INTRODUCTION
Shwachman–Diamond syndrome (SDS; OMIM 260400) is a rare autosomal-recessive multisystem disorder characterized by exocrine pancreatic dysfunction and neutropenia.1, 2 It is mostly caused by biallelic germline variants in the Shwachman–Bodian–Diamond syndrome (SBDS ) gene.3 The disease’s natural evolution is characterized by a long phase of mild neutropenia, which can evolve into aplastic anemia, myelodysplastic syndrome (MDS) and/or acute myeloid leukemia (AML) (∼35% by 30 years of age).2, 4 In addition, results of a recent study emphasized the relationship between tumor protein 53 (TP53 ) mutations and malignant hemopathies in SDS.5 Solid tumors have rarely been reported in SDS. Only four have been published so far: ovarian cancer,6breast ductal adenocarcinoma,7dermatofibrosarcoma8and pancreatic adenocarcinoma9(Table 1). Moreover, to date, no cohort study has estimated the solid-tumor rate or identified risk factors. That context incited us to report the French Severe Chronic Neutropenia Registry’s experience.