Clival prolactinoma masquerading as a chordoma: a case report

Abstract

Ectopic pituitary adenomas are a rare clinical entity and are frequently mistaken for other base of skull lesions on imaging. We report the clinical presentation and management of a woman presenting with an ectopic prolactinoma located in the clivus. A 66-year-old female presented with a 6-month history of headaches and light-headedness. Anatomical imaging demonstrated a clival lesion most suspicious for chordoma. Endocrinological assessment revealed modestly increased prolactin level with lower-than-expected gonadotrophins levels for her age. Surgical resection confirmed an ectopic prolactinoma. A skull base lesion in a patient with hormonal derangement should lend to a high clinical suspicion of an EPA as they may be treated with medications before surgery. Guidelines could assist clinicians investigating skull-based lesions to identify the rare, but important diagnosis of ectopic pituitary adenomas.

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“…Clinical manifestations of EPAs typically arise secondary to hormonal derangements and involvement of adjacent structures causing symptoms of mass effect [1][2][3][4][5]. Common hormonal derangement presentations include Cushing's syndrome secondary to hypercortisolism, acromegaly secondary to growth hormone excess, and hypogonadism and/or galactorrhea secondary to hyperprolactinemia [1,2].…”

“…EPAs are thought to arise from neoplastic transformation occurring within ectopic pituitary tissue that remains along the embryonic pituitary gland migratory path [1][2][3][4][5]. To understand the proposed pathologic transformation that occurs, it is beneficial to briefly review pituitary gland embryogenesis and anatomy.…”