ECE2014 Poster Presentations Clinical case reports Pituitary/Adrenal (50 abstracts)
Acromegaly with a normal pituitary gland
Alfredo Sanchez Cruz , Jose H Martinez , Maria de Lourdes Miranda , Coromoto Palermo , Eva Gonzalez , Oberto Torres , Monica Santiago , Carlos Figueroa , Rafael Trinidad , Michelle Mangual & Madeleine Gutierrez
San Juan Hospital, San Juan, Puerto Rico.
Background: Acromegaly most commonly results from an excess production of GH and secondary increase in IGF1. More than 95% of the time from the pituitary gland, but the source of excess GH secretion may not necessarily be pituitary in origin. Ectopic acromegaly may arise due to neuroendocrine tumors by production of GHRH and in <0.5% of the cases from ectopic pituitary remnants in the sphenoid sinus.
Clinical case: We present a case of a 37 years old man with typical symptoms of acromegaly. Initial laboratory evaluation consistent with the diagnosis of acromegaly, hGH=2.699 ng/ml (0.014–1.406 ng/ml) and IGF1=956.00 ng/ml (109–284 ng/ml), prolactin and TSH levels were normal. A brain MRI with special attention to pituitary gland revealed normal size and configuration with no definitive lesions identified. However, a large heterogeneous mass involving the sphenoid sinus extending into the posterior ethmoidal air cells was seen. Patient underwent transphenoidal surgery. Immunostaining analysis of the sphenoidal mass revealed; GH(+), prolactin(+), TSH(+), ACTH(−), FSH(−), and LH(+). Post-surgical evaluation showed persistent levels of GH and IGF1 (hGH=1.413 ng/ml and IGF1=802 ng/ml), and no glucose suppression after an oral glucose tolerance test. After surgery MRI showed normal size pituitary gland but residual heterogeneous material involving the sphenoidal sinus. Due to the persistence of biochemical and radiological abnormalities patient required Gamma-knife treatment and medical therapy with Cabergoline and Sandostatin.
Conclusion: In patients with clinically and biochemical evidence of acromegaly but with normal findings in the pituitary gland, ectopic origin due to embryological pituitary remnants should be consider. Ectopic acromegaly is a very rare clinical presentation. Lack of consideration of this etiology could be responsible for failed TES in endocrinologically active tumors.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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