Renal medullary carcinoma: the Bronx experience.
ABSTRACT Renal medullary carcinoma (RMC) is a devastating and extremely rare malignancy primarily afflicting young men with sickle cell trait. We present our clinical experience with 9 cases of RMC during a 10-year period and briefly review the published data.
A retrospective chart review of 9 cases of RMC during a 10-year period at our institutions was performed. The clinical patient characteristics, presentations, treatments, and outcomes were recorded. The radiographic images and pathologic specimens were reviewed. Applicable studies were selected from a Medline search.
All 9 patients had sickle cell trait, the male/female ratio was 6:3, and the age range was 13 to 31 years. All the patients presented with flank pain, two thirds had hematuria, and 3 of the 9 patients presented with a palpable mass. Eight of the nine tumors were right sided, ranging from 4 to 12 cm in the greatest diameter. Of the 9 patients, 7 underwent radical nephrectomy. One patient was deemed to have unresectable disease by the operating surgeon, and one was given initial chemotherapy after biopsy of a metastatic lesion. The neoadjuvant therapies varied. Overall survival ranged from 4 to 16 months, with 2 patients still living at the last follow-up visit.
Our urban setting likely explains our relatively large experience with this rare and extremely aggressive tumor. An early diagnosis is critical, and a high index of suspicion should be given to any individual with sickle cell trait and new-onset hematuria, especially in the setting of a right-sided mass. Prospective trials are needed for chemotherapy/immunotherapy, because surgical intervention alone is inadequate.
Article: Sickle cell nephropathyDisease-a-Month 10/2014; · 0.54 Impact Factor
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ABSTRACT: Renal medullary carcinoma (RMC) is a rare but aggressive malignancy affecting young individuals with sickle cell trait. Renal medullary carcinoma commonly presents with advanced or metastatic disease and is associated with a rapidly progressive clinical course and an extremely short overall survival measured in weeks to few months. Due to the rarity of RMC, there is no proven effective therapy and patients are often treated with platinum-based chemotherapy. We report near-complete radiological and pathological response in a patient treated with dose-dense MVAC (methotrexate, vinblastine, doxorubicin, and cisplatin) chemotherapy. The patient underwent consolidation nephrectomy and retroperitoneal lymph node dissection and had a 16-month progression-free survival, one of the longest reported in patients with RMC.Case reports in oncological medicine. 01/2014; 2014:615895.
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ABSTRACT: Renal cell carcinoma (RCC) management has been imbued with new interest, in large part due to the recent success of new treatment options for advanced and metastatic disease. This has also been accompanied by less generally well known advances in the understanding of the molecular characterizations of subtypes of RCC with potential to lead to new therapeutic options. Additionally, the urologic oncology community is focusing on nephron-sparing surgical approaches with limited surgery if possible, and in conjunction with interventional radiologists, on ablative procedures for incidentally determined small renal masses. This report reviews some of the new biologic findings of adenocarcinoma of the kidney, and reviews the new therapeutics which continue to change the landscape for treatment of RCC.Therapeutic Advances in Urology 12/2013; 5(6):338-353.