Renal medullary carcinoma: Clinical and therapeutic aspects of a newly described tumor

• Published in: Cancer 1996-07, Vol.78 (1), p.128-132
• Main Authors: Avery, Robert A., Harris, Jules E., Davis, Charles J., Borgaonkar, Digamber S., Byrd, John C., Weiss, Raymond B.
• Format: Article
• Language: English
• Subjects: Adult; Age Factors; Biological and medical sciences; Black or African American; Black People; Carcinoma, Medullary - drug therapy; Carcinoma, Medullary - genetics; Carcinoma, Medullary - pathology; chemotherapy; Chromosome Aberrations - genetics; Chromosome Disorders; cytogenetics; Female; Humans; Karyotyping; kidney neoplasms; Kidney Neoplasms - drug therapy; Kidney Neoplasms - genetics; Kidney Neoplasms - pathology; Kidneys; Male; Medical sciences; Nephrology. Urinary tract diseases; renal medullary carcinoma; sickle cell trait; Sickle Cell Trait - complications; Tumors of the urinary system
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/(SICI)1097-0142(19960701)78:1<128::AID-CNCR18>3.0.CO;2-1

BACKGROUND Renal medullary carcinoma is a newly described, aggressive kidney tumor. All patients with the disease have been African‐American with sickle cell (SC) trait or hemoglobin SC disease. METHODS Patient information was obtained from individual patient records and from the Department of Defense national data bank, The Defense Enrollment and Eligibility Reporting System. Data were obtained from either personal review of the patient's records or from discussion with the patient's physician. Cytogenetic studies were performed on one patient. RESULTS Six patients are presented. All had SC trait. Median age was 24.5 years, and 1 patient was female. Time from diagnosis to death averaged 3 months (range: 1–7 mos). No objective responses were reported to a wide variety of chemo and immunotherapies: cyclophosphamide, doxorubicin, cisplatin; methotrexate, vinblastine, doxorubicin, and cisplatin; single agent interferon; single agent paclitaxel; or single agent vinblastine. Investigational regimens included topotecan, doxorubicin, and filgrastim; α‐interferon, interleukin‐2, and 5‐fluorouracil; and single agent paclitaxel. Cytogenetic studies revealed numerous structural, as well as numerical anomalies. Of the cells successfully karyotyped (n = 4), 2 contained abnormalities of chromosome 3 and all contained monosomy 11. CONCLUSIONS Renal medullary carcinoma is an aggressive, chemoresistant tumor. Time from discovery of tumor to patient death is very short and has not been altered by a wide variety of chemotherapies and immunotherapies. An unidentified genetic component is likely present. Cancer 1996;78:128‐32.