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SMARCB1-Deficient Kidney Medullary Carcinoma · CancerIndex

Cancers
Neoplasm
Neoplasm by Site
Genitourinary System Neoplasm
Urinary System Neoplasm
Malignant Urinary System Neoplasm
Malignant Kidney Neoplasm
Kidney Carcinoma
Kidney Medullary Carcinoma
SMARCB1-Deficient Kidney Medullary Carcinoma

Molecular Subtype

SMARCB1-Deficient Kidney Medullary Carcinoma

CI-CAN-00009146Explore in graph →

1 active trials1 evidence items

NCIt: C189247
DOID: 0070475

Overview
Statistics
Survival
Genomics
Variants & evidence1
Drugs
Trials1
Research
Rankings
Sources & provenance

Data completeness3 / 7 dimensions

Epidemiology (no data yet)
Survival (no data yet)
Trials (data present)
Literature (data present)
Genomics (no data yet)
Evidence (data present)
Therapies (no data yet)

Computed from entity_counters.completeness · refreshed after each connector run

Definition

What this entity is

A high-grade carcinoma that arises from the renal medulla and is characterized by inactivation of the SMARCB1 gene. It affects children and adults and occurs mainly in patients with sickle cell trait. The majority of the cases occur in the right kidney.

Definition text as published by ncit-evsProvenance

Source: NCI Thesaurus (NCIt)
Dataset: NCI Thesaurus
Version: 26.08e
Retrieved: Sep 8, 2026
Layer: raw (as published)
Evidence: expert curation
License: CC BY 4.0
Run: ING-NCITEVS-20260908-000001

Open at source Curated

Data updated 22 days agoSource updated 26.08e

Taxonomy

Position in the hierarchies

Several hierarchies coexist; edges are listed per hierarchy type with their source.

Parents

ncit

Kidney Carcinoma Molecular SubtypesSubtype
Kidney Medullary CarcinomaSubtype

Children (1)

ncit

Childhood SMARCB1-Deficient Kidney Medullary Carcinoma

Anatomy

Anatomical sites

Genitourinary SystemC156482

Record

Identity

CancerIndex ID: CI-CAN-00009146
Entity type: Molecular Subtype
Malignant: Yes
Solid / hematologic: Solid tumor
Pediatric relevant: Not flagged
Rare cancer: Unknown (no incidence data yet)
Hierarchy depth: 8
Classification version: 26.08e
Semantic types: Neoplastic Process
Status: Active
Record updated: 2026-09-08

Coverage

Data completeness

Trials (all / active): 1 / 1

Publications (all / 5y)

19 / 19

Evidence items

1

Genes / variants

1 / 1

Drugs (any / approved)

0 / 0

Genomic cohorts

0

Epidemiology / survival obs.

0 / 0

Data updated 19 hours agoSource updated unknowncounters aggregate over descendants

Names

Synonyms (1)

SMARCB1-Deficient Renal Medullary Carcinomasynonym · NCIt

CancerIndex is a research and information platform. It does not diagnose and does not recommend treatment.

Key figures

At a glance

Each figure carries its unit, period and source; ranks link to "Why this rank?".

US deaths15,215countUnited States · 2024 · both sexes · all agescdc-wonderObservedrank #12 of 31 (USA · both sexes · all ages · 2024 · top-level cancers)figures shown for Malignant Kidney Neoplasm (registry level) — open Malignant Kidney Neoplasm
US new cases77,334countUnited States · 2023 · both sexes · all agescdc-uscsObservedrank #7 of 32 (USA · both sexes · all ages · 2023 · top-level cancers)figures shown for Malignant Kidney Neoplasm (registry level) — open Malignant Kidney Neoplasm
US age-standardized mortality3.4per 100,000United States · 2024 · both sexes · all ages · 2000 U.S. Std. Populationcdc-wonderObservedrank #11 of 23 (USA · both sexes · all ages · 2024 · top-level cancers)figures shown for Malignant Kidney Neoplasm (registry level) — open Malignant Kidney Neoplasm
Active trials1countcurrent registry status · entity + descendantsclinicaltrialsComputedrank #1186 of 1,654 (World · both sexes · all ages · latest · all malignant entities)
Recruiting trials1countstatus RECRUITING · entity + descendantsclinicaltrialsComputedrank #887 of 1,277 (World · both sexes · all ages · latest · all malignant entities)
Curated evidence items1countaccepted CIViC items · entity + descendantscivicComputedrank #392 of 492 (World · both sexes · all ages · latest · all malignant entities)
Publications, last 12 months8countPubMed records · stored query · 12-month windowpubmedComputedrank #1082 of 2,294 (World · both sexes · all ages · latest · all malignant entities)
Genomic cohorts0countopen GDC projects · entity + descendantsgdcComputednot in a current ranking snapshot

Data updated 19 hours agoSource updated unknownregistry figures: cdc-uscs · latest year available per metric · counters aggregate over descendants