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The Desmoid Tumor Research Foundation

June 2024

CLINICAL AND EPIDEMIOLOGIC EVALUATION OF DESMOID TUMORS IN A BRAZILIAN SARCOMA REFERENCE CENTER

Author Information

Cassia da Silva 1, Fábio Fernando Eloi Pinto 2, Ademar Lopes 1, Suely Akiko Nakagawa 2, Marcelo Porfirio Sunagua Aruquipa 3, Samuel Aguiar 1, Celso Abdon Lopes de Mello 3

1A.C. Camargo Cancer Center, Sarcoma and Bone Tumor Reference Center, Department of Surgical Oncology, Sao Paulo, SP, Brazil. 2A.C. Camargo Cancer Center, Sarcoma and Bone Tumor Reference Center, Departament of Oncologic Orthopedics, Sao Paulo, SP, Brazil. 3A.C. Camargo Cancer Center, Sarcoma and Bone Tumor Reference Center, Department of Clinical Oncology, Sao Paulo, SP, Brazil.

Publication

Acta Ortop Bras

Summary

Introduction: Desmoid Tumors (DT) are rare neoplasms with higher incidence in younger women. Methods: Retrospective, single-center analysis of patients with DT. Variables were age, sex, biopsy, treatment and recurrence. The disease-free survival (DFS) was calculated with the Kaplan-Meier method. Results: 242 patients were evaluated, mean age was 34 years, 70.7% women, 44.4% originated in the trunk/abdomen and 54.5% had size > 5cm. Surgery was performed in 70.2%, 31% with negative margin and only 57% with previous biopsy. Recurrence rate was 38% and 1,2,5-year DFS was 75.3%, 64.2%, 57.8%, respectively. Size (p = 0.018) and tumor location in the dorsum (p = 0.001), extremities (p = 0.003) and pelvis (p = 0.003) were related to higher relapse rate. Conclusion: our data reinforces the need to gather data from real world practice and the importance of awareness of DT and medical education about DT behavior and best approach due to the high rates of surgery and elevated number of patients treated without biopsy. Level of Evidence III; Retrospective Comparative Study.

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