Successful management of aggressive fibromatosis of the neck using wide surgical excision: a case report

Abstract

Introduction: Aggressive fibromatosis is a benign tumor, thought to arise from deep musculoaponeurotic structures, rarely found in the head or neck. However, when it does occur in the head and neck region, it tends to be more aggressive and associated with significant morbidity, which may be attributed to the vital vascular, neurological or anatomical structures in close proximity.

Case presentation: We report the case of a 39-year-old Pakistani man who presented with a two-month history of a lump on the right side of his neck. The mass was excised and histopathological analysis revealed a case of aggressive fibromatosis.

Conclusion: Due to the rarity of the condition no guidelines are available on the indications and extent of each modality. Due to its aggressive behavior and tendency to invade local structures and recur, a multi-modality management strategy is usually employed. On the basis of this case, we suggest that aggressive surgery is a viable management option and may be successfully used as a single modality treatment.

Figure 1

39-year-old male with a right sided neck mass. Scars from previous excisions are seen. The reccurring mass on examination.

Figure 2

Axial (A) and sagittal (B) T1 MRI scans with contrast showing the mass.

Figure 3

Coronal (A), axial (B) and sagittal (C) CT scans with contrast showing the mass measuring about 9.3 × 5.9 × 12 cm with areas of central necrosis. The carotid sheath is displaced anteriorly.

Figure 4

Histopathological examination of the excised tissue. (A) Fibrosarcoma arising in a background of dermatofibrosarcoma protuberans showing proliferation of spindle cells with a prominent storiform pattern and cells exhibiting pleomorphism with increase mitotic activity (H&E, 10 ×). (B) Fibromatosis showing proliferation of bland spindle shaped cells with infiltration into the muscle at the periphery and perivascular lymphocytic infiltrate (H&E, 10 ×).