J Neurol Surg B 2014; 75 - A234
DOI: 10.1055/s-0034-1370640

ACTH-Secreting Esthesioneuroblastoma Occurring after Treatment for Crooke's Cell Adenoma

Sarah J. Burgin 1, Andrew S. McDaniel 1, Jonathan B. McHugh 1, Stephen Sullivan 1, Erin McKean 1
  • 1Ann Arbor, USA

Introduction: Crooke's cell adenomas are a rare but clinically aggressive variant of ACTH-secreting pituitary macroadenoma. They have a high rate of recurrence, reported around 60%, and a 10–15% mortality rate. Esthesioneuroblastoma is a rare neuroendocrine tumor which arises from the olfactory epithelium of the anterior cranial base. Rare cases of ACTH-secreting esthesioneuroblastomas have been reported. Here, we report a case of ACTH-secreting esthesioneuroblastoma occurring 2 years after treatment for Crooke's cell adenoma.

Methods: Case report

Results: A 70 year old gentleman developed Cushing's syndrome and underwent transsphenoidal biopsy at an outside institution. This was diagnosed as an ACTH secreting pituitary adenoma, and was treated with radiation therapy. He developed a delayed cerebrospinal fluid leak, and presented to the University of Michigan Cranial Base Clinic. He underwent repair with a fat graft and a nasoseptal flap. Two years later, he presented with acute onset right abducens palsy. Imaging was negative for tumor recurrence. Over the ensuing 6 weeks, he developed progressive right retroorbital and temporal headaches and daily epistaxis, and imaging was repeated. This revealed an aggressive mass centered in the sphenoid and sella turcica, and extending into the clivus and right petrous apex. The mass encased the internal carotid artery. Repeat biopsy revealed an ACTH-positive esthesioneuroblastoma, which stained positive for CAM5.2 in rare cells and was negative for AE1/AE3. Review of the initial biopsy revealed a CAM5.2 and AE1/AE3 positive tumor, consistent with Crooke's cell adenoma. The patient was treated with repeat radiation therapy, cisplatin and etopiside.

Conclusion: It is unclear if the esthesioneuroblastoma represents a second primary tumor, a response to radiation, or transformation of the original adenoma. This case represents the first report of a pituitary macroadenoma and an esthesioneuroblastoma occurring in a single patient.