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Virchows Archiv

, Volume 472, Issue 3, pp 441–449 | Cite as

Paratesticular alveolar rhabdomyosarcomas do not harbor typical translocations: a distinct entity with favorable prognosis?

  • Tobias M. Dantonello
  • Christian Vokuhl
  • Monika Scheer
  • Monika Sparber-Sauer
  • Sabine Stegmaier
  • Guido Seitz
  • Heike Scheithauer
  • Jörg Faber
  • Iris Veit-Friedrich
  • Peter Kaatsch
  • Stefan S. Bielack
  • Thomas Klingebiel
  • Ewa Koscielniak
  • on behalf of the Cooperative Weichteilsarkom Studiengruppe (CWS)
Original Article

Abstract

The alveolar subtype of rhabdomyosarcoma (RMA) is a strong risk factor. Cases of RMA located in paratesticular sites have however been reported to have similar outcomes to those of embryonal rhabdomyosarcoma (RME). We wanted to re-evaluate the impact of subtype in paratesticular rhabdomyosarcoma (PT-RMS). Patients from a population-based cohort diagnosed with paratesticular RMA in 1990–2013 were analyzed. All tumor samples were re-reviewed using conventional morphology, immunohistochemistry, and molecular testing. Seven patients were eligible. Four tumors showed focal areas morphologically compatible with RMA (mixed RMA/RME). One case was undifferentiated, with a solid round-cell morphology which had to be reclassified as poorly differentiated RME. Two cases had a “microalveolar” morphology which is today regarded as sclerosing RME. No tumor showed the characteristic gene fusion of RMA. Five children had localized disease, one bone metastases, and another lymph-node involvement. All primaries were grossly resected. One locoregional relapse occurred. At a median follow-up of 7 years, all patients were alive disease-free. PT-RMS can show a focal alveolar histology combined with typical features of RME. In current morphological classifications, all rhabdomyosarcomas qualify for the alveolar subtype if typical features of RMA are realized at least focally. Rhabdomyosarcomas consisting of pure RMA morphology were however not found in our patients with PT-RMS. The mixed RMA/RMEs identified in our population-based study did not show a translocation typical for RMA and had a good prognosis. Further prospective studies need to evaluate if mixed RMA/RMEs have a similar favorable outcome in non-paratesticular sites as well.

Keywords

Rhabdomyosarcoma Paratesticular Alveolar subtype Pediatric oncology 

Notes

Acknowledgements

This article is dedicated to Ivo Leuschner, who did most of the pathologic review. We are indebted to the personnel of all participating institutions and thank all patients, parents, and guardians for their willingness to participate in the CWS trials. We thank Erika Hallmen and Simone Feuchtgruber for their excellent data management. This research was supported by the Deutsche Kinderkrebsstiftung, Bonn; Deutsche Krebshilfe e.V., Bonn; and the Förderkreis Krebskranke kinder e.V. Stuttgart, Germany.

Compliance with ethical standards

Research involving human participants and informed consent

All CWS studies were approved by the appropriate ethics committees and written informed consent was obtained from patients, guardians/parents, or both.

Conflict of interest statement

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Tobias M. Dantonello
    • 1
    • 2
  • Christian Vokuhl
    • 3
  • Monika Scheer
    • 1
  • Monika Sparber-Sauer
    • 1
  • Sabine Stegmaier
    • 1
  • Guido Seitz
    • 4
  • Heike Scheithauer
    • 5
  • Jörg Faber
    • 6
  • Iris Veit-Friedrich
    • 1
  • Peter Kaatsch
    • 7
  • Stefan S. Bielack
    • 1
    • 8
  • Thomas Klingebiel
    • 9
  • Ewa Koscielniak
    • 1
    • 10
  • on behalf of the Cooperative Weichteilsarkom Studiengruppe (CWS)
  1. 1.Pediatrics 5 (Oncology, Hematology, Immunology), Stuttgart Cancer CenterKlinikum Stuttgart—OlgahospitalStuttgartGermany
  2. 2.Olgahospital, Pediatrics 3 (Cardiology, Pulmonology, Intensive Care)Klinikum StuttgartStuttgartGermany
  3. 3.Kiel Pediatric Tumor Registry, Department of Pediatric PathologyUniversity of KielKielGermany
  4. 4.Department for Pediatric SurgeryUniversity of MarburgMarburgGermany
  5. 5.Department of RadiotherapyLudwig Maximilian UniversityMunichGermany
  6. 6.Department of Pediatric OncologyUniversity of MainzMainzGermany
  7. 7.German Childhood Cancer RegistryMainzGermany
  8. 8.Department of Pediatric Hematology and OncologyUniversity Children’s Hospital MünsterMünsterGermany
  9. 9.Department of Pediatric OncologyUniversity of Frankfurt (Main)FrankfurtGermany
  10. 10.Department of Pediatric OncologyUniversity of TubingenTubingenGermany

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