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Dermatofibrosarcoma Protu-Berans with Lung Metastasis Requiring Pneumonectomy
Ana Collazo Lorduy1, Berta Obispo2, Karen Villar3
1Department of Medical Oncology, Hospital Universitario de la Princesa, Madrid, Spain; Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Rare Tumors
|January 21, 2016
Summary
Dermatofibrosarcoma protuberans rarely metastasizes to the lungs without prior recurrence. Targeted therapy with Imatinib, alongside surgery and radiotherapy, achieved stable disease in a unique case.
Area of Science:
- Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing skin cancer.
- Metastasis from DFSP is uncommon, typically occurring after local recurrence or with fibrosarcomatous change.
Observation:
- A young man presented with lung metastases of DFSP seven years post-resection, with no prior local recurrence.
- Histopathology did not reveal fibrosarcomatous transformation, a known risk factor for metastasis.
Findings:
- A multimodal treatment approach including surgery, radiotherapy, and targeted therapy with Imatinib was employed.
- The patient achieved stable disease for 30 months following the diagnosis of metastases.
Implications:
- This case highlights rare metastatic DFSP presentation without antecedent local recurrence or specific histological features.
- It underscores the importance of considering targeted therapies like Imatinib for advanced DFSP to improve outcomes.
