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Renal Medullary Carcinoma: Establishing Standards in Practice
#MMPMID28697319
Beckermann KE
; Sharma D
; Chaturvedi S
; Msaouel P
; Abboud MR
; Allory Y
; Bourdeaut F
; Calderaro J
; de Cubas AA
; Derebail VK
; Hong AL
; Naik RP
; Malouf GG
; Mullen EA
; Reuter VE
; Roberts CWM
; Walker CL
; Wood CG
; DeBaun MR
; Van Poppel H
; Tannir NM
; Rathmell WK
J Oncol Pract
2017[Jul]; 13
(7
): 414-421
PMID28697319
show ga
Although renal medullary carcinoma (RMC) is a rare subtype of kidney cancer, it
is particularly devastating in that it is nearly uniformly lethal. No established
guidelines exist for the diagnosis and management of RMC. In April 2016, a panel
of experts developed clinical guidelines on the basis of a literature review and
consensus statements. The goal was to propose recommendations for standardized
diagnostic and management approaches and to establish an international clinical
registry and biorepository for RMC. Published data are limited to case reports
and small retrospective reviews. The RMC Working Group prepared recommendations
to inform providers and patients faced with a low level of medical evidence. The
diagnosis of RMC should be considered in all patients younger than 50 years with
poorly differentiated carcinoma that arises from the renal medulla. These
patients should be tested for sickle cell hemoglobinopathies, and if positive,
SMARCB1/INI1 loss should be confirmed by immunohistochemistry. The majority of
patients with RMC are diagnosed with metastatic disease. Upfront radical
nephrectomy should be considered in patients with good performance status and low
metastatic burden or after response to systemic therapy. Currently, cytotoxic,
platinum-based chemotherapy provides the best, albeit brief, palliative clinical
benefit. Vascular endothelial growth factor-directed therapies and mammalian
target of rapamycin inhibitors are ineffective in RMC as monotherapy. Therapeutic
trials of novel agents are now available for RMC, and every effort should be made
to enroll patients in clinical studies.
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