Skip to main content
SpringerLink
Log in

Sclerosing Rhabdomyosarcoma in Childhood: Case Report and Review of the Literature

  • Case report
  • Published:
Pediatric and Developmental Pathology

Abstract

Rhabdomyosarcoma is the most common soft tissue malignancy in children but is rare in adults. The latest World Health Organization classification of soft tissue tumors recognizes embryonal, alveolar, and pleomorphic rhabdomyosarcomas. More recently, a sclerosing variant of rhabdomyosarcoma has been recognized and reported in seven adult patients. We describe a pediatric case of sclerosing rhabdomyosarcoma presenting as a sacral mass in a 3-year-old girl. Morphologically, the tumor showed a prominent sclerosing hyaline matrix and demonstrated pseudovascular and microalveolar architectural foci. Focal positivity was seen with desmin, smooth muscle actin, and myogenin. MyoD1 showed uniform diffuse nuclear staining. Fusion transcripts were not demonstrated by reverse transcriptase-polymerase chain reaction analysis. The histology, immunohistochemistry, and molecular genetics matched those reported in the seven adult cases of sclerosing rhabdomyosarcoma. This is the first case report, to our knowledge, of this rare tumor arising in the pediatric age group, and we compare the features with those reported in adult sclerosing rhabdomyosarcoma.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Figure 1.
Figure 2.

Similar content being viewed by others

References

  1. DM Parham (2001) ArticleTitlePathologic classification of rhabdomyosarcomas and correlations with molecular studies Mod Pathol 14 506–514 Occurrence Handle10.1038/modpathol.3880339 Occurrence Handle1:STN:280:DC%2BD3M3lsV2isg%3D%3D Occurrence Handle11353062

    Article  CAS  PubMed  Google Scholar 

  2. T Mentzel D Katenkamp (2000) ArticleTitleSclerosing, pseudovascular rhabdomyosarcoma in adults; clinicopathological and immunohistochemical analysis of three cases Virchows Arch 436 305–311 Occurrence Handle10.1007/s004280050451 Occurrence Handle1:STN:280:DC%2BD3c3pt1ersQ%3D%3D Occurrence Handle10834531

    Article  CAS  PubMed  Google Scholar 

  3. AL Folpe JK McKenney JA Bridge SW Weiss (2002) ArticleTitleSclerosing rhabdomyosarcoma in adults: report of four cases of a hyalinizing, matrix-rich variant of rhabdomyosarcoma that may be confused with osteosarcoma, chondrosarcoma, or angiosarcoma Am J Surg Pathol 26 1175–1183 Occurrence Handle10.1097/00000478-200209000-00008 Occurrence Handle12218574

    Article  PubMed  Google Scholar 

  4. HP McDowell (2003) ArticleTitleUpdate on childhood rhabdomyosarcoma Arch Dis Child 88 354–357 Occurrence Handle10.1136/adc.88.4.354 Occurrence Handle1:STN:280:DC%2BD3s7js1Cktg%3D%3D Occurrence Handle12651771

    Article  CAS  PubMed  Google Scholar 

  5. SJ Qualman CM Coffin WA Newton et al. (1998) ArticleTitleIntergroup Rhabdomyosarcoma Study: update for pathologists Pediatr Dev Pathol 1 550–561 Occurrence Handle10.1007/s100249900076 Occurrence Handle1:STN:280:DyaK1MzjsFKmsA%3D%3D Occurrence Handle9724344

    Article  CAS  PubMed  Google Scholar 

  6. WA Newton SuffixJr EA Gehan BL Webber et al. (1995) ArticleTitleClassification of rhabdomyosarcoma and related sarcomas: pathologic aspect and proposal for a new classification—an Intergroup Rhabdomyosarcoma Study Cancer 76 1073–1085 Occurrence Handle8625211

    PubMed  Google Scholar 

  7. Chiles M, Parham D, Qualman S, et al. Pediatric matrix-rich rhabdomyosarcomas (Abstract). Pediatr Dev Pathol 2002;5:606.

    Google Scholar 

  8. D Boué D Parham L Teot WM Crist SJ Qualman (2000) ArticleTitleClinicopathologic study of pediatric ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Group III and IV Pediatr Dev Pathol 3 290–300 Occurrence Handle10.1007/s100249910039 Occurrence Handle10742419

    Article  PubMed  Google Scholar 

  9. NJ Sebire M Malone (2003) ArticleTitleMyogenin and MyoD1 expression in paediatric rhabdomyosarcomas J Clin Pathol 56 412–416 Occurrence Handle10.1136/jcp.56.6.412 Occurrence Handle1:STN:280:DC%2BD3s3lvVKrug%3D%3D Occurrence Handle12783965

    Article  CAS  PubMed  Google Scholar 

  10. PHB Sorensen JC Lynch SJ Qualman et al. (2002) ArticleTitlePAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the Children’s Oncology Group J Clin Oncol 20 2672–2679 Occurrence Handle10.1200/JCO.2002.03.137 Occurrence Handle1:CAS:528:DC%2BD38XkvF2mtr0%3D Occurrence Handle12039929

    Article  CAS  PubMed  Google Scholar 

  11. DM Parham FG Barr (2002) Embryonal rhabdomyosarcoma CDM Fletcher KK Unni F Mertens (Eds) World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone IARC Press Lyon, France 146–149

    Google Scholar 

  12. P Dias B Chen B Dilday et al. (2000) ArticleTitleStrong immuostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass Am J Surg Pathol 156 399–408 Occurrence Handle1:STN:280:DC%2BD3c7jt1yjuw%3D%3D

    CAS  Google Scholar 

  13. S Cui H Hano T Harada S Takai F Masui S Ushigome (1999) ArticleTitleEvaluation of the new monoclonal anti-MyoD1 and anti-myogenin antibodies for the diagnosis of rhabdomyosarcoma Pathol Int 49 62–68 Occurrence Handle10.1046/j.1440-1827.1999.00825.x Occurrence Handle1:CAS:528:DyaK1MXktlKgsLs%3D Occurrence Handle10227726

    Article  CAS  PubMed  Google Scholar 

  14. S Williams NJ Sebire A Ramsay (2002) ArticleTitleImmunohistochemical expression of myogenin and MyoD1 in embryonal, classic alveolar and solid-variant paediatric alveolar rhabdomyosarcoma (Abstract) J Pathol 198 25A

    Google Scholar 

  15. FG Barr SJ Qualman MH Macris et al. (2002) ArticleTitleGenetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions Cancer Res 62 4704–4710 Occurrence Handle1:CAS:528:DC%2BD38XmsVShsLc%3D Occurrence Handle12183429

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Histopathology, Camelia Botnar Laboratories, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK

    Bhumita Vadgama, Neil James Sebire, Marian Malone & Alan Drummond Ramsay

Authors
  1. Bhumita Vadgama
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Neil James Sebire
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Marian Malone
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Alan Drummond Ramsay
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Alan Drummond Ramsay.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Vadgama, B., Sebire, N., Malone, M. et al. Sclerosing Rhabdomyosarcoma in Childhood: Case Report and Review of the Literature. Pediatr. Dev. Pathol. 7, 391–396 (2004). https://doi.org/10.1007/s10024-003-9453-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10024-003-9453-5

Keywords

Search

Navigation