Progress and challenges in experimental models for pheochromocytoma and paraganglioma

in Endocrine-Related Cancer
Authors:
Arthur S TischlerA Tischler, Department of Pathology and Laboratory Medicine, Tufts University School of Medicine, Boston, United States

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Judith FavierJ Favier, Inserm, UMR970, PARCC, Université Paris Cité, Paris, France

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Correspondence: Arthur Tischler, Email: atischler@tuftsmedicalcenter.org
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Experimental models for pheochromocytoma and paraganglioma are needed for basic pathobiology research and for pre-clinical testing of drugs to improve treatment of patients with these tumors, especially patients with metastatic disease. The paucity of models reflects the rarity of the tumors, their slow growth and their genetic complexity. While there are no human cell line or xenograft models that faithfully recapitulate the genotype or phenotype of these tumors, the past decade has shown progress in development and utilization of animal models, including a mouse and a rat model for SDH-deficient pheochromocytoma associated with germline Sdhb mutations. There are also innovative approaches to pre-clinical testing of potential treatments in primary cultures of human tumors. Challenges with these primary cultures include how to account for heterogeneous cell populations that will vary depending on the initial tumor dissociation, and how to distinguish drug effects on neoplastic versus normal cells. The feasible duration for maintaining cultures must also be balanced against time required to reliably assess drug efficacy. Considerations potentially important for all in vitro studies include species differences, phenotype drift, changes that occur in transition from tissue to cell culture and the O2 concentration in which cultures are maintained.

 

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