Desmoid-type fibromatosis of the chest wall: a case report

Abstract

Desmoid-type fibromatosis (DF), also known as deep fibromatosis or desmoid tumor, is an extremely rare neoplasm that develops from fascia and musculoaponeurotic tissue. These tumors are characterized by slow progressive growth, local invasion, and local recurrence after surgical excision, but they lack metastatic potential. DF accounts for 3.5% of all fibrous tumors, with an annual incidence of approximately 2-4/million. Until now, only a small number of cases have been found in the chest wall.Herein, we present a rare case of chest wall DF in a 43-year-old female, which was discovered accidentally due to a thoracic wall mass that extended outward from the sternum. Computed tomography scans revealed a subcutaneous soft tissue mass anterior to the sternum, which was considered to be a mesenchymal tumor or an inflammatory lesion. The patient underwent surgical excision of the mass. The mass was completely removed and all margins were negative. According to the pathological results, the patient was finally diagnosed as DF. Postoperative radiotherapy was suggested subsequently, especially considering the locally aggressive and infiltrative nature of the tumor. However, this was rejected by the patient, and biannual reexamination was recommended instead. Despite the absence of postoperative radiotherapy, there was no evidence of local recurrence 2 years later. We consider regular postoperative follow-up may be able to replace postoperative radiotherapy, and if there exist an opportunity to completely resect the mass, surgical is a worthwhile choice.

A 10-year-old male with chest wall Desmoid tumor – a rare tumor with unusual presentation

“…Also, prior surgery and excessive estrogen exposure may be linked to desmoids [ 8 ]. These tumors cannot spread by metastasis, they are characterized by slow, gradual growth, local invasion, and local recurrence after surgical excision [ 4 ]. Recurrence remains a problem following resection of desmoid tumors with as many as 50% of patients experiencing a recurrence within 5 years, factors associated with recurrence included age, tumor location, and margin status [ 8 ].…”

“…DF may affect any region but is most frequently found in the extremities, abdominal wall, and abdominal mesentery [ 9 ]. There haven’t been many specialized studies on tumors of the chest wall [ 4 , 10 ]. It is a very rare site for these tumors [ 11 ].…”

“…However, trauma to the tumor site has been identified in 25% of known cases [ 1 ]. Surgery is considered a potentially curative treatment [ 4 ], but local recurrence after surgical excision is expected [ 5 ]. Herein, we present a 12-year-old male patient who presented with a desmoid tumor on the chest wall 2 years ago – at age 10-year-old - after mild trauma managed successfully with surgery without recurrence in the last 2 years with close follow-up monthly with physical exam, chest X-ray and chest CT every 3–6 months.…”