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Undifferentiated Pleomorphic Sarcoma after Pirfenidone Use: A Case Report
Christine A Moore1, Aaysha Kapila2
1Internist at East Tennessee State University at Kingsport. mooreca2@etsu.edu.
The Permanente Journal
|April 28, 2018
Summary
Pirfenidone treats idiopathic pulmonary fibrosis but a rare soft-tissue sarcoma developed in one patient. Further studies are needed to assess long-term pirfenidone safety and potential adverse effects.
Area of Science:
- Oncology
- Pulmonology
- Pharmacology
Background:
- Pirfenidone is approved for idiopathic pulmonary fibrosis (IPF), inhibiting growth factors to reduce fibroblast proliferation and collagen synthesis.
- The drug demonstrates improved progression-free survival and good tolerability in IPF patients.
- Long-term safety data for pirfenidone are limited.
Observation:
- A rare case of undifferentiated pleomorphic sarcoma (UPS) is presented in a 59-year-old male IPF patient.
- The patient received pirfenidone treatment for over one year prior to UPS development.
- UPS, also known as malignant fibrous histiocytoma, is a soft-tissue sarcoma originating from fibroblasts.
Findings:
- This case highlights a potential, albeit rare, adverse event associated with long-term pirfenidone use.
- UPS typically affects elderly patients and presents in extremities or trunk, rarely retroperitoneum.
- Surgical excision is primary treatment for UPS, but recurrence with metastasis is common.
Implications:
- This case underscores the need for postmarketing surveillance of pirfenidone.
- Longitudinal studies are crucial to identify potential additional adverse effects in IPF patients on pirfenidone.
- Further research should investigate the relationship between pirfenidone therapy and sarcoma development.
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