Dense Pattern of Embryonal Rhabdomyosarcoma, a Lesion Easily Confused with Alveolar Rhabdomyosarcoma: a Report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group

Overview

Journal Am J Clin Pathol

Publisher Oxford University Press

Specialty Pathology

Date 2013 Jun 15

PMID 23765537

Citations 39

Authors

Erin R Rudzinski

Lisa A Teot

James R Anderson

Julie Moore

Julia A Bridge

Frederic G Barr

Julie M Gastier-Foster

Stephen X Skapek

Douglas S Hawkins

David M Parham

Affiliations

Soon will be listed here.

Abstract

Objectives: To examine whether the frequency of fusion-negative alveolar rhabdomyosarcoma (ARMSn) increased coincident with changes in the definition of alveolar histology.

Methods: We re-reviewed alveolar rhabdomyosarcoma (ARMS) in the Children's Oncology Group study D9803, comparing histopathology with fusion status.

Results: Our review of 255 original ARMS cases (compared with a control group of 38 embryonal rhabdomyosarcomas [ERMS] cases) revealed that many had an ARMS-like densely cellular pattern with cytologic features and myogenin expression more typical of ERMS. Following re-review, 84 (33%) cases of original ARMS were rediagnosed as ERMS. All reclassified ERMS, including dense ERMS, were fusion negative, whereas 82% of confirmed ARMS cases were fusion positive. Total ARMS diagnoses returned to historic rates of 25% to 30% of all rhabdomyosarcomas, and ARMSn decreased from 37% to 18% of ARMS cases. The outcome of reclassified ERMS was similar to confirmed ERMS.

Conclusions: To address the role of fusion status in risk stratification, pathologists should include both a histologic diagnosis and an evaluation of fusion status for all new ARMS diagnoses.

Citing Articles

Rhabdomyosarcoma in children and young adults.

Chen S, Kelsey A, Rudzinski E Virchows Arch. 2024; 486(1):101-116.

PMID: 39694930 DOI: 10.1007/s00428-024-03961-y.

Addition of temsirolimus to chemotherapy in children, adolescents, and young adults with intermediate-risk rhabdomyosarcoma (ARST1431): a randomised, open-label, phase 3 trial from the Children's Oncology Group.

Gupta A, Xue W, Harrison D, Hawkins D, Dasgupta R, Wolden S Lancet Oncol. 2024; 25(7):912-921.

PMID: 38936378 PMC: 11550893. DOI: 10.1016/S1470-2045(24)00255-9.

Biological and therapeutic insights from animal modeling of fusion-driven pediatric soft tissue sarcomas.

Kucinski J, Calderon D, Kendall G Dis Model Mech. 2024; 17(6).

PMID: 38916046 PMC: 11225592. DOI: 10.1242/dmm.050704.

Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies.

Zarrabi A, Perrin D, Kavoosi M, Sommer M, Sezen S, Mehrbod P Cancers (Basel). 2023; 15(21).

PMID: 37958442 PMC: 10650215. DOI: 10.3390/cancers15215269.

Retroperitoneal alveolar rhabdomyosarcoma intruding into spinal canal: A case report and literature review.

Zhang Y, Huang W, Li L, Qiu Y, Jiao H, Chen Z Front Med (Lausanne). 2022; 9:1019964.

PMID: 36405590 PMC: 9670135. DOI: 10.3389/fmed.2022.1019964.

References

Hostein I, Andraud-Fregeville M, Guillou L, Terrier-Lacombe M, Deminiere C, Ranchere D . Rhabdomyosarcoma: value of myogenin expression analysis and molecular testing in diagnosing the alveolar subtype: an analysis of 109 paraffin-embedded specimens. Cancer. 2004; 101(12):2817-24. DOI: 10.1002/cncr.20711. View

Tsokos M, Webber B, Parham D, Wesley R, Miser A, Miser J . Rhabdomyosarcoma. A new classification scheme related to prognosis. Arch Pathol Lab Med. 1992; 116(8):847-55. View

Morotti R, Nicol K, Parham D, Teot L, Moore J, Hayes J . An immunohistochemical algorithm to facilitate diagnosis and subtyping of rhabdomyosarcoma: the Children's Oncology Group experience. Am J Surg Pathol. 2006; 30(8):962-8. DOI: 10.1097/00000478-200608000-00005. View

NEWTON Jr W, Gehan E, Webber B, Marsden H, van Unnik A, Hamoudi A . Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification--an Intergroup Rhabdomyosarcoma Study. Cancer. 1995; 76(6):1073-85. DOI: 10.1002/1097-0142(19950915)76:6<1073::aid-cncr2820760624>3.0.co;2-l. View

Dias P, Chen B, Dilday B, Palmer H, Hosoi H, Singh S . Strong immunostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass. Am J Pathol. 2000; 156(2):399-408. PMC: 1850049. DOI: 10.1016/S0002-9440(10)64743-8. View

Folpe A, McKenney J, Bridge J, Weiss S . Sclerosing 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. 2002; 26(9):1175-83. DOI: 10.1097/00000478-200209000-00008. View

Sorensen P, Lynch J, Qualman S, Tirabosco R, Lim J, Maurer H . PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group. J Clin Oncol. 2002; 20(11):2672-9. DOI: 10.1200/JCO.2002.03.137. View

Williamson D, Missiaglia E, De Reynies A, Pierron G, Thuille B, Palenzuela G . Fusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcoma. J Clin Oncol. 2010; 28(13):2151-8. DOI: 10.1200/JCO.2009.26.3814. View

Barr F, Smith L, Lynch J, Strzelecki D, Parham D, Qualman S . Examination of gene fusion status in archival samples of alveolar rhabdomyosarcoma entered on the Intergroup Rhabdomyosarcoma Study-III trial: a report from the Children's Oncology Group. J Mol Diagn. 2006; 8(2):202-8. PMC: 1867584. DOI: 10.2353/jmoldx.2006.050124. View

10.

Jo V, Marino-Enriquez A, Fletcher C . Epithelioid rhabdomyosarcoma: clinicopathologic analysis of 16 cases of a morphologically distinct variant of rhabdomyosarcoma. Am J Surg Pathol. 2011; 35(10):1523-30. DOI: 10.1097/PAS.0b013e31822e0907. View

11.

Nishio J, Althof P, Bailey J, Zhou M, Neff J, Barr F . Use of a novel FISH assay on paraffin-embedded tissues as an adjunct to diagnosis of alveolar rhabdomyosarcoma. Lab Invest. 2006; 86(6):547-56. DOI: 10.1038/labinvest.3700416. View

12.

Parham D, Qualman S, Teot L, Barr F, Morotti R, Sorensen P . Correlation between histology and PAX/FKHR fusion status in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group. Am J Surg Pathol. 2007; 31(6):895-901. DOI: 10.1097/01.pas.0000213436.99492.51. View

13.

Chiles M, Parham D, Qualman S, Teot L, Bridge J, Ullrich F . Sclerosing rhabdomyosarcomas in children and adolescents: a clinicopathologic review of 13 cases from the Intergroup Rhabdomyosarcoma Study Group and Children's Oncology Group. Pediatr Dev Pathol. 2005; 7(6):583-94. DOI: 10.1007/s10024-004-5058-x. View

14.

Skapek S, Anderson J, Barr F, Bridge J, Gastier-Foster J, Parham D . PAX-FOXO1 fusion status drives unfavorable outcome for children with rhabdomyosarcoma: a children's oncology group report. Pediatr Blood Cancer. 2013; 60(9):1411-7. PMC: 4646073. DOI: 10.1002/pbc.24532. View

15.

Xia S, Pressey J, Barr F . Molecular pathogenesis of rhabdomyosarcoma. Cancer Biol Ther. 2002; 1(2):97-104. DOI: 10.4161/cbt.51. View

16.

RIOPELLE J, Theriault J . [An unknown type of soft part sarcoma: alveolar rhabdomyosarcoma]. Ann Anat Pathol (Paris). 1956; 1(1):88-111. View

17.

Arndt C, Stoner J, Hawkins D, Rodeberg D, Hayes-Jordan A, Paidas C . Vincristine, actinomycin, and cyclophosphamide compared with vincristine, actinomycin, and cyclophosphamide alternating with vincristine, topotecan, and cyclophosphamide for intermediate-risk rhabdomyosarcoma: children's oncology group study D9803. J Clin Oncol. 2009; 27(31):5182-8. PMC: 2773476. DOI: 10.1200/JCO.2009.22.3768. View

18.

Barr F, Qualman S, Macris M, Melnyk N, Lawlor E, Strzelecki D . Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions. Cancer Res. 2002; 62(16):4704-10. View

19.

Meza J, Anderson J, Pappo A, Meyer W . Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: the Children's Oncology Group. J Clin Oncol. 2006; 24(24):3844-51. DOI: 10.1200/JCO.2005.05.3801. View

20.

Davicioni E, Anderson M, Graf Finckenstein F, Lynch J, Qualman S, Shimada H . Molecular classification of rhabdomyosarcoma--genotypic and phenotypic determinants of diagnosis: a report from the Children's Oncology Group. Am J Pathol. 2009; 174(2):550-64. PMC: 2630563. DOI: 10.2353/ajpath.2009.080631. View