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J Neurol Surg B Skull Base 2022; 83(S 02): e253-e259
DOI: 10.1055/s-0041-1725024
Original Article

Delayed Craniospinal Metastasis of Aggressive Nonfunctioning Pituitary Adenomas as Pituitary Carcinomas

Ashley L. B. Raghu 
1   Nuffield Department of Surgical Sciences, University of Oxford, Oxford, United Kingdom
,
Megan C. Everson
2   Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, United States
,
Ahmed Helal 
2   Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, United States
,
Satoshi Kiyofuji 
2   Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, United States
,
Michelle J. Clarke
2   Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, United States
,
Michael J. Link
2   Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, United States
› Author Affiliations Funding None.
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Abstract

Background Clinical behavior of pituitary neoplasms is peculiar and notoriously difficult to predict. While aggressive tumors are common, metastasis is very rare, can be highly delayed, and there are no histological or clinical features to meaningfully predict this happening. Endocrinologically silent tumors are particularly difficult, as there is less opportunity to detect early metastasis. Together, this amounts to a situation of uncertainty over the appropriate management of such tumors before and after metastasis.

Case Description The authors report two cases of nonfunctioning aggressive pituitary adenoma (APA) each requiring two transsphenoidal surgeries, a transcranial resection and radiotherapy. Both these tumors subsequently metastasized caudally along the neuraxis, years later, as a null cell carcinoma associated with a germline CHEK2 mutation and a silent Crooke's cell carcinoma. The former represents a novel oncogenetic association.

Conclusion Delayed drop dural metastasis of pituitary carcinoma is becoming increasingly recognized. Surgical resection of the distant disease to confirm the diagnosis and relieve the mass effect, followed by temozolomide chemotherapy, is the current treatment of choice. The need for both long-term follow-up in patients with APA, and a high degree of suspicion toward dural-based radiographic findings is emphasized.

Keywords

pituitary carcinoma - pituitary adenoma - nonfunctioning - CHEK2 - Crooke's cell

Note

This manuscript has not been previously published in whole or in part or in submission elsewhere for review.




Publication History

Received: 15 April 2020

Accepted: 13 January 2021

Article published online:
01 March 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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