Background: Fibromatosis or desmoid tumor is a rare soft tissue tumor that lacks a metastatic potential, but is characterized by a locally aggressive and infiltrating growth pattern and a high propensity toward local recurrence if incompletely excised.
Case presentation: We report a patient with three post-surgical recurrences of fibromatosis of the breast over a seven year period. The fibromatosis was found to be involving the chest wall musculature and causing persistent and worsening pain. An aggressive operative strategy was undertaken, consisting of mastectomy with en bloc resection of the underlying chest wall musculature, ribs, and parietal pleura.
Conclusion: Aggressive surgical management of fibromatosis of the breast with suspected chest wall involvement is appropriate to attempt to obtain a long-term durable cure.