ECE2016 Eposter Presentations Pituitary - Clinical (83 abstracts)
Combined treatment with octreotide LAR and pegvisomant in patients with gigantism – acromegaly: clinical evaluation and genetic screening
Liliya Rostomyan 1 , Ruth Mangupli 3 , Emilie Castermans 2 , Jean-Hubert Caberg 2 , Paul Camperos 3 , Elvia Cuauro 3 , Vincent Bours 2 , Adrian F Daly 1 & Albert Beckers 1
1Department of Endocrinology, Centre Hospitalier Universitaire de Liège, University of Liège, Domaine Universitaire du Sart-Tilman, Liège, Belgium; 2Department of Clinical Genetics, Centre Hospitalier Universitaire de Liège, University of Liège, Liège, Belgium; 3Section of Neuroendocrinology, Department of Neurosurgery, Hospital Universitario de Caracas, Caracas, Venezuela.
Pituitary gigantism is a rare condition caused by growth hormone secreting lesions, where treatment is usually challenging, especially in cases with genetic predisposition. Aim: We studied a gigantism cohort from Venezuela for genetic defects and their response to treatment. Subjects: 160 somatotropinoma patients were evaluated at the University hospital (from 1985–2015); eight (6M) were diagnosed with acrogigantism and underwent genetic analysis including aCGH for Xq26.3 duplications. Results: All patients had accelerated growth rates/tall stature at first evaluation. The mean age at diagnosis was 18.7 years (range 12–28). Among other frequent signs/symptoms at presentation were acral enlargement (7/8) and headache (5/8). All presented with growth hormone-secreting macroadenomas (all except one were invasive) with prolactin co-secretion seen in 4 cases. Six cases received primary medical treatment with the long-acting somatostatin analogue (SSA) octreotide LAR 20 mg/28 days for 6–12 months resulting in tumor control in 64%, in 2 patients SSA treatment was administrated after unsuccessful surgery (radiotherapy was also used in one case). Cabergoline was added in those with elevated prolactin levels. None of the patients had normalization of IGF-1 levels with SSA. Pegvisomant (20 mg daily) was added. And this combination resulted in a decrease of IGF-1 levels to normal ranges while tumor volume was stable in all patients. Regression of clinical symptoms was seen after 1–4 months of treatment including a decrease in growth velocity. Three novel AIP mutations were identified and none of the patients had Xq26.3 microduplications. Clinical characteristics at baseline and treatment responses were not different in patients with AIP mutations compared with AIP negatives.
Conclusions: Patients with gigantism have large and aggressive GH secreting pituitary lesions difficult to control with conventional treatment options. Prolactin co-secresion is frequent. Combined therapy (long-lasting SSA and pegvisomant) as primary treatment or after pituitary surgery and radiotherapy can permit the normalization of IGF-1 levels and achieve clinical improvement in these difficult to manage patients.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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