Desmoplastic small round cell tumors: Multimodality treatment and new risk factors.

Abdominal Neoplasms / pathology Adolescent Adult Antineoplastic Agents / therapeutic use Antineoplastic Combined Chemotherapy Protocols / therapeutic use C-Reactive Protein / analysis Child Combined Modality Therapy Desmoplastic Small Round Cell Tumor / pathology Female Humans Male Pleural Effusion / diagnosis Prognosis Risk Factors Stem Cell Transplantation Venous Thrombosis / diagnosis Young Adult

C-reactive protein Trousseau’s syndrome desmoplastic small round cell tumor maintenance therapy soft tissue sarcoma

Journal

Cancer medicine

ISSN: 2045-7634

Titre abrégé: Cancer Med

Pays: United States

ID NLM: 101595310

Informations de publication

Date de publication:
02 2019

Historique:

received: 17 07 2018

revised: 28 11 2018

accepted: 29 11 2018

pubmed: 18 1 2019

medline: 27 3 2020

entrez: 18 1 2019

Statut: ppublish

Résumé

To evaluate optimal therapy and potential risk factors. Data of DSRCT patients <40 years treated in prospective CWS trials 1997-2015 were analyzed. Median age of 60 patients was 14.5 years. Male:female ratio was 4:1. Tumors were abdominal/retroperitoneal in 56/60 (93%). 6/60 (10%) presented with a localized mass, 16/60 (27%) regionally disseminated nodes, and 38/60 (63%) with extraperitoneal metastases. At diagnosis, 23/60 (38%) patients had effusions, 4/60 (7%) a thrombosis, and 37/54 (69%) elevated CRP. 40/60 (67%) patients underwent tumor resection, 21/60 (35%) macroscopically complete. 37/60 (62%) received chemotherapy according to CEVAIE (ifosfamide, vincristine, actinomycin D, carboplatin, epirubicin, etoposide), 15/60 (25%) VAIA (ifosfamide, vincristine, adriamycin, actinomycin D) and, 5/60 (8%) P6 (cyclophosphamide, doxorubicin, vincristine, ifosfamide, etoposide). Nine received high-dose chemotherapy, 6 received regional hyperthermia, and 20 received radiotherapy. Among 25 patients achieving complete remission, 18 (72%) received metronomic therapies. Three-year event-free (EFS) and overall survival (OS) were 11% (±8 confidence interval [CI] 95%) and 30% (±12 CI 95%), respectively, for all patients and 26.7% (±18.0 CI 95%) and 56.9% (±20.4 CI 95%) for 25 patients achieving remission. Extra-abdominal site, localized disease, no effusion or ascites only, absence of thrombosis, normal CRP, complete tumor resection, and chemotherapy with VAIA correlated with EFS in univariate analysis. In multivariate analysis, significant factors were no thrombosis and chemotherapy with VAIA. In patients achieving complete remission, metronomic therapy with cyclophosphamide/vinblastine correlated with prolonged time to relapse. Pleural effusions, venous thrombosis, and CRP elevation were identified as potential risk factors. The VAIA scheme showed best outcome. Maintenance therapy should be investigated further.

Sections du résumé

BACKGROUND

To evaluate optimal therapy and potential risk factors.

METHODS

Data of DSRCT patients <40 years treated in prospective CWS trials 1997-2015 were analyzed.

RESULTS

Median age of 60 patients was 14.5 years. Male:female ratio was 4:1. Tumors were abdominal/retroperitoneal in 56/60 (93%). 6/60 (10%) presented with a localized mass, 16/60 (27%) regionally disseminated nodes, and 38/60 (63%) with extraperitoneal metastases. At diagnosis, 23/60 (38%) patients had effusions, 4/60 (7%) a thrombosis, and 37/54 (69%) elevated CRP. 40/60 (67%) patients underwent tumor resection, 21/60 (35%) macroscopically complete. 37/60 (62%) received chemotherapy according to CEVAIE (ifosfamide, vincristine, actinomycin D, carboplatin, epirubicin, etoposide), 15/60 (25%) VAIA (ifosfamide, vincristine, adriamycin, actinomycin D) and, 5/60 (8%) P6 (cyclophosphamide, doxorubicin, vincristine, ifosfamide, etoposide). Nine received high-dose chemotherapy, 6 received regional hyperthermia, and 20 received radiotherapy. Among 25 patients achieving complete remission, 18 (72%) received metronomic therapies. Three-year event-free (EFS) and overall survival (OS) were 11% (±8 confidence interval [CI] 95%) and 30% (±12 CI 95%), respectively, for all patients and 26.7% (±18.0 CI 95%) and 56.9% (±20.4 CI 95%) for 25 patients achieving remission. Extra-abdominal site, localized disease, no effusion or ascites only, absence of thrombosis, normal CRP, complete tumor resection, and chemotherapy with VAIA correlated with EFS in univariate analysis. In multivariate analysis, significant factors were no thrombosis and chemotherapy with VAIA. In patients achieving complete remission, metronomic therapy with cyclophosphamide/vinblastine correlated with prolonged time to relapse.

CONCLUSION

Pleural effusions, venous thrombosis, and CRP elevation were identified as potential risk factors. The VAIA scheme showed best outcome. Maintenance therapy should be investigated further.

Identifiants

DOI: 10.1002/cam4.1940 PMID: 30652419 PMC: PMC6382921

pubmed: 30652419

doi: 10.1002/cam4.1940

pmc: PMC6382921

doi:

Substances chimiques

Antineoplastic Agents 0

C-Reactive Protein 9007-41-4

Types de publication

Clinical Trial Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

Pagination

527-542

Informations de copyright

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