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    Phaeochromocytoma and acromegaly: a unifying diagnosis.

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    Authors
    Mumby, C
    Davis, J
    Trouillas, J
    Higham, Claire E
    Affiliation
    Department of Endocrinology Manchester Royal Infirmary Manchester UK.
    Issue Date
    2014
    
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    Abstract
    A 52-year-old lady was referred after a 5 cm left adrenal mass was detected on computed tomography (CT) scanning. She was asymptomatic although was noted to have acromegalic facies. Blood pressure (BP) was normal but plasma normetanephrines were raised to 2.81 mmol/l (<1.09) and urinary normetadrenaline excretion 5.3 μmol/24 h (0-4.3). Adrenal biochemistry screen was otherwise normal. Metaiodobenzylguanidine (MIBG) scan demonstrated uptake in the adrenal lesion. Growth hormone (GH) nadir on oral glucose tolerance test (OGTT) was 2.2 ng/ml with an elevated IGF1 level of 435 ng/ml (72-215), confirming acromegaly biochemically. The remainder of the pituitary screen was normal. A magnetic resonance imaging (MRI) scan of the pituitary revealed an enlarged pituitary gland with a microadenoma/cyst of 2-3 mm in diameter. Alpha blockade was achieved with a titrated dose of phenoxybenzamine before a successful laparoscopic hand-assisted left adrenalectomy. Postoperative biochemical testing revealed a normal plasma normetanephrine level of 0.6 nmol/l (<1.09) and a metanephrine level of 0.35 nmol/l (<0.46 nmol/l). Nadir on OGTT was normal at 0.07 ng/ml with an IGF1 level within the reference range at 111 ng/ml (75-215). Histology demonstrated a well-circumscribed and encapsulated oval mass with microscopic features typical for a phaeochromocytoma. The sections stained strongly positive for GHRH in 20% of cells on immunocytochemistry. Genetic analysis showed no pathogenic mutation. This is a report of the rare condition of a phaeochromocytoma co-secreting GHRH resulting in clinical and biochemical acromegaly. Neuroendocrine tumours can stain positive for GHRH without coexisting acromegaly, but the resolution of patient symptoms and normalisation of serum GH and IGF1 levels following surgery imply that this was functional secretion. Pituitary surgery should be avoided in such cases.
    Citation
    Phaeochromocytoma and acromegaly: a unifying diagnosis. 2014, 2014:140036 Endocrinol Diabetes Metab Case Rep
    Journal
    Endocrinology, Diabetes & Metabolism Case Reports
    URI
    http://hdl.handle.net/10541/321818
    DOI
    10.1530/EDM-14-0036
    PubMed ID
    24897038
    Type
    Article
    Language
    en
    ISSN
    2052-0573
    ae974a485f413a2113503eed53cd6c53
    10.1530/EDM-14-0036
    Scopus Count
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