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The Desmoid Tumor Research Foundation

January 2023

Case Report: Desmoid fibromatosis in the mediastinum of a 6-month-old toddler, what to do?

Author Information

Endara SA (1), Dávalos GA (1), Molina GA (2), Armijos CA (3), Narvaez DS (4) and Montalvo N (5).

(1) Department of General Surgery, Division of Cardiothoracic Surgery, Hospital Metropolitano, Quito-Ecuador, (2) Universidad San Francisco de Quito, Quito-Ecuador, (3) Department of Internal Medicine, Imaging and Interventional Radiology Service, Hospital Metropolitano, Quito-Ecuador, (4) Universidad Internacional del Ecuador-Hospital Metropolitano PGY1 General Surgery, Quito-Ecuador, (5) Department of Internal Medicine, Pathology Service, Hospital Metropolitano, Quito-Ecuador

Publication

Frontiers in Surgery

Summary

Desmoid fibromatosis is a rare, aggressive borderline lesion arising from soft tissues. Treatment will depend on the structures that the tumor has involved. Surgery with negative margins is the recommended strategy as it can usually achieve disease control; however, the tumor's location sometimes does not allow it. Therefore, a combination of medical therapies along with strict surveillance is crucial. We present the case of a 6-month-old boy with a chest mass. After further evaluation, a rapidly growing mediastinal mass involving the sternum and costal cartilage was detected. Desmoid fibromatosis was the final diagnosis.

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