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A 53-year-old woman presented to our hospital with visual impairment and diplopia. The symptoms appeared about 2 weeks before. The patient had a diagnosis of neuroendocrine breast carcinoma (NBC) 10 years earlier. The cerebral MR showed the presence of a brain tumour with neuroradiological features suggestive of both high-grade glioma and primitive neuroectodermal tumour (figure 1). We performed a subtotal resection that led to transient aphasia and right hemiparesis.
Histological examination revealed metastasis from poorly differentiated neuroendocrine carcinoma composed of clusters of cells with abundant cytoplasm and vesicular nuclei with finely granular chromatin and high number of mitoses (figure 2A,B). Immunostains showed intense and diffuse positivity for pan-cytokeratin and synaptophysin (figure 2C, lefts panels), suggesting their neuroendocrine phenotype, and positivity for E-Cadherin and GATA-3, confirming the breast primitivity (figure 2C, right panels). Assessment of prognostic and predictive factors showed diffuse positivity for oestrogen receptors, very few positive nuclei for progesterone receptors (figure 2D, left panels) and low expression of c-erbB-2 (Her2-neu) (score 1). Proliferation index was very high (figure 2D, right panels).
Neuroendocrine tumours (NETs) are rare and usually affect lung, pancreas and gastrointestinal tract.1 Breast localisation is observed in less than 0.5% of cases.2 3 NETs rarely metastasise to the brain. The literature reported only six cases of cerebral metastases of NETs, but none of these were an NBC.4 5 NBC was first described in 1977 and was characterised by the presence of positive neuroendocrine markers in over 50% of the tumour cells. According to the 2012 WHO classification, NBC was subclassified into well-differentiated NET, poorly differentiated neuroendocrine carcinoma and invasive breast carcinoma with neuroendocrine differentiation, regardless of the percentage of neuroendocrine markers.3
Our case showed that NBC may metastasise to the brain. Moreover, we observed that in case of NBC cerebral metastasis, the neuroradiological pattern is absolutely atypical and may look like both glioblastoma and neuroectodermal tumour.
Neuroendocrine breast carcinoma (NBC) is a rare tumour that may metastasise to the brain even over 10 years.
NBC cerebral metastases have an atypical neuroradiological pattern with features of both high-grade glioma and primitive neuroectodermal tumour.
Contributors LZ: planning, interpretation of data and reporting. LP: planning of the paper and data analysis. RL: reporting and acquisition of data. PPP: conception, design and conduct of the work.
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 Obtained.
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