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Clin Genitourin Cancer. 2018 Sep 12. pii: S1558-7673(18)30384-7. doi: 10.1016/j.clgc.2018.09.005. [Epub ahead of print]

Updated Recommendations on the Diagnosis, Management, and Clinical Trial Eligibility Criteria for Patients With Renal Medullary Carcinoma.

Author information

1
Department of Genitourinary Medical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, TX.
2
Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA.
3
Lombardi Comprehensive Cancer Center, Georgetown University, Washington, DC.
4
Center for Precision Environmental Health, Department of Molecular and Cell Biology, Baylor College of Medicine, Houston, TX.
5
Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
6
Departments of Pediatrics and Medicine, Division of Hematology, UNC School of Medicine, Chapel Hill, NC.
7
Department of Genitourinary Medical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, TX; Department of Genomic Medicine, the University of Texas M.D. Anderson Cancer Center, Houston, TX.
8
Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD.
9
Department of Pathology, The University of Texas M.D. Anderson Cancer Center, Houston, TX.
10
Translational Surgical Pathology Section, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.
11
Division of Pediatric Hematology/Oncology, BronxCare Health System, Bronx, NY.
12
Division of Pediatric Oncology, Children's National Medical Center, Washington, DC.
13
Division of Pediatric Hematology/Oncology, IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada.
14
Division of Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH.
15
Genitourinary Malignancies Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD.
16
Department of Pediatrics, The University of Texas M.D. Anderson Cancer Center, Houston, TX.
17
Center for Precision Environmental Health, Department of Molecular and Cell Biology, Baylor College of Medicine, Houston, TX; Center for Cancer Epigenetics, The University of Texas M.D. Anderson Cancer Center, Houston, TX.
18
Division of Nephrology, Children's National Medical Center, Washington, DC.
19
Department of Urology, Klinikum Grosshadern, Ludwig Maximilians University of Munich, Munich, Germany.
20
Department of Urology, The University of Texas M.D. Anderson Cancer Center, Houston, TX.
21
Division of Hematology/Oncology, Vanderbilt-Ingram Cancer Center, Vanderbilt University Medical Center, Nashville, TN.
22
Department of Genitourinary Medical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, TX. Electronic address: ntannir@mdanderson.org.

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.

KEYWORDS:

INI1; Renal cell carcinoma; SMARCB1; Sickle hemoglobinopathies; Unclassified renal cell carcinoma with medullary phenotype

PMID:
30287223
DOI:
10.1016/j.clgc.2018.09.005

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