Histiocytic and dendritic cell neoplasms.

BACKGROUND

Histiocytic and dendritic cell neoplasms (HDCNs) represent very rare tumors, which have been the subject of debate during the last few years.

OBJECTIVES

We aimed to provide a comprehensive review of the subject.

MATERIALS AND

METHODS

The experience gained by the authors in large international studies and as a national reference center has been summarized to highlight the characteristics of each entity.

RESULTS

The clinical, morphologic, phenotypic, and molecular data of the different entities included under the heading of HDCNs are extensively discussed.

CONCLUSION

Currently, HDCNs are classified in the group of orphan diseases for which a standardized therapy is lacking. An international registry would facilitate expansion and dissemination of knowledge of these diseases and improve their treatment.