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Rare disease
Case report
Primary neuroendocrine carcinoma of the brain
  1. Clay T Reed1,
  2. Narjust Duma2,
  3. Thorvardur Halfdanarson2 and
  4. Jan Buckner2
  1. 1 Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA
  2. 2 Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Clay T Reed, clayreed7{at}gmail.com

Abstract

Neuroendocrine neoplasms (NENs) are malignancies with rare reports of central nervous system development. A 34-year-old woman was found to have a primary NEN of the brain, and she had recurrence with identical histology 10 years later. Extracranial NENs were excluded. She had routine surveillance for the first 5 years with MRIs and positron emission tomography/CTs after the initial presentation which was treated with radiation followed by cisplatin and etoposide. This case highlights the difference in primary NENs versus NEN metastases to the brain, and that longer periods of surveillance are likely required for primary NENs. This is important because the prognosis between primary NENs and metastatic NENs to the brain are vastly different and should not be treated as equal diseases. The patient eventually died of her recurrence secondary to complications of a ventriculoperitoneal shunt placed for treatment of hydrocephalus from the disease.

  • neurooncology
  • neuroendocrinology
  • CNS Cancer

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/bcr-2019-230582

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    Footnotes

    • Contributors CTR was the primary author of this paper and did the primary research and writing. ND helped with literature review and editing of the paper. TH and JB helped with guidance of current neuroendocrine tumour guidelines and helped review/edit the paper.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Patient consent for publication Next of kin consent obtained.