Langerhans cell histiocytosis as differential diagnosis of a mediastinal tumor
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Published on: March 30, 2018
René Fahrner1, Beatrix Hoksch, Mathias Gugger
1Division of Thoracic Surgery, University Hospital Bern, Switzerland.
Insights
This case report details a rare instance of adult Langerhans cell histiocytosis (LCH) originating in the mediastinum. The study highlights the diagnostic process for this unusual chest wall tumor.
Area of Science:
- Oncology
- Pathology
- Radiology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease of myeloid origin.
- While typically affecting children, LCH can occur in adults, presenting with diverse clinical manifestations.
- Mediastinal involvement in adult LCH is exceptionally uncommon.
Observation:
- A 55-year-old male presented with parasternal swelling, indicative of a chest wall mass.
- Imaging revealed a large, infiltrative chest wall tumor.
- Open biopsy and histopathologic examination were crucial for diagnosis.
Findings:
- Histopathology showed skeletal muscle infiltrated by histiocytic cells.
- Immunohistochemistry confirmed the presence of CD20, CD3, S-100 proteins, and CD1a.
- These findings were characteristic of Langerhans cell histiocytosis (LCH).
Implications:
- This case represents a potentially novel presentation of adult LCH originating in the mediastinum.
- Highlights the importance of considering rare diagnoses in adult chest wall tumors.
- Contributes to the understanding of extraskeletal LCH in adults.
Abstract:
We describe the case of a 55-year-old man who presented with parasternal swelling. The chest CT scan showed a large tumor of the chest wall infiltrating the subcutaneous tissue. To assume histologic diagnosis an open biopsy was performed. Between the myofibrils a coarse, white tumor with infiltrative growth was noted. Histopathologic examination revealed expanded atrophic skeletal muscle that was infiltrated by histiocytic cells. Numerous eosinophilic granulocytes and lymphocytes CD20 and CD3 positive could be detected and immunohistochemical staining was also positive for S-100 proteins and CD1a. Histologic findings were characteristic of Langerhans cell histiocytosis (LCH). To the best of our knowledge a LCH originating from the mediastinum in an adult as presented has not been previously described.