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CANCER:

Kidney Medullary Carcinoma

Related cancers:
7d
Compassionate Use of Ubamatamab (clinicaltrials.gov)
P=N/A, N=0, Available, Regeneron Pharmaceuticals
New trial
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ubamatamab (REGN4018)
13d
Paediatric kidney tumours: Update on the challenges of nephroblastoma and other kidney tumours (PubMed, Ann Pathol)
In this group, each tumour has its own genetics, whose molecular mechanisms are increasingly well understood, with fusions, tandem duplications or gene mutations which can help the pathologist to achieve to an accurate diagnosis in each morphological context. In some situations, the identification of these molecular alterations may lead to a targeted treatment.
Journal
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TP53 (Tumor protein P53) • MYCN (MYCN Proto-Oncogene BHLH Transcription Factor) • CTNNB1 (Catenin (cadherin-associated protein), beta 1) • FBXW7 (F-Box And WD Repeat Domain Containing 7) • WT1 (WT1 Transcription Factor) • TRIM28 (Tripartite Motif Containing 28)
13d
Phase II Trial of Sacituzumab Tirumotecan in Patients With SMARCB1-Deficient Renal Medullary Carcinoma (clinicaltrials.gov)
P2, N=20, Not yet recruiting, M.D. Anderson Cancer Center | Trial primary completion date: Feb 2028 --> Jun 2028
Trial primary completion date
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SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1)
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Jiataile (sacituzumab tirumotecan)
14d
Unclear territory: navigating metastatic nonclear cell renal cell carcinoma. (PubMed, Curr Opin Oncol)
Therapeutic advances are reshaping the management of nccRCC, with IO/TKI regimens and histology-specific therapies showing promise. Continued integration of molecular classification, rare subtype-specific trials, and international collaboration will be essential to establish evidence-based treatment standards for this diverse and understudied population.
Journal
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ALK (Anaplastic lymphoma kinase) • FH (Fumarate Hydratase)
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ALK rearrangement
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Keytruda (pembrolizumab) • Opdivo (nivolumab) • Avastin (bevacizumab) • erlotinib • Yervoy (ipilimumab) • Tyvyt (sintilimab) • Lenvima (lenvatinib) • axitinib
1m
New P2 trial
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SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1)
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Jiataile (sacituzumab tirumotecan)
2ms
Testing the Effectiveness of Two Immunotherapy Drugs (Nivolumab and Ipilimumab) With One Anti-cancer Targeted Drug (Cabozantinib) for Rare Genitourinary Tumors (clinicaltrials.gov)
P2, N=314, Recruiting, National Cancer Institute (NCI) | Trial completion date: Feb 2026 --> Feb 2027 | Trial primary completion date: Feb 2026 --> Feb 2027
Trial completion date • Trial primary completion date
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PD-L1 (Programmed death ligand 1) • PD-1 (Programmed cell death 1) • CTLA4 (Cytotoxic T-Lymphocyte Associated Protein 4) • CD4 (CD4 Molecule)
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Opdivo (nivolumab) • Yervoy (ipilimumab) • Cabometyx (cabozantinib tablet) • Cometriq (cabozantinib capsule) • ABP 206 (nivolumab biosimilar)
2ms
Clinicopathological features of SMARCB1/INI1-deficient medullary-like renal cell carcinoma: Report of 3 cases and literature review. (PubMed, Ann Diagn Pathol)
Given its highly aggressive nature, early and accurate diagnosis is crucial. Pathologists must integrate clinical history, morphological characteristics, immunophenotype for a comprehensive diagnosis.
Journal
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SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1) • VIM (Vimentin)
2ms
Pembrolizumab in Treating Patients With Rare Tumors That Cannot Be Removed by Surgery or Are Metastatic (clinicaltrials.gov)
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
Trial completion date • Trial primary completion date • Tumor mutational burden
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PD-L1 (Programmed death ligand 1)
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Keytruda (pembrolizumab)
3ms
SMARCB1-Deficient Renal Medullary Carcinoma: A Case Report Highlighting the Value of History and Race Information. (PubMed, Cureus)
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.
Journal
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ALK (Anaplastic lymphoma kinase) • CCND1 (Cyclin D1) • SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1) • VIM (Vimentin) • MME (Membrane Metalloendopeptidase) • GATA3 (GATA binding protein 3) • PAX8 (Paired box 8)
4ms
Enrollment closed
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Cabometyx (cabozantinib tablet) • Cometriq (cabozantinib capsule) • Xofigo (radium Ra-223 dichloride)
4ms
Molecularly defined renal cell carcinomas: practical approaches for surgical pathologists. (PubMed, Histopathology)
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.
Review • Journal
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ALK (Anaplastic lymphoma kinase) • SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1) • FH (Fumarate Hydratase) • TFE3 (Transcription Factor Binding To IGHM Enhancer 3) • TFEB (Transcription Factor EB 2)
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ALK rearrangement • ALK mutation
4ms
Phase II Trial of Nivolumab Plus Ipilimumab in Patients With Renal Medullary Carcinoma (clinicaltrials.gov)
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.
Trial completion date • Trial termination • Trial primary completion date
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SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1)
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Opdivo (nivolumab) • Yervoy (ipilimumab)