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Indian J Endocrinol Metab. 2014 Jul;18(4):542-5. doi: 10.4103/2230-8210.137514.

A retrospective study of surgically excised phaeochromocytomas in Newfoundland, Canada.

Indian journal of endocrinology and metabolism

Joanna Holland, Vikram Chandurkar

Affiliations

  1. Fellow, Endocrinology, University of Alberta, Edmonton, Canada.
  2. Assistant Professor, Division of Endocrinology, Faculty of Medicine, Memorial University of Newfoundland, Health Sciences Center, St. John's, NL, Canada.

PMID: 25143914 PMCID: PMC4138913 DOI: 10.4103/2230-8210.137514

Abstract

OBJECTIVE: A retrospective study detailing the circumstances surrounding diagnosis and treatment of pheochromocytomas with the associated genetic disorders.

MATERIALS AND METHODS: All patients with surgically excised pheochromocytomas in the Health Sciences Center, St. John's, Newfoundland, Canada between January 2001 and December 2010 were retrospectively analyzed to determine associated familial syndromes, age, tumor size, symptomatology, and percentage of paragangliomas and bilateral pheochromocytomas. Pathology specimen reports, adrenalectomy lists and Meditech (electronic medical record) diagnostic codes provided a comprehensive database for this study.

RESULTS: Twenty-four patients were studied; familial disorder patients comprised 42% (10/24). Average age at diagnosis was 57 among the sporadic and 34 in familial disorder groups (P = 0.006). Average tumor size was 4.5 cm in the sporadic group and 3 cm in the familial disorder group (P = 0.19). All atypical cases including bilateral or extra-adrenal tumors and malignancy occurred in familial disorder patients.

CONCLUSIONS: The proportion of familial disorder patients (42%) was higher in this study than would be expected, likely a result of the relatively high incidence of hereditary autosomal dominant disorders within Newfoundland. Among familial disorder patients, the average younger age at diagnosis and the smaller tumor size suggest syndromic pheochromocytomas may develop earlier, however they are more likely to be diagnosed sooner due to biochemical surveillance testing in known genetic disorder patients. We also demonstrate a relatively high incidence of surgically resected pheochromocytomas of 4.679/million/year in Newfoundland.

Keywords: Familial; Newfoundland; phaeochromocytoma; von hippel lindau

References

  1. Eur J Surg. 1996 Sep;162(9):695-702 - PubMed
  2. N Engl J Med. 2002 May 9;346(19):1459-66 - PubMed
  3. Hormones (Athens). 2009 Jan-Mar;8(1):29-38 - PubMed
  4. J Hum Hypertens. 1988 Oct;2(3):187-9 - PubMed
  5. J Clin Endocrinol Metab. 2011 Feb;96(2):375-84 - PubMed
  6. Best Pract Res Clin Endocrinol Metab. 2012 Aug;26(4):507-15 - PubMed
  7. Medicine (Baltimore). 1991 Jan;70(1):46-66 - PubMed
  8. CMAJ. 1986 Jan 15;134(2):133-8, 146 - PubMed
  9. J Clin Endocrinol Metab. 2003 Oct;88(10):4533-9 - PubMed
  10. J Urol. 1999 Sep;162(3 Pt 1):659-64 - PubMed
  11. Kidney Int. 2002 Jun;61(6):1925-34 - PubMed
  12. Eur J Endocrinol. 2004 May;150(5):681-6 - PubMed
  13. BJU Int. 2008 Jun;101(12):1561-4 - PubMed
  14. Aust N Z J Surg. 1985 Oct;55(5):471-5 - PubMed
  15. Acta Med Scand. 1986;220(3):225-32 - PubMed
  16. J Intern Med. 1994 Dec;236(6):675-7 - PubMed
  17. J Clin Hypertens (Greenwich). 2002 Jan-Feb;4(1):62-72 - PubMed
  18. J Natl Cancer Inst. 2003 Aug 20;95(16):1196-204 - PubMed

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