastasis.
    Yonsei medical journal 01/2011; 52(1):45-50. DOI:10.3349/ymj.2011.52.1.45 · 1.29 Impact Factor
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