Usefulness of basal catecholamine plasma levels and clonidine suppression test in the diagnosis of pheochromocytoma

Mannelli, Massimo; Feo, Maria Laura De; Maggi, Mario; Pupilli, Cinzia; Opocher, Giuseppe; Valenza, T; Baldi, Elisabetta; Serio, Mario

doi:10.1007/bf03348153

Cited by 19 publications

(7 citation statements)

References 16 publications

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“…Our data also confirm that the clonidine suppression test is a useful tool in the diagnosis of pheo (10,19). In fact, it gave a false negative response in 2/38 patients.…”

Section: Discussionsupporting

confidence: 82%

Pheochromocytoma in Italy: a multicentric retrospective study

Mannelli

Ianni

Cilotti

et al. 1999

European Journal of Endocrinology

196

109

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Objective: To conduct an epidemiological study on pheochromocytoma in Italy. Methods: Data on 284 patients with pheochromocytoma observed between 1978 and 1997 were collected from 18 Italian centers through a questionnaire reporting epidemiological, clinical, laboratory, radiological and surgical data. Results: 53.6% of the patients were females and 46.4% were males. Thirty-two tumors were discovered as incidental adrenal masses. The most frequent referred symptoms were palpitations (58.1%), headache (51.9%), sweating (48.8%) and anxiety (35.3%). Their association was present only in 15.5% of patients. Paroxysmal symptoms were reported in 67.1% and hypertensive crises in 59.7% of patients. Normal blood pressure (systolic and diastolic) was present both in the supine and upright positions in 21.1% of patients. Among laboratory assays, urinary vanylmandelic acid (VMA) was the most widely used (58.1%) and was the least sensitive (25% of false negative results). Basal plasma catecholamines were found to be normal in 11.3% of patients but were always elevated when sampled during a hypertensive paroxysm. A clonidine suppression test was performed in 38 patients with no adverse side effects. It gave a false negative response in 2 patients. A glucagon test was performed in 21 patients. It was interrupted for acute hypertension in 52.4% of patients. Only 5/21 patients were normotensive and had normal basal plasma catecholamines. In these patients the test gave a positive response in four (80%). CT (79.6%) and I-MIBG scintigraphy (68.5%) were the most widely used methods for tumor localization. CT sensitivity was 98.9% for intra-adrenal and 90.9% for extraadrenal tumors. MIBG sensitivity was 88.5%. In the 263 patients who underwent surgery, the tumor was intra-adrenal in 89.4%, extra-adrenal in 8.5%, intra-and extra-adrenal in 2.1%, and bilateral in 11.0% of patients. Malignancy was reported in 9.9% of cases. Surgery caused remission of hypertension in 59.3%, improvement in 26.8%, and no changes in 13.9% of patients. In the last group the interval between initial symptoms and diagnosis was significantly longer. Conclusions: The present study confirms that the clinical presentation of pheochromocytoma is variable and aspecific. Normotension is often present and often the tumor is discovered incidentally. An indication for the routine use of screening methods more sensitive than urinary VMA is strongly suggested. The clonidine test was found to be safe and should be preferred to the glucagon test which has to be restricted to very selected patients. CT and MIBG scintigraphy are almost always successful in localizing the tumor. Reversal of hypertension by surgery seems to depend on an early diagnosis. European Journal of Endocrinology 141 619-624Introduction Pheochromocytoma (pheo) is a rare tumor arising from the chromaffin tissue. It causes hypertension in about 0.1% of hypertensive patients and presents itself with a highly variable clinical picture (1-7). These two characteristics (rarity and variability) render ...

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“…Our data also confirm that the clonidine suppression test is a useful tool in the diagnosis of pheo (10,19). In fact, it gave a false negative response in 2/38 patients.…”

Section: Discussionsupporting

confidence: 82%

Pheochromocytoma in Italy: a multicentric retrospective study

Mannelli

Ianni

Cilotti

et al. 1999

European Journal of Endocrinology

196

109

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“…Indeed, plasma metanephrine concentrations do not decrease after clonidine in patients without PHEO (Gutmann et al 1994). In addition, clonidine-induced changes of E offer limited help (Gross et al 1987, Mannelli et al 1987.…”

Section: Suppression Testsmentioning

confidence: 99%

Pheochromocytoma: an update on genetics and management

Karagiannis¹,

Mikhailidis²,

Athyros³

et al. 2007

Endocrine Related Cancer

116

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Pheochromocytomas (PHEOs) are rare neoplasms that produce catecholamines and usually arise from the adrenal medulla and are considered to be an adrenal paraganglioma (PGL). Closely related tumors of extraadrenal sympathetic and parasympathetic paraganglia are classified as extraadrenal PGLs. Most PHEOs are sporadic, but a significant percentage (w25%) may be found in patients with germline mutations of genes predisposing to the development of von Hippel-Lindau disease, neurofibromatosis 1, multiple endocrine neoplasia type 1 (MEN1) and 2 (MEN2), and the PGL/PHEOs syndrome, based on the described mutations of the genes for succinate dehydrogenase subunit D (SDHD), B (SDHB), and C (SDHC). As one out of four PHEOs turns out to be a hereditary clinical entity, screening for genetic alterations is important, as it provides useful information for a rational diagnostic approach and management. This review discusses the genetics, the pathophysiology of hypertension, the clinical picture, the biochemical and imaging diagnosis, and the preferred therapeutic approach for PGLs/PHEOs. Furthermore, it emphasizes the need for genetic testing in cases with apparently sporadic PHEOs.

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“…Clonidin aktiviert die alpha 2 -Rezeptoren im Gehirn und unterdrückt dadurch die Katecholaminfreisetzung an den sympathischen Nervenendigungen. Ein Abfall der Katecholaminplasmakonzentration nach Clonidingabe spricht infolgedessen für eine erhöhte sympathische Aktivität, ein unveränder-ter Wert dagegen für eine autonome Sekretion durch ein P/PGL [3,15]. Die Aussagefähigkeit des CST verbunden mit der Bestimmung der Katecholamine im Plasma ist in mehreren Untersuchungen bestä-tigt worden [9,10,16].…”

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