Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2008) 16 P38

ECE2008 Poster Presentations Adrenal (61 abstracts)

Retrospective clinical data on more than 160 pheochromocytoma in three east german endocrine centers

Robert Kopetschke 1 , Aylin Kilisli 1 , U Tuschy 2 , Henri Wallaschofski 3 , Wolfgang Oelkers 4 , Manfred Ventz 1 & Marcus Quinkler 1


1Klinische Endokrinologie, Charité Campus Mitte, Charite Universitätsmedizin Berlin, Berlin, Germany; 2Bereich Endokrinologie, 2. Medizinische Klinik, Helios Klinikum Erfurt, Erfurt, Germany; 3Klinik für Innere Medizin A, Ernst-Moritz-Arndt-Universität, Greifswald, Germany; 4Endokrinologikum Berlin, Berlin, Germany.


Objective: Pheochromocytoma is a rare disease with an incidence of 2–6 per million, and a prevalence of 0.1–0.6% in patients with hypertension. Pheochromocytoma may occur sporadically or as part of hereditary syndrome. According to the latest studies, among patients with non-syndromic pheochromocytoma, up to 24% of tumors may be inherited. However, genetic testing was not generally performed until recently. There is still a debate which pheochromocytoma patient should be tested for hereditary syndrome. We started an initiative to create a database of sufficient epidemiological strength to investigate pheochromocytoma in more detail.

Methods: The preliminary registry has at present 3 participating centres in 3 locations and uses an electronic database to assure comparison of different centres. Since 07/2006 retrospective data was included in the database from patients with pheochromocytoma between 1973 and 2007.

Results: Evaluation of the retrospective data entry shows the following results: Of the 166 patients (49.3±14.4 years, range 12–81 years, 49.4% males), 115 pheochromocytomas were unilateral (64 right, 53 left), 19 bilateral, and 4 extraadrenal (no information in 28 patients). The mean size of unilateral pheochromocytoma was 64±25 mm. Fourteen pheochromocytoma were diagnosed as malignant, with only 10 patients showing evidence of metastasis. Genetic testing was performed in only 31 patients revealing a hereditary syndrome in 13 patients (8 RET, 3 VHL, 2 NF1). In addition, a hereditary syndrome was diagnosed clinically in 21 patients (14 MEN, 4 VHL, 3 NF1).

Conclusion: In general, documentation and follow-up of patients with pheochromocytoma was insufficient over the recent years. This emphasizes the need for a clinical national registry including follow-up information of the patients. In our cohort 34 patients (20.5%) had a hereditary form of pheocromocytoma, which is in accordance to previous studies.

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