Introduction: The association of acromegaly and polyostotic fibrous dysplasia is a rare entity that presents more difficulties in achieving an effective treatment than classical acromegaly. Pregnancy is infrequent and carries a high risk when acromegaly is active.
Clínical case: A 27 year old woman surgically treated for facial asymmetry, without café-au-lait spots or precocious puberty, was evaluated in 1992 after a normal pregnancy. In the biochemical tests the following was observed: PRL 95 ng/ml (1.240), GH 20.9 ng/ml not suppressed during oral glucose overload, IGF-I 943 ng/ml (101333), T4 and T3 elevated with suppressed TSH. The rest of the data including basal hormones did not show significant alterations. Imaging tests showed diffuse hyperplasia/adenomatosis of the pituitary gland (14 x 20 x 6 mm) with increased left lining and cavernous sinus invasion and findings compatible with fibrous dysplasia affecting numerous bones of the base of the skull and the cranial vault. In the thyroid scan, a multinodular goiter with calcifications was found. Therapy with bromocriptine (BC) 2,5 mg/day and methimazole was initiated, but acromegaly remained active because the patient refused treatment with subcutaneous octreotide. In 1996 she had an uncomplicated second pregnancy and in January 2000 intramuscular Sandostatin LAR 30 mg/month could be included in the treatment. Magnetic resonance performed after pregnancy showed a small 5-mm focal image compatible with a microadenoma and a pituitary gland close to normal. In 2007 Sandostatin was replaced by GH receptor antagonist Pegvisomant, 20 mg/day subcutaneous because the control objective for IGF-I was not achieved. With this therapy the patient, who also received cabergoline 0.5 mg/wk and metimazol 5 mg/d, was asymptomatic and IGF-I, PRL, thyroid function, cardiac tests, fundus and campimetry were normal.
Discussion: This patient with MAS presented primary hyperthyroidism, hyperprolactinemia and acromegaly with partial response to Sandostatin LAR but complete response to pegvisomant. This drug can be especially useful in the management of MAS-acromegaly for the risks involved in surgery and radiotherapy. Although uncontrolled acromegaly can complicate pregnancy, especially by increasing the risk of gestational diabetes and hypertension, in this case, pregnancy evolved without incident.
20 - 23 May 2017
European Society of Endocrinology