Rhabdomyosarcoma classification: Difference between revisions

Jump to navigation Jump to search
Line 11: Line 11:
**Alveolar rhabdomyosarcoma (ARMS)
**Alveolar rhabdomyosarcoma (ARMS)
**Botryoid and spindle cell (leiomyomatous) RMS
**Botryoid and spindle cell (leiomyomatous) RMS
**Sclerosing and spindle cell RMS  
**Sclerosing and spindle cell RMS with MyoD1 mutation
*Separate categories for other subtypes which do not classify into above groups:
*Separate categories for other subtypes which do not classify into above groups:
**Undifferentiated
**Undifferentiated
Line 39: Line 39:
* Mostly occur in the trunk and extremities
* Mostly occur in the trunk and extremities
|-
|-
|Botryoid and spindle cell
|Botryoid and spindle cell RMS
|
|
* Botryoid RMS contain 6% of all RMS
* Spindle cell RMS contains 3% of all RMS
* Less common varients of ERMS
* Accompanied with more favorable prognosis
* Botryoid name related to grape-like appearance of tumor
* Botryoid RMS occur within bladder wall and vagina
* Spindle cell is prevalent among infants and neonates under age of 1 years old
* Spindle cell RMS occurs in paratesticular location
|-
|Sclerosing and spindle cell RMS
|
* Less common variants of ERMS
* Poor outcome
*
|}
|}



Revision as of 19:04, 11 January 2019

Rhabdomyosarcoma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Rhabdomyosarcoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

Staging

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Rhabdomyosarcoma classification On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Rhabdomyosarcoma classification

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Rhabdomyosarcoma classification

CDC on Rhabdomyosarcoma classification

Rhabdomyosarcoma classification in the news

Blogs on Rhabdomyosarcoma classification

Directions to Hospitals Treating Rhabdomyosarcoma

Risk calculators and risk factors for Rhabdomyosarcoma classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Shadan Mehraban, M.D.[2]

Overview

Classification

Histologic classification of rhabdomyosarcoma

  • The international classification of rhabdomyosarcoma (RMS) was created by the Intergroup Rhabdomyosarcoma Study group (IRSG) therapeutic trials (IRS-I to IV).[1][2]
  • The four main subgroups are:
    • Embryonal rhabdomyosarcoma (ERMS)
    • Alveolar rhabdomyosarcoma (ARMS)
    • Botryoid and spindle cell (leiomyomatous) RMS
    • Sclerosing and spindle cell RMS with MyoD1 mutation
  • Separate categories for other subtypes which do not classify into above groups:
    • Undifferentiated
    • Pleomorphic/anaplastic
    • Sarcoma
RMS subtypes Definition
Embryonal RMS (ERMS)
  • The most common group
  • Contains 59% of all RMS
  • Intermediate prognosis
  • Mostly occurs in head, neck, and genitourinary system[3]
  • Larger peak between age of 0-5 years and smaller peak among adolescents
  • Specific translocation is not yet designated[4]
  • Loss of heterozygosity (LOH) at the 11p15 locus, the site of the IGF-2 gene
Alveolar RMS (ARMS)
  • Determined by FOXO1 rearrangement, t (1;13) or t (2;13)[5]
  • Poorer prognosis
  • Contains 21% of all RMS
  • More likely among adolescents
  • Mostly occur in the trunk and extremities
Botryoid and spindle cell RMS
  • Botryoid RMS contain 6% of all RMS
  • Spindle cell RMS contains 3% of all RMS
  • Less common varients of ERMS
  • Accompanied with more favorable prognosis
  • Botryoid name related to grape-like appearance of tumor
  • Botryoid RMS occur within bladder wall and vagina
  • Spindle cell is prevalent among infants and neonates under age of 1 years old
  • Spindle cell RMS occurs in paratesticular location
Sclerosing and spindle cell RMS
  • Less common variants of ERMS
  • Poor outcome

References

  1. Qualman SJ, Coffin CM, Newton WA, Hojo H, Triche TJ, Parham DM; et al. (1998). "Intergroup Rhabdomyosarcoma Study: update for pathologists". Pediatr Dev Pathol. 1 (6): 550–61. PMID 9724344.
  2. Hicks J, Flaitz C (2002). "Rhabdomyosarcoma of the head and neck in children". Oral Oncol. 38 (5): 450–9. PMID 12110339.
  3. De Giovanni C, Landuzzi L, Nicoletti G, Lollini PL, Nanni P (2009). "Molecular and cellular biology of rhabdomyosarcoma". Future Oncol. 5 (9): 1449–75. doi:10.2217/fon.09.97. PMID 19903072.
  4. Scrable HJ, Witte DP, Lampkin BC, Cavenee WK (1987). "Chromosomal localization of the human rhabdomyosarcoma locus by mitotic recombination mapping". Nature. 329 (6140): 645–7. doi:10.1038/329645a0. PMID 3657988.
  5. Helman LJ, Meltzer P (2003). "Mechanisms of sarcoma development". Nat Rev Cancer. 3 (9): 685–94. doi:10.1038/nrc1168. PMID 12951587.

Template:WH Template:WS