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.
"In this retrospective analysis, survival of patients with advanced RMC ranged from 4 days to 9 months . A number of other case reports and series have described the role of surgery, chemotherapy, immunotherapy, or radiation in the treatment of RMC and found rapid progression despite these therapies    . Rathmell and Monk initially reported the use of highdose intensity MVAC chemotherapy in a case-series of three patients with RMC . "
[Show abstract][Hide abstract] 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 08/2014; 2014:615895. DOI:10.1155/2014/615895
"Most patients present with the triad commonly seen in renal carcinoma, that is, gross hematuria, flank pain, and a palpable abdominal mass. Nearly 75% of the tumor masses are reported to be on the right side [3, 4]. Evidence of metastatic disease at the time of presentation is not uncommon. "
[Show abstract][Hide abstract] ABSTRACT: Renal medullary cancer is a rare malignancy almost exclusively seen in young patients of African ethnicity. These patients often present with the cardinal symptoms of hematuria, flank pain, and an abdominal mass, and this malignancy has been associated with patients carrying sickle cell trait. It is estimated that 300 million people worldwide carry sickle cell trait, and the presence of hematuria in these patients should be treated as a harbinger of a possible malignancy. Notably, this tumor mostly develops on the right side of the body. Patients often present with it at an advanced stage and the prognosis is poor. Therefore, a high index of suspicion in a patient of African descent presenting with a right sided abdominal mass and hematuria may assist in an early diagnosis. Current chemotherapy options are very limited, and early detection may provide a chance for surgical resection. It may also provide a bigger time frame for the initiation of novel chemotherapy regimens in patients who fail current chemotherapy regimens.
Case Reports in Oncological Medicine 10/2013; 2013:129813. DOI:10.1155/2013/129813
"It is highly aggressive and more common among young men between ten and forty years. There is strong association with sickle cell trait, described in 98% of the cases.1-3 In several texts, renal medullary carcinoma is classified as a subtype of collecting duct carcinoma. "
[Show abstract][Hide abstract] ABSTRACT: Renal medullary carcinoma (RMC) is rare, accounting for less than 1% of all renal neoplasms. Case reports suggest RMC is highly aggressive, poorly responsive to chemotherapy, often metastatic at diagnosis, affects young men with sickle cell trait, and median overall survival (mOS) is less than 12 months. We report the epidemiological characteristics, treatments performed, response rate to each treatment and mOS of five patients with RMC. All patients had sickle cell trait, four were male, three had metastatic disease at diagnosis and mean age at diagnosis was 25 years. Non-metastatic patients were submitted to nephrectomy. Two patients had partial response to first line chemotherapy including cisplatin and gemcitabine. There was no response to sunitinib or second line chemo - therapy; mOS was 6 months. Due to its rarity, case series are the only evidence available to discuss the treatment for RMC. In our experience, only cisplatin and gemcitabine based regimen offered response.
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.