Low-grade fibromyxoid sarcoma: A report of the Cooperative Weichteilsarkom Studiengruppe (CWS)
| Autor: | Jörg Fuchs, Bernarda Kazanowska, Iris Veit-Friedrich, Marc Münter, Michael Greulich, Thomas Klingebiel, Thekla von Kalle, Felix Niggli, Stefan S. Bielack, Kirsi Jahnukainen, Gustaf Ljungman, Christian Vokuhl, Monika Sparber-Sauer, Ewa Koscielniak, Sabine Stegmaier, Steffan Loff, Monika Scheer, Ruth Ladenstein |
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| Rok vydání: | 2019 |
| Předmět: |
Surgical resection
Male medicine.medical_specialty Adolescent Fibrosarcoma Fibroma Thigh Low-grade fibromyxoid sarcoma 03 medical and health sciences 0302 clinical medicine medicine Humans Neoplasm Metastasis Child Univariate analysis Lung business.industry Soft tissue sarcoma Infant Margins of Excision Hematology medicine.disease Prognosis Confidence interval Survival Rate medicine.anatomical_structure Oncology 030220 oncology & carcinogenesis Child Preschool Pediatrics Perinatology and Child Health Female Radiology Sarcoma Neoplasm Grading Neoplasm Recurrence Local business 030215 immunology Follow-Up Studies |
| Zdroj: | Pediatric bloodcancerREFERENCES. 67(2) |
| ISSN: | 1545-5017 |
| Popis: | BACKGROUND Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor with benign histologic appearance, though fully malignant behavior is possible. METHODS Patients with LGFMS 5 cm. The best surgical result was resection with free margins (R0) in 24 and microscopic residuals (R1) in seven. Five-year event-free (EFS), 5-year local-relapse-free (LRFS), and 5-year overall-survival were 71 ± 18.6% confidence interval (CI) 95%, 76 ± 17.6% CI 95%, and 100%, respectively. Six patients suffered local relapse in a median of 1 year, one combined within 1.3 year and one metastatic relapse with lesions in the lung, back muscles, and thigh discovered in whole-body imaging 6 years after the first diagnosis. In univariate analysis, T status correlated with EFS (T1 79.6 ± 18.6%, T2 50.0 ± 49.0%, P = .038). Resection with free margins tends to be associated with better LRFS (R0 82.4 ± 18.6%, R1 53.6 ± 39.4%, P = .053). Among 24 patients with R0 resection, five (21%) suffered relapse, thereof three local, one metastatic, and one combined. Among seven patients with R1-resection, three (43%) suffered local relapse. CONCLUSION Special caution is advisable in T2 tumors. The metastatic potential with lesions in unusual sites indicates that affected patients need to be informed. If long-term follow-up with whole-body imaging is beneficial, it may be addressed in larger intergroup analyses. Further research in disease biology is essential for optimal treatment and follow-up care. |
| Databáze: | OpenAIRE |
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