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An Institutional Experience of Tumor Progression to Pituitary Carcinoma in a 15-Year Cohort of 1055 Consecutive Pituitary Neuroendocrine Tumors

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Abstract

Pituitary carcinoma is a rare disease, defined by the presence of cerebrospinal or distant metastasis of a pituitary neuroendocrine tumor (PitNET). To review our institutional experience of pituitary carcinoma, we searched the database of the UHN Endocrine Oncology Site group and the University Health Network pathology laboratory information system from 2001 to 2016. Among 1055 PitNETs from 1169 transsphenoidal resections, we identified 4 cases of pituitary carcinoma, indicating that pituitary carcinoma represents around 0.4% of PitNETs. All four patients were women. The age at initial presentation ranged from 23 to 54 years. Two patients had Cushing disease with corticotroph tumors; one was initially a densely granulated corticotroph tumor that evolved to become sparsely granulated, while the other was a Crooke cell tumor. One patient had a functioning sparsely granulated lactotroph tumor and one had a clinically silent poorly differentiated PIT1 lineage tumor. Apart from a relatively high Ki67 labeling index (≥ 10%) in three tumors, there were no cytomorphologic features at the time of initial presentation that could predict subsequent metastatic behavior. The time from diagnosis of the pituitary neuroendocrine tumor to the diagnosis of malignancy was 3 to 14 years. Therapies included somatostatin analogs, external beam radiotherapy, chemotherapies including capecitabine/temozolomide, everolimus, sunitinib, bevacizumab, and peptide receptor radionuclide therapy (PRRT). One patient died of disease 18 years after initial diagnosis, underscoring the protracted course of this ultimately fatal neuroendocrine malignancy.

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Authors and Affiliations

  1. Department of Medicine, Princess Margaret Cancer Centre, University Health Network and University of Toronto, Toronto, Ontario, Canada

    Omalkhaire M. Alshaikh & Shereen Ezzat

  2. Department of Medicine, Al Imam Mohammad Ibn Saud Islamic University, Riyadh, Saudi Arabia

    Omalkhaire M. Alshaikh

  3. Department of Pathology, University Health Network, Toronto, Canada

    Sylvia L. Asa & Ozgur Mete

  4. Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada

    Sylvia L. Asa & Ozgur Mete

  5. Princess Margaret Cancer Centre, University Health Network, 610 University Avenue, Room 7-327, Toronto, Ontario, M5G 2M9, Canada

    Shereen Ezzat

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  1. Omalkhaire M. Alshaikh
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Correspondence to Shereen Ezzat.

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Dr. Asa is a member of the Medical Advisory Boards of Leica Pathology Imaging and Ibex Medical Analytics. Dr. Ezzat has served on Medical Advisory Boards of Novartis Canada, Pfizer, Ipsen, and Esai. Drs. Alshaikh and Mete have no conflicts of interest or funding to disclose.

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Alshaikh, O.M., Asa, S.L., Mete, O. et al. An Institutional Experience of Tumor Progression to Pituitary Carcinoma in a 15-Year Cohort of 1055 Consecutive Pituitary Neuroendocrine Tumors. Endocr Pathol 30, 118–127 (2019). https://doi.org/10.1007/s12022-019-9568-5

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