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ID Design 2012/DOOEL Skopje
Open Access Macedonian Journal of Medical Sciences.
http://dx.doi.org/10.3889/oamjms.2015.130
eISSN: 1857-9655
Case Report
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Pheochromocytoma and
Neurofibromatosis Type 1 in a Patient with Hypertension
Julijana Petrovska1, Biljana Gerasimovska
Kitanovska2, Stevka Bogdanovska2, Svetlana Pavleska
Kuzmanoska2
1Medical Faculty, Ss Cyril and Methodius University of Skopje,
Skopje, Republic of Macedonia (Resident in gastroenterology); 2University
Clinic of Nephrology, Medical Faculty, Ss Cyril and Methodius University of
Skopje, Skopje, Republic of Macedonia
BACKGROUND: Neurofibromatosis type 1 is an autosomal dominant
condition that has a variety of clinical manifestations. Essential or
secondary hypertension may be associated with neurofibromatosis. A rare
finding is hypertension due to pheochromocytoma in patient with
neurofibromatosis type 1.
CASE REPORT: We present a case with a 7-year medical history of
hypertension which was poorly controlled and with wide variations of blood
pressure before the examination. Investigations did not reveal a secondary
cause of hypertension. After the physical examination and establishing the
diagnosis of neurofibromatosis, as well as the history of symptomes
suggestive of catecholamine discharge, diagnostic procedures for
pheochromocytoma were undertaken. Abdominal CT and MRI have proven the
presence of a right adrenal tumor mass which was suspected to be a
pheochromocytoma. Patient was preoperatively treated for two weeks with
alpha and beta blokers and right adrenalectomy was performed.
Perioperatively and on a longer term, blood pressure remained well
controlled with less antihypertensive therapy. Diagnosis and management of
pheochromocytoma in neurofibromatosis involves a dermatologist,
endocrinologist, nephrologist and an urologist and requires a
well-coordinated multidisciplinary approach.
CONCLUSIONS: Pheochromocytoma, although a rare condition in patients
with neurofibromatosis, may be a cause for uncontrolled hypertension, as
well as other cardiovascular complications and the clinician should do all
available clinical investigations to confirm it or exclude it on time.
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Citation: Petrovska J, Gerasimovska Kitanovska B,
Bogdanovska S, Pavleska Kuzmanoska S. Pheochromocytoma and Neurofibromatosis
Type 1 in a Patient with Hypertension. OA Maced J Med Sci. http://dx.doi.org/10.3889/oamjms.2015.130
Key words: Pheochromocytoma; neurofibromatosis type 1; hypertension;
antihypertensives; adrenalectomy.
*Correspondence: Assoc. Prof. Biljana Gerasimovska Kitanovska. Clinic
of Nephrology, Medical Faculty, Vodnjanska 17, Skopje 1000, Republic of
Macedonia. E-Mail: bgerasimovska@yahoo.com
Received: 31-Oct-2015; Revised: 01-Nov-2015; Accepted: 01-Dec-2015; Online
first: 06-Dec-2015
Copyright: © 2015 Julijana Petrovska, Biljana Gerasimovska Kitanovska,
Stevka Bogdanovska, Svetlana Pavleska Kuzmanoska.
This is an open access article distributed under the terms of the Creative
Commons Attribution License, which permits unrestricted use, distribution,
and reproduction in any medium, provided the original author and source are
credited.
Competing Interests: The authors have declared that no competing
interests exist.
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