Ectopic Pituitary Adenomas Presenting as Sphenoid or Clival Lesions: Case Series and Management Recommendations

Abstract

Background An ectopic pituitary adenoma presenting as a clival or sphenoid mass is a rare clinical occurrence that may mislead the clinician and result in unnecessary interventions or potential medicolegal consequences. Here, we present one of the largest multi-institutional case series and review the literature with an emphasis on radiological findings and critical preoperative workup. Methods Retrospective chart review. Results Nine patients were identified with ectopic pituitary adenomas of the sphenoid or clivus. There were four females and five males. Median age was 60 years old (range, 36-73 years). The most common presenting symptom was headache (56%). Five (56%) patients presented with a mass arising from the clivus while four (44%) presented with a mass in the sphenoid. Six (67%) patients demonstrated biochemical evidence of hypersecretion on full endocrinology panel. All masses showed evidence of enhancement with gadolinium with a propensity for adjacent bone involvement. Lesions also had a predilection for growth toward the cavernous sinus, carotid artery, or sellar floor. Surgical intervention was performed in eight patients (89%). In eight patients (89%), tumors demonstrated immunoreactivity to prolactin. Conclusions Pituitary adenomas can rarely present as an isolated sphenoid or clival mass. Lesions displayed similar magnetic resonance imaging findings with an erosive growth pattern toward the sellar floor, cavernous sinus, or adjacent carotid artery. Patients with clival or parasellar lesions with comparable features should have a preoperative workup which includes prolactin level and alert the physician to consider an ectopic pituitary adenoma in the differential to prevent unnecessary surgery and potential complications.

Ectopic invasive ACTH-secreting pituitary adenoma mimicking chordoma: a case report and literature review

“…The patient in our case demonstrated ACTH immunoreactivity but with nonspecific symptoms. Although lack of full-workups of pituitary function, it is similar to a previous case report [ 15 ]. This type of tumor is usually incidentally discovered in routine imaging for other conditions.…”

“…EPA may show aggressive behavior. Bone invasion [ 15 ], tumor seeding [ 16 ], and malignant transformations [ 17 – 19 ] have also been reported in several case series. The mechanism of this aggressive behavior is unclear.…”

Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenoma in the Clivus Region: A Case Report

“…It is generally considered that during the development of the anterior pituitary lobe, the incompletely degraded Rathke cleft cyst remnants of the Rathke pouch lead to the formation of EPAs in the nasopharynx, sphenoid, and clivus. 10,11 EPA is rare in China. Zhu et al 5 recorded 14,357 pituitary gland patients in the last 20 years; of these patients, only 14 were diagnosed with EPA (0.098% of all cases), but none of the lesions The postoperative pathological and immunohistochemical results of the tumor tissue in the patient demonstrated that it was an ectopic ACTH-secreting pituitary macroadenoma in the clivus region.…”

Clival prolactinoma masquerading as a chordoma: a case report

“…Extra-sellar or ectopic pituitary adenomas (EPAs) most likely arise from remnants of the embryological migratory path of the pituitary gland [ 1 , 3 , [5] , [6] , [7] ]. EPAs are a rare presentation of pituitary adenomas and have mostly been described in case reports [ 3 , 5 , [8] , [9] , [10] , [11] , [12] ]. Because they are so uncommon, EPAs may be misdiagnosed as other skull lesions such as chordomas, chondrosarcoma, meningioma or astrocytomas [ 3 , 8 ].…”