Desmoid tumours are uncommon. They are locally invasive and incomplete excision leads to recurrence, which can pose a significant management challenge. Patients therefore require effective treatment, which essentially entails tumour excision with a clear surgical margin. The resulting wide defect may lead to difficulty in closure of the anterior abdominal wall. We report our experience in treating large desmoid tumours of the anterior abdominal wall. Between January 2000 and December 2001, three patients with large desmoid tumour of the anterior abdominal wall were treated with wide excision, which included a 3-cm margin of uninvolved tissues. This led to a considerable abdominal wall defect. The peritoneal defect was closed as a separate layer, though under considerable tension, while the abdominal wall musculature defect was closed with a polypropylene mesh. All three patients recovered well with no immediate or late postoperative morbidity. Follow-up until December 2006 has not revealed any tumour recurrence or hernia development. Wide excision of an anterior abdominal wall desmoid tumour with a clear margin of 3 cm including the peritoneum should be considered when managing such tumours. Closure under tension of the peritoneum did not seem to produce any morbidity.
Desmoid fibromatosis is a benign yet locally aggressive tumor with a tendency to recur. It causes considerable morbidity particularly when it arises in a small area in the head and neck region. This tumor is extremely rare in the submandibular region. We report a case of desmoid tumor in the submandibular region in a 32-year-old male who presented with right submandibular swelling postextraction of right lower wisdom tooth. Excision biopsy was carried out initially following inconclusive fine needle aspiration and discussion at multidisciplinary meeting. The tumor recurred 4 months following initial excisional biopsy necessitating a more radical secondary approach involving segmental mandibulectomy. Intraoperatively we also noted that the tumor was originating from the site of previous wisdom tooth extraction, raising the question of surgical trauma as precursor of desmoid tumor. We achieved a negative resection margin and a complete remission for 24 months.
Desmoid type fibromatosis of the breast is a rare stromal tumor that accounts for <0.2% of all breast tumors. Bilateral and multicentric lesions are extremely rare, with only less than ten cases reported in the literature. Although benign, it is locally aggressive with frequent recurrence in up to almost one-third of the cases. We experienced our first case of bilateral multicentric breast fibromatosis in a 19-year-old woman, with a paternal aunt diagnosed with breast cancer at age 30, who presented to our institution with the chief complaint of retracted nipples for 1 year. The patient denied any history of trauma to her chest. Sonography showed suspicious bilateral hypoechoic masses. Magnetic resonance imaging (MRI) was performed for further evaluation because of the extensive involvement of both the breasts. This report aimed to illustrate the main clinical, radiological, and histopathological characteristics of this rare disease to increase awareness of this entity and discuss the role of MRI.