Beware of the Iceberg Phenomenon: A Case Report of Chest Wall Fibrous Dysplasia

TL;DR

A rare case of chest wall fibrous dysplasia is reported, highlighting the importance of regular imaging to manage tumor progression and avoid severe symptoms.

Contribution

This case report emphasizes the diagnostic and management challenges of intrathoracic fibrous dysplasia and advocates for timely surgical intervention.

Findings

Thoracic fibrous dysplasia can remain stable extrathoracically while intrathoracically progressing significantly.

A hemi-clamshell surgical approach effectively removed the tumor while preserving mediastinal structures.

Regular follow-up imaging is essential to prevent symptom onset and complex resections.

Abstract

Thoracic fibrous dysplasia (FD) is a benign, osseous chest wall tumor. It originates from bone marrow and accounts for 30–50% of all benign osseous neoplasms in the chest wall. In FD, normal bone marrow is replaced by fibrous stroma and immature bone. We present a rare case in which massive intrathoracic polyostotic FD originating from the rib was diagnosed and treated. The extrathoracic part of the tumor appeared stable and unalarming for decades; however, in hindsight, the intrathoracic part significantly progressed, eventually leading to symptoms. The tumor was removed through a hemi-clamshell approach, which allowed adequate visualization and control of mediastinal structures. After establishing the diagnosis of FD, regular follow-up imaging is crucial for timing of a surgical intervention to prevent symptoms, impairment of quality of life, and unnecessarily complex resections.

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