SFEBES2018 Featured Clinical Cases Featured Clinical Cases (10 abstracts)
Clinical and biochemical acromegaly associated with pituitary FSHomas
Isabel Huang Doran 1, , Olympia Koulouri 1, , Sue Oddy 3 , David Halsall 3 , Dominic O’Donovan 4 , Federico Roncaroli 5, , Richard Mannion 7 , Kieran Allinson 4 & Mark Gurnell 1,
1University of Cambridge Metabolic Research Laboratories, Wellcome-MRC Institute of Metabolic Science, Addenbrooke’s Hospital, Cambridge, UK; 2Wolfson Diabetes & Endocrine Clinic, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK; 3Department of Clinical Biochemistry, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK; 4Department of Pathology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK; 5Department of Cellular Pathology, Salford Royal NHS Foundation Trust, Salford, UK; 6Division of Neuroscience & Experimental Psychology, University of Manchester, Manchester, UK; 7Department of Neurosurgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
Acromegaly is a clinical manifestation of excessive peripheral growth hormone (GH) action. Most cases result from pituitary somatotroph adenomas displaying varying degrees of GH immunoreactivity. Occasionally, GH is cosecreted with a second hormone from adenomas containing mixed cell populations (e.g. somatolactotroph tumours). Coexistence of multiple discrete adenomas, identical or distinct in hormone secretion, is infrequent. In very rare cases, acromegaly results from neuroendocrine tumours producing ectopic GHRH or even GH. We describe three male patients (P1-3) presenting with clinical acromegaly associated with elevated IGF1 (1.43×, 1.64× and 2.64× ULN), elevated basal GH (1.5, 2.4, 5.6 mcg/L) and failure to suppress GH after a 75 g glucose load (nadir GH 1.2, 2.5, 7.6 mcg/L). P1 also had elevated FSH (107 U/L [1.0–10.1]), associated clinically with macro-orchidism, whilst FSH was mildly elevated in P2, and just below ULN in P3. All three patients had pituitary macroadenomas with abundant FSH immunoreactivity, no or low LH immunoreactivity and, contrary to clinical presentation, no GH immunoreactivity (verified externally). All three expressed SF-1 ubiquitously but not Pit-1. Consistent with immunoreactivity, culture medium from a primary adenoma culture contained abundant FSH but undetectable GH. Cross sectional imaging (with functional imaging in 2/3 patients) failed to identify an ectopic source of GHRH or GH secretion. No circulating GHRH was detectable by immunoassay in any of the patients. Serial follow up of P1 and P2 revealed persistent mild biochemical acromegaly, elevated FSH and slowly enlarging pituitary remnants. A six-month trial of somatostatin analogue in P1 was unsuccessful in suppressing IGF1 or GH. Both patients underwent further tumour resection, with histology again demonstrating gonadotroph adenomas only. In summary, we describe a previously unreported phenomenon of clinical and biochemical acromegaly associated with pure gonadatroph adenomas, without evidence of somatotroph adenomas or hyperplasia, and without evidence of ectopic GH/GHRH secretion.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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