Kidney Medullary Carcinoma

P2, N=20, Not yet recruiting, M.D. Anderson Cancer Center

P2, N=314, Recruiting, National Cancer Institute (NCI) | Trial completion date: Feb 2026 --> Feb 2027 | Trial primary completion date: Feb 2026 --> Feb 2027

Given its highly aggressive nature, early and accurate diagnosis is crucial. Pathologists must integrate clinical history, morphological characteristics, immunophenotype for a comprehensive diagnosis.

P2, N=157, Active, not recruiting, M.D. Anderson Cancer Center | Trial completion date: Dec 2025 --> Dec 2028 | Trial primary completion date: Dec 2025 --> Dec 2028

RMC should be considered in the differential diagnosis of renal tumors in young patients. The patient was managed by the medical oncology team and was treated with chemotherapy and radiation but subsequently developed multiple metastases and passed away three years after the initial disease diagnosis.

P2, N=134, Active, not recruiting, National Cancer Institute (NCI) | Recruiting --> Active, not recruiting

Although they account for only about 5% of RCC, they are clinically significant due to distinctive biology, frequent diagnostic pitfalls, and therapeutic implications. Many pathology laboratories lack immediate access to fluorescence in situ hybridization (FISH) or next-generation sequencing (NGS) to confirm MDRC; this review emphasizes morphologic recognition and immunohistochemical surrogates, followed by rational triage for ancillary testing when available.

P2, N=10, Terminated, M.D. Anderson Cancer Center | Trial completion date: Jul 2027 --> Nov 2025 | Active, not recruiting --> Terminated | Trial primary completion date: Jul 2027 --> Nov 2025; The study met its prespecified futility criteria during the prespecified interim futility analysis after enrolling the first cohort of 10 patients.

Selective pharmacologic inhibition of p300 suppressed this program and restored sensitivity to ICT. These findings reveal an adaptive mechanism of resistance to ICT in RMC and support targeting master myeloid regulators to enable therapeutic benefit.

P1/2, N=58, Completed, Epizyme, Inc. | Active, not recruiting --> Completed

This study represents the most extensive molecular characterization of RMC to date, identifying TROP2 and other potential therapeutic targets. Sacituzumab govitecan demonstrates potential clinical benefit, warranting further evaluation in prospective trials to confirm its efficacy and explore additional targets identified herein.

Given the exceptional rarity of RCCU-MP and its overlapping characteristics with RMC, we systematically analyze the clinical features, pathological morphology, and immunohistochemistry of RCCU-MP separately. Additionally, we explore potential molecular pathogenesis and emerging therapeutic strategies based on recent evidence.