Two case reports of brain metastases in patients with pancreatobiliary neuroendocrine carcinoma

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CASE REPORT

Two case reports of brain metastases in patients with pancreatobiliary neuroendocrine carcinoma Takaaki Furukawa1 · Takashi Sasaki1   · Tsuyoshi Takeda1 · Takafumi Mie1 · Ryo Kanata1 · Akiyoshi Kasuga1 · Masato Matsuyama1 · Masato Ozaka1 · Naoki Sasahira1 Received: 13 August 2020 / Accepted: 10 October 2020 © Japanese Society of Gastroenterology 2020

Abstract Brain metastases are extremely rare in patients with pancreatobiliary neuroendocrine caricnoma (PB-NEC). In this case report, we report two rare cases of brain metastases in patients with PB-NEC. Each patient was diagnosed with brain metastases five and ten months after the initial diagnosis of PB-NEC. It is noteworthy that the serum tumor marker neuron-specific enolase (NSE) or pro-gastrin-releasing peptide (Pro-GRP) was elevated, although the primary and metastatic lesions other than in the brain were under control with systemic chemotherapy. Moreover, the patients complained of no neurological symptoms until they were diagnosed with brain metastases. Although the incidence of brain metastases of PB-NEC is exceedingly low, it is important to keep in mind the possibility of brain metastases during the course of treatment for PB-NEC. In addition, we discuss a strategy of treatment and screening for brain metastases of PB-NEC in this case report. Keywords  Pancreatobiliary neuroendocrine carcinoma · Brain metastasis · Chemotherapy · Radiation therapy · Surgical resection Abbreviations PB-NEC Pancreatobiliary neuroendocrine carcinoma GEP-NEC Gastroenteropancreatic neuroendocrine carcinoma CDDP Cisplatin CPT-11 Irinotecan NSE Neuron-specific enolase MRI Magnetic resonance imaging WBRT Whole-brain radiation therapy CPA Cardiopulmonary arrest Pro-GRP Pro-gastrin-releasing peptide CT Computed tomography SRS Stereotactic radiosurgery SFRT Stereotactic fractionated radiotherapy SCLC Small-cell lung cancer BBB Blood–brain barrier CNS Central nervous system PCI Prophylactic cranial irradiation

* Takashi Sasaki sasakit‑[email protected] 1



Department of Hepato‑Biliary‑Pancreatic Medicine, Cancer Institute Hospital of Japanese Foundation for Cancer Research, 3‑8‑31, Ariake, Koto, Tokyo 135‑8550, Japan

Introduction Pancreatobiliary neuroendocrine carcinoma (PB-NEC) is a rare neoplasm [1], which is often included under gastroenteropancreatic NEC (GEP-NEC) upon statistical analysis. The annual incidence of GEP-NEC is estimated to be 0.04–0.54 per 100,000 population worldwide [2–4] and 0.32 per 100,000 population in Japan [4]. Among cases of GEPNEC, pancreatic NEC accounts for approximately 40% of the cases, whereas biliary NEC accounts for only 0.4–2% of the cases [5–9]. The most common site of metastasis is the liver (70%), followed by the lungs (15%) and bone (15%); additionally, intracranial metastasis is very rare (1–5%) [10]. Herein, we report two cases of brain metastases in patients with PB-NEC and discuss a strategy of treatment and screening for brain metastases of PB-NEC.

Case reports Case 1 A 46-year-old man was diagnosed with gall