Synchronous solid neuroendocrine breast carcinoma and abdominal lymphoma: A case report and review of the literature

Neuroendocrine tumors (NETs) are frequently associated with second primary malignancies (SPMs). Earlier studies have demonstrated that NETs are highly associated with synchronous or metachronous gastrointestinal and genitourinary SPMs. We report, for the first time, a case of pure NE breast carcinoma (NEBC) exhibiting all of the World Health Organization (WHO)-categorized morphological and phenotypic NE features (i.e., round solid nests of spindle cells, plasmacytoid cells, large clear or mucinous signet-ring cells with a peripheral palisading tendency and immunohistochemical positivity for the NE markers synaptophysin and chromogranin in more than 50% of the tumor cell population) along with synchronous abdominal non-Hodgkin’s lymphoma. In the present study, we review the diagnosis, clinicopathological features and histogenetic profiling of NEBC and discuss the literature relevant to the clinical and anatomopathological management of this case. This previously unreported case of synchronous solid NEBC and abdominal lymphoma, together with earlier studies showing that primary symptoms are caused by SPMs in a significant subgroup of NET patients, strongly supports the notion that NETs should be cautiously considered to be index tumors. Therefore, risk-adapted clinicopathological follow-up with systematic investigation is strongly recommended.