Rhabdomyosarcoma diagnosed in the first year of life: Localized, metastatic, and relapsed disease. Outcome data from five trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS)

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Rhabdomyosarcoma diagnosed in the first year of life : Localized, metastatic, and relapsed disease. Outcome data from five trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS)

Sparber-Sauer, Monika (author): Klinikum Stuttgart Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany

Stegrnaier, Sabine (author): Klinikum Stuttgart Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany

Vokuhl, Christian (author): Kiel Pediat Tumor Registry, Dept Pathol, Sect Pediat Pathol, Kiel, Germany

Seitz, Guido (author): Univ Childrens Hosp Marburg, Dept Pediat Surg, Marburg, Germany

von Kalle, Thekla (author): Olga Hosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed, Klinikum Stuttgart,Inst Radiol, Stuttgart, Germany

Scheer, Monika (author): Klinikum Stuttgart Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany

Muenter, Marc (author): Klinikum Stuttgart, Inst Radiotherapy, Stuttgart, Germany

Bielack, Stefan S. (author): Klinikum Stuttgart Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany;Univ Munster, Dept Pediat Hematol & Oncol, Munster, Germany

Weclawek-Tompol, Jadwiga (author): Univ Wroclaw, Dept Pediat Hematol Oncol & BMT, Wroclaw, Poland

Ladenstein, Ruth (author): St Anna Childrens Hosp, Pediat Oncol, Vienna, Austria

Niggli, Felix (author): Univ Zurich, Dept Pediat Oncol, Zurich, Switzerland

Ljungman, Gustaf, 1958- (author): Uppsala universitet,Barnneurologi/Barnonkologi

Fuchs, Joerg (author): Univ Childrens Hosp, Dept Pediat Surg & Urol, Tubingen, Germany

Hettmer, Simone (author): Univ Freiburg, Fac Med, Dept Pediat & Adolescent Med, Div Pediat Hematol & Oncol, Freiburg, Germany

Koscielniak, Ewa (author): Klinikum Stuttgart Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany;Childrens Hosp, Dept Pediat Hematol & Oncol, Tubingen, Germany

Klingebiel, Thomas (author): Univ Hosp Frankfurt, Dept Children & Adolescents, Frankfurt, Germany

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2019-02-14
2019
English.
In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 66:6

Related links:: https://urn.kb.se/re...; show more...; https://doi.org/10.1...; show less...

Journal article (peer-reviewed)

Abstract Subject headings

Background Rhabdomyosarcoma (RMS) diagnosed during the first year of life is reported to have poor outcome. Little is known about treatment and outcome data of relapsed disease (RD).MethodsCharacteristics, treatment, and outcome of 155 patients <= 12 months registered within the Cooperative Weichteilsarkom Studiengruppe (CWS) between 1981 and 2016 were evaluated.Results Localized disease (LD) was diagnosed in 144 patients and metastatic disease (MD) in 11. The histological diagnosis was alveolar (RMA) (n = 38, 23/25 examined patients PAX7/3:FOXO1-positive), embryonal (RME) (n = 100), botryoid (n = 10), anaplastic (n = 1), and spindle-cell RMS (n = 6). Multimodal treatment including conventional (age-adjusted) chemotherapy (CHT) (n = 150), resection (n = 137), and radiotherapy (RT) (n = 37) was administered. Complete remission was achieved in 129 of 144 patients with LD. RD occurred in 51 infants at a median age of 1.7 years (range, 0.3-8.8). Sixty-three percent of patients with RMA suffered RD, in contrast to 28% of patients with RME. Relapse treatment consisted of conventional CHT (n = 48), resection (n = 28), and RT (n = 21). The pattern of relapse and best resection were significant prognostic factors for patients with RD (P = 0.000 and P = 0.002). Late effects occurred as secondary malignancies in 6%, long-term toxicity in 21%, and resection-related impairment in 33% of the 105 surviving patients. The 5-year event-free survival and overall survival for infants with initial LD were 51% and 69%, 14% and 14% for patients with initial MD and 39% and 41% for relapsed patients, respectively.Conclusion Multimodal treatment including microscopically complete resection is strongly recommended to achieve a good prognosis in LD and RD of infants with RMS.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Pediatrik (hsv//swe)
MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Pediatrics (hsv//eng)

Keyword

CWS Group
infants
localized disease
metastatic disease
relapsed disease
rhabdomyosarcoma

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By the author/editor: Sparber-Sauer, M ...; Stegrnaier, Sabi ...; Vokuhl, Christia ...; Seitz, Guido; von Kalle, Thekl ...; Scheer, Monika; show more...; Muenter, Marc; Bielack, Stefan ...; Weclawek-Tompol, ...; Ladenstein, Ruth; Niggli, Felix; Ljungman, Gustaf ...; Fuchs, Joerg; Hettmer, Simone; Koscielniak, Ewa; Klingebiel, Thom ...; show less...

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MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Hematology

MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Pediatrics

Articles in the publication: Pediatric Blood ...

By the university: Uppsala University

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