Endocrine Abstracts

Introduction: Acromegaly with GH excess affects up to 20% of the patients with McCune–Albright syndrome (MAS). Surgical treatment for acromegaly in MAS is often difficult because of skull-base dysplasia. Somatostatin analogs are frequently only partially effective and GH receptor antagonist – pegvisomant is more potent in normalizing IGFI levels. Radiotherapy is controversial. Pregnancy in MAS patients is described in literature but no case of successful delivery in MAS patient with acromegaly treated with both somatostatin analog and pegvisomant was published till now. Only three cases of pegvisomant treated pregnant acromegalic patients have been published (two with medication throughout the whole pregnancy).

Case report: At the age of 32, diagnosis of MAS with multiple endocrine disorders (acromegaly, peripheral hyperthyroidism, hyperparathyroidism) was diagnosed. Neurosurgery and/or irradiation was not possible due to serious osteodysplasia in sellar region and medicament treatment was started using long-acting somatostatin analogue lanreotide, which was later combined with GH antagonist pegvisomant. At the age of 36, woman became pregnant. The patient insisted on pregnancy. We continued lanreotide and pegvisomant treatment. Pregnancy and delivery was not complicated and healthy girl was in 38th week of gestation born (cesarean section). Lactation was blocked. Further development of this girl is normal. We continue with lanreotide and pegvisomant treatment.

Conclusion: We report successful pregnancy in an acromegalic patient with MAS treated by combination of pegvisomant and lanreotide. Child’s weight and length was lower, influence of therapy by lanreotide and pegvisomant can be discussed. Father’s body constitution could have some influence as well. No congenital malformations were present, development is physiological, there are no signs of endocrine/bone disorders.