Impact of preoperative pasireotide therapy on invasive octreotide-resistant acromegaly

[摘要] A 43-year-old woman with an 8-year history of diabetes, hypertension, and dyslipidemia presented with amenorrhea and convulsion. Her MRI scan revealed a 3.5-cm T2-hyperintense pituitary macroadenoma with suprasellar extension to the frontal lobe and bilateral cavernous sinus invasion. Her serum levels of GH and insulin-like growth factor-I (IGF-I) were elevated to 9.08 ng/mL (normal range: <2.1 ng/mL) and 1,000 ng/mL (normal range: 90–233 ng/mL, SD score +10.6), respectively. Bromocriptine insufficiently suppressed her GH levels, while octreotide paradoxically increased her GH levels. Together with her characteristic features, she was diagnosed with acromegaly caused by an invasive GH-producing pituitary macroadenoma. As performing a one-stage operation would have been extremely difficult, she was first treated with pasireotide long-acting release (40 mg monthly) for 5 months followed by a successful transsphenoidal surgery. One month after the first injection, biochemical control was achieved (IGF-I, 220 ng/mL; GH, 1.26 ng/mL), and tumor shrinkage of approximately 50% was observed. The resected tumor was histologically diagnosed as a sparsely granulated somatotroph adenoma, with higher expression of somatostatin receptor subtype 5 (SSTR5) than that of SSTR2A. The germline aryl hydrocarbon receptor interacting protein (AIP) mutation was negative, and several tumor cells were weakly immunoreactive for AIP. Despite the presence of a residual tumor postoperatively, biochemical control was achieved 6 months after the final injection of pasireotide. In conclusion, this case suggests that pasireotide may be an option for preoperative first-line therapy in invasive and octreotide-resistant sparsely granulated somatotroph adenomas.

[学科分类] 内分泌与代谢学

[关键词] Acromegaly;Pasireotide;Octreotide resistance;Somatostatin receptor 5