Renal medullary carcinoma: the Bronx experience.

OBJECTIVES: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.