Endocrine Abstracts

Introduction

Silent corticotroph adenoma represent an uncommon subtype of nonfunctioning adenoma, immunoreactive for ACTH, without clinical or biochemical evidence of hypercortisolism and unclear pathogenesis. Usually, they present with local mass effect (visual deterioration being the most common) and endocrine dysfunctions. They carry a more aggressive behavior, particularly upon earlier recurrence.

Case presentation

A 47-year-old, obese male patient, recently diagnosed with pituitary macroadenoma, came to our clinic for emergency endocrine evaluation, accusing suddenly visual field dysfunction and headache 7 days before admission with the left temporal hemianopsy development. At admission, IGF-1, thyroid and adrenal hormone status within normal, mild hyperprolactinemia and hypogonadotrophic hypogonadism. A second opinion on CT and IRM cerebral scans certified pituitary macroadenoma with suprasellar and intrasellar extension, optical chiasm compression and pituitary apoplexy. Ophthalmological examination confirmed right quadranopsy-superior temporal and left temporal hemianopsy. Transphenoidal adenomectony was performed. The immunohistochemistry exam showed positive staining for ACTH, Ki67 proliferation index-4%, p53 positive in rare tumor cells, positive cytokeratin CAM 5.2 and cromogranin with variable intensity in the tumor cells. Visual dysfunctions have markedly improved post surgery. He was discharged with hydrocortisone, levothyroxine and testosterone replacement therapy and periodic follow-up.

Particularities

Silent corticotroph macroadenoma probably grew aggressively. Despite the local mass effect and visual deterioration that are the most common manifestations, our patient didn’t complain about sexual dynamic disorder. Age at diagnose is also peculiar with peak incidence in the 3rd decade, being more frequent in women. The presence of tumor proliferation markers such as ki67 and p53 associate an aggressive pattern and a poor prognosis.

Conclusions

Management of silent corticotroph macroadenoma is complex. Surgery remains the main therapeutic approach. This patient needs to be monitored closely for quick hypopituitarism onset and careful follow-up due to frequent and early recurrences. Rarely, they can change to a more aggressive phenotype or possibly transform to Cushing disease and multimodal therapy is necessary.