Abstract
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.
Zusammenfassung
Hintergrund
Histiozytische und dendritische Zelltumoren sind sehr seltene Tumoren, die in den letzten Jahren Gegenstand von Diskussionen waren.
Ziel der Arbeit
Ziel der Arbeit war es, einen umfassenden Überblick über das Thema zu geben.
Material und Methoden
Die Erfahrung der Autoren, die sie im Rahmen großer internationaler Studien und als Teil des nationalen Referenzzentrums erlangt haben, wurde zusammengefasst, um die Eigenschaften jeder dieser Entitäten darzustellen.
Ergebnisse
Dabei wurden die klinischen, morphologischen, phänotypischen und molekularen Daten der verschiedenen Entitäten, die sich unter dem Oberbegriff der histiozytischen und dendritischen Zelltumoren finden, ausführlich erörtert.
Schlussfolgerung
Derzeit werden histiozytische und dendritische Zelltumoren der Gruppe der seltenen Krankheiten zugeordnet, für die es keine standardisierte Behandlung gibt. Ein internationales Register würde die Erweiterung und Verbreitung des Wissens über diese Erkrankungen erleichtern und zur Verbesserung ihrer Behandlung beitragen.
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References
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Funding
This manuscript was supported by a grant from the Italian Association for Cancer Research (AIRC, Milan; n. 21198).
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S.A. Pileri, F. Melle, G. Motta and V. Tabanelli declare that they have no competing interests.
For this article no studies with human participants or animals were performed by any of the authors. All studies mentioned were in accordance with the ethical standards indicated in each case.
The supplement containing this article is not sponsored by industry.
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Pileri, S.A., Melle, F., Motta, G. et al. Histiocytic and dendritic cell neoplasms. Pathologie 43 (Suppl 1), 119–124 (2022). https://doi.org/10.1007/s00292-022-01116-x
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DOI: https://doi.org/10.1007/s00292-022-01116-x
Keywords
- Histiocytic sarcoma
- Langerhans cell tumors
- Dendritic cell neoplasms of myeloid derivation
- Follicular dendritic cell sarcoma
- Dendritic cell neoplasms of mesenchymal derivation