Large pituitary gland with an expanding lesion in the context of neurofibromatosis 1

Description

A 29-year-old woman with neurofibromatosis 1 (NF1) due to NF1 gene deletion presented for evaluation of long-standing secondary amenorrhea. She noted increasing growth of her hands and feet, inability to fit her ring on her fingers and increasing shoe size (currently US size 12). Physical examination revealed coarse and asymmetrical facial features, arthropathy with large hands and feet and multiple café-au-lait lesions. Biochemical evaluation was consistent with growth hormone (GH) and prolactin excess. Pituitary MRI with T1-weighted images showed a voluminous and symmetrically enlarged gland (overall dimensions 15x9x13 mm), with a heterogeneous hypointense lesion within the posterior pituitary, measuring 6x6x10 mm (figure 1A, arrow). On the fluid-attenuated inversion recovery and T2-weighted images, the lesion was markedly hypointense (not shown). There was no radiographic evidence of optic pathway tumours. Due to both elevations in GH and prolactin, it was decided to treat her acromegaly with cabergoline. A follow-up...

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