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Case Reports
. 2023 Jan 30:9:1007760.
doi: 10.3389/fsurg.2022.1007760. eCollection 2022.

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

Santiago A Endara  1 , Gerardo A Dávalos  1 , Gabriel A Molina  2 , Christian A Armijos  3 , D Sebastian Narvaez  4 , Nelson Montalvo  5
Affiliations
Case Reports

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

Santiago A Endara et al. Front Surg. .

Abstract

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.

Keywords: desmoid disease; desmoid fibromatosis; desmoid tumor; mediastinal mass; pediatric.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
(A) chest mass, (B) ultrasound, echogenic mass in the anterior mediastinum. (C) CT, The mass is in close contact with the xiphoid process. (D) MRI, the mass is near the pericardium.
Figure 2
Figure 2
(A) Mass invading the costal cartilages and sternum. (B) Reconstruction with prosthetic material.
Figure 3
Figure 3
Pathology HE 4X, fusiform cells without nuclear hyperchromatism, hypocellular, and hyalinized areas are seen. Immunohistochemistry positive for Beta-catenin (Inset 40x).
See this image and copyright information in PMC

References

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