{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:20:39Z","timestamp":1784557239798,"version":"3.55.0"},"reference-count":39,"publisher":"Bioscientifica","issue":"5","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,2,22]]},"abstract":"<jats:p>\n                    Experimental models for pheochromocytoma and paraganglioma are needed for basic pathobiology research and for preclinical 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\n                    <jats:italic>Sdhb<\/jats:italic>\n                    mutations. There are also innovative approaches to preclinical 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 vs 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\n                    <jats:italic>in vitro<\/jats:italic>\n                    studies include species differences, phenotype drift, changes that occur in the transition from tissue to cell culture, and the O\n                    <jats:sub>2<\/jats:sub>\n                    concentration in which cultures are maintained.\n                  <\/jats:p>","DOI":"10.1530\/erc-22-0405","type":"journal-article","created":{"date-parts":[[2023,2,22]],"date-time":"2023-02-22T05:37:31Z","timestamp":1677044251000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-8","source":"Crossref","is-referenced-by-count":6,"title":["Progress and challenges in experimental models for pheochromocytoma and paraganglioma"],"prefix":"10.1530","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9726-5739","authenticated-orcid":true,"given":"Arthur S","family":"Tischler","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Tufts Medical Center and Tufts University School of Medicine, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8190-5853","authenticated-orcid":true,"given":"Judith","family":"Favier","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris cit\u00e9, Inserm UMR970 PARCC, Equipe Labellis\u00e9e par la Ligue contre le cancer, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Cell Reports","article-title":"SDHB knockout and succinate accumulation are insufficient for tumorigenesis but dual SDHB\/NF1 loss yields SDHx-like pheochromocytomas","volume":"38","author":"Armstrong","year":"2022","unstructured":"ArmstrongNStoreyCMNollSEMargulisKSoeMHXuHYehBFishbeinLKebebewEHowittBE, 2022SDHB knockout and succinate accumulation are insufficient for tumorigenesis but dual SDHB\/NF1 loss yields SDHx-like pheochromocytomas. 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