Updated recommendations on the diagnosis, management, and clinical trial eligibility criteria for patients with renal medullary carcinoma Journal Article


Authors: Msaouel, P.; Hong, A. L.; Mullen, E. A.; Atkins, M. B.; Walker, C. L.; Lee, C. H.; Carden, M. A.; Genovese, G.; Linehan, W. M.; Rao, P.; Merino, M. J.; Grodman, H.; Dome, J. S.; Fernandez, C. V.; Geller, J. I.; Apolo, A. B.; Daw, N. C.; Hodges, H. C.; Moxey-Mims, M.; Wei, D.; Bottaro, D. P.; Staehler, M.; Karam, J. A.; Rathmell, W. K.; Tannir, N. M.
Article Title: Updated recommendations on the diagnosis, management, and clinical trial eligibility criteria for patients with renal medullary carcinoma
Abstract: Renal medullary carcinoma (RMC) is one of the most aggressive renal cell carcinomas. It predominantly afflicts young adults and adolescents with sickle cell trait and other sickle hemoglobinopathies, and is refractory to targeted and antiangiogenic therapies used in patients with clear-cell renal cell carcinoma. Platinum-based cytotoxic chemotherapy is the mainstay for RMC treatment. On the basis of recent advances in the diagnosis, management, and clinical trial development for RMC, a panel of experts met in October 2017 and developed updated consensus recommendations to inform clinicians, researchers, and patients. Because RMC often aggressively recurs while patients are still recovering from nephrectomy, upfront chemotherapy should be considered for most patients, including those with localized disease. After safety and dosing information has been established in adults, phase II and III trials enrolling patients with RMC should allow patients aged 12 years and older to be accrued. Patients with the very rare unclassified renal cell carcinoma with medullary phenotype variant should be included in RMC trials. Medical providers should be aware that RMC can afflict subjects of all races, and not only those of African descent, and that the presence of sickle cell trait, or of other sickle hemoglobinopathies, can affect drug responses and toxicity. © 2018 Elsevier Inc.
Keywords: cancer chemotherapy; cancer surgery; clinical feature; note; cancer patient; antineoplastic agent; cancer diagnosis; cancer therapy; renal cell carcinoma; nephrectomy; awareness; medullary carcinoma; clinical trial (topic); phase 2 clinical trial (topic); phase 3 clinical trial (topic); hemoglobinopathy; smarcb1; sickle cell trait; patient advocacy; ini1; human; sickle hemoglobinopathies; unclassified renal cell carcinoma with medullary phenotype
Journal Title: Clinical Genitourinary Cancer
Volume: 17
Issue: 1
ISSN: 1558-7673
Publisher: Elsevier Inc.  
Date Published: 2019-02-01
Start Page: 1
End Page: 6
Language: English
DOI: 10.1016/j.clgc.2018.09.005
PUBMED: 30287223
PROVIDER: scopus
PMCID: PMC6348017
DOI/URL:
Notes: Export Date: 1 February 2019 -- Source: Scopus
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MSK Authors
  1. Chung-Han   Lee
    42 Lee