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Resume Les tumeurs neuroendocrines du sein sont des tumeurs rares, peu connues et dont l’existence meme est parfois remise en cause. Les donnees de la litterature sont issues de series retrospectives, heterogenes et de faibles effectifs. Leur incidence est estimee entre 2 et 5 % des cancers du sein par l’Organisation mondiale de la sante (OMS). Elles sont definies par une architecture neuroendocrine et l’expression par les cellules tumorales des marqueurs neuroendocrines chromogranine A et/ou synaptophysine. La classification OMS revisee en 2012 distingue trois sous-types : (i) les tumeurs neuroendocrines bien differenciees ou carcinoid-like, (ii) les tumeurs neuroendocrines peu differenciees ou carcinomes a petites cellules, et (iii) les carcinomes invasifs du sein presentant une differentiation neuroendocrine. Leur presentation clinique et radiologique n’est pas specifique. Les donnees de la litterature etant discordantes, leur impact pronostique est encore debattu. La prise en charge therapeutique n’est pas codifiee et se superpose habituellement a celle des cancers du sein classiques. La place des traitements plus specifiques de type neuroendocrine comme les associations sels de platine – etoposide, les analogues de la somatostatine, la radiotherapie metabolique ou le temozolomide reste a definir. Des cas de reponse ont ete observes. Une meilleure connaissance des voies impliquees dans la carcinogenese de ces tumeurs pourrait egalement permettre de decouvrir des cibles therapeutiques potentielles. L’efficacite de therapies ciblees dans cette indication est a evaluer.

TPS2066 Background: Better knowledge on the evolving epidemiology and biology of CNS metastases are key elements for the development of new treatment strategies and identification of promising therapeutic targets. A multidisciplinary Brain Metastases Clinical Research Platform called BrainStorm was launched by the Jules Bordet Institute and the Oncodistinct network. The BrainStorm program includes mainly patients with non-CNS metastatic solid tumors with high risk of developing CNS metastases allowing to build a large database focusing on three time periods: before the diagnosis of CNS metastases (Part A), at diagnosis (Part B) and after the diagnosis of CNS metastases (Part C). Methods: Subjects with newly diagnosed non-CNS metastases or up to 24 months from diagnosis of non-CNS metastases from triple-negative and HER2-positive breast cancer, lung cancer and melanoma are eligible for part A of the program. Subjects presenting with a 1st CNS event and not yet enrolled (previously mentioned cohorts and a cohort of other tumor types) can enter directly in parts B and subsequently part C of the study. Eligible subjects are followed for 48 months for relevant clinical data, neurological examinations, quality of life, survival status, and undergo examinations and samplings (Table 1). A total of 280 subjects (40 per cohort) with a 1st CNS event will be enrolled. The main objectives of the program are to collect clinical and biological data with the aim to identify risk factors for CNS metastases development (Part A) and to better understand the biology of CNS metastases (brain and leptomeningeal –Parts B and C) aiming to discover new targets for therapy and intensify the multidisciplinary approach for the management of CNS metastases from solid tumors. The translational part of the program will evaluate among others the use of cerebrospinal fluid circulating tumor DNA (CSF-ctDNA) as a surrogate for CNS metastases in order to characterize its molecular landscape. Currently, the study is recruiting in several sites within the Oncodistinct network. The data collected will help to develop innovative multidisciplinary research projects that could be implemented in all parts of the BrainStorm program. Clinical trial information: NCT04109131. [Table: see text]

Primary neuroendocrine breast carcinomas are rare and little-known tumors. Only a limited number of studies on neuroendocrine breast carcinomas have been reported in the literature, and the vast majority of them are small retrospective series or case reports. According to the World Health Organization (WHO), they account for only 2 % to 5 % of breast cancers. Their diagnosis relies on the presence of a neuroendocrine architecture and the expression of neuroendocrine markers (chromogranin A and/or synaptophysin). The revised 2012 WHO classification subdivides them into three categories: (i) well-differentiated neuroendocrine carcinomas, (ii) poorly differentiated neuroendocrine carcinomas or small-cell carcinomas, and (iii) invasive breast carcinomas with neuroendocrine differentiation. Their clinical features and radiological characteristics are not different from those of other types of breast cancer. Because of discordant results, their clinical outcome is still poorly defined. So far, no standard treatment has been established, and most clinicians draw on their experience of invasive ductal cancer. The role of specific treatments like platinum-based chemotherapy, somatostatin analogues, peptide receptor radionucleide therapy or temozolomide remains unclear. A better knowledge of the molecular pathways involved in their carcinogenesis could help to identify new potential therapeutic targets. The efficacy of targeted therapies has to be studied.