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Related Concept Videos

The Retinoblastoma Gene01:20

The Retinoblastoma Gene

Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
The Retinoblastoma Gene01:20

The Retinoblastoma Gene

Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
Rous Sarcoma Virus (RSV) and Cancer01:03

Rous Sarcoma Virus (RSV) and Cancer

Rous Sarcoma virus or RSV was discovered by F. Peyton Rous in the year 1911 as a filterable transmissible agent that could cause tumors in chickens. He won a Nobel Prize for this discovery in 1966. His experiments clearly demonstrated that some cancers could be caused by infectious agents and led to the discovery of many more cancer-causing viruses in animals as well as humans.
RSV is a retrovirus that contains two copies of a plus-strand  RNA genome. Its genome consists of four main open...
Rous Sarcoma Virus (RSV) and Cancer01:03

Rous Sarcoma Virus (RSV) and Cancer

Rous Sarcoma virus or RSV was discovered by F. Peyton Rous in the year 1911 as a filterable transmissible agent that could cause tumors in chickens. He won a Nobel Prize for this discovery in 1966. His experiments clearly demonstrated that some cancers could be caused by infectious agents and led to the discovery of many more cancer-causing viruses in animals as well as humans.
RSV is a retrovirus that contains two copies of a plus-strand  RNA genome. Its genome consists of four main open...
The Ras Gene02:38

The Ras Gene

The Ras-gene-encoded proteins are regulators of signaling pathways controlling cell proliferation, differentiation, or cell survival. The Ras-gene family in humans constitutes three primary members—the HRas, NRas, and KRas. These genes code for four functionally distinct yet closely related proteins—the HRas, NRas, KRas4A, and KRas4B. The involvement of mutant Ras genes in human cancer was first discovered in 1982 and is among the most common causes of human tumorigenesis.
Ras is a superfamily...
Abnormal Proliferation02:23

Abnormal Proliferation

Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the daughter...

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Related Experiment Video

Updated: May 19, 2026

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
09:21

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas

Published on: September 13, 2019

[Rhabdoid tumours].

P Vasovčák1, A Puchmajerová, A Křepelová

  • 1Ústav biologie a lékařské genetiky, Fakultní nemocnice v Motole, Praha. peter.vasovcak@fnmotol.cz

Klinicka Onkologie : Casopis Ceske a Slovenske Onkologicke Spolecnosti
|August 28, 2012
PubMed
Summary

Rhabdoid tumors (RTs) are rare, aggressive childhood cancers. Molecular genetic analysis, particularly of the SMARCB1 gene, is crucial for diagnosing RTs, including rhabdoid predisposition syndrome.

Area of Science:

  • Oncology
  • Genetics
  • Pediatric Pathology

Background:

  • Rhabdoid tumors (RTs) are rare, highly malignant embryonic tumors predominantly affecting infants (0-2 years).
  • Commonly found in the brain and kidney, RTs can also arise from soft tissues throughout the body.
  • Histologically, RTs consist of rhabdoid cells, sometimes mixed with other cell types, forming atypical teratoid/rhabdoid tumors (AT/RTs).

Purpose of the Study:

  • To highlight the diagnostic challenges posed by the rarity and non-specific histology of RTs.
  • To emphasize the critical role of molecular genetic studies in confirming RT diagnosis.
  • To discuss the significance of SMARCB1 gene mutations in RT pathogenesis and hereditary predisposition.

Main Methods:

  • Review of histological and molecular genetic findings in RT cases.

Related Experiment Videos

Last Updated: May 19, 2026

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
09:21

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas

Published on: September 13, 2019

  • Focus on the diagnostic utility of SMARCB1 gene analysis.
  • Discussion of germline mutations and their link to rhabdoid predisposition syndrome.
  • Main Results:

    • Biallelic inactivation of the SMARCB1 gene is implicated in the majority of RT cases.
    • Approximately one-third of SMARCB1 mutations are germline, leading to rhabdoid predisposition syndrome.
    • Molecular genetic analysis aids in accurate diagnosis, genetic counseling, and epidemiological studies.

    Conclusions:

    • Molecular genetic analysis, especially of the SMARCB1 gene, is essential for accurate RT diagnosis.
    • Identifying SMARCB1 mutations is vital for understanding RT pathogenesis and hereditary risk.
    • Genetic insights facilitate improved patient management, counseling, and research into these rare tumors.