Management of a female with recurrence of fibromatosis of the chest wall adjacent to the breast: a case report
© Shen et al; licensee BioMed Central Ltd. 2013
Received: 19 September 2012
Accepted: 4 March 2013
Published: 8 March 2013
Extra-abdominal desmoid tumor is a rare soft tissue tumor that is histologically benign, but may behave aggressively. This case report specifically describes the clinical, radiographic, and pathologic features of 27 year-old female who experienced a post-surgical recurrence of fibromatosis of the chest wall over a two-year period of time secondary to previous inadequate excision. The fibromatosis was found to be involving the lower-inner quadrant of her right breast and causing worsening pain. A surgical management strategy was successfully undertaken.
KeywordsAggressive fibromatosis Recurrent desmoid tumour Chest wall Breast
Desmoid tumor is an aggressive fibromatosis that may occur in abdominal and extra-abdominal areas . They constitute less than 0.03% of all neoplasms . For extra-abdominal desmoids, local recurrence rates range from 24% to 77% in reported series [3–5]. Although the fibromatosis of chest wall represents 8-10% of all cases and surgery is the primary treatment modality, there remains a significant lack of agreement amongst surgeons on how to manage the disease that is suspected to have accompanied involvement of adjacent breast especially for an unmarried woman. We report a case where the tumour had a repeated recurrence over a 2-year period.
A 27 year-old unmarried female presented to our hospital with worsening pain of the right chest wall region and a recurrent obvious mass adjacent to the lower-inner quadrant of her right breast for 5 months. Two years prior to her current presentation, she presented to a local hospital with a palpable right chest wall mass close to the same quadrant of right breast and underwent a surgical resection. The histological examination showed a typical picture of an aggressive fibromatosis.
As diagnosis was established, surgery was scheduled. Under general anesthesia with selective intubation, the patient lay on the operating table with supine position. She underwent an en bloc resection of the tumor and the underlying musculature (inferior lateral portion of the right pectoralis major muscle and superior portion of the right rectus abdominis musculature, and anterior portion of the right latissimus dorsi muscle) and en bloc resection of the underlying chest wall structures (seventh rib and intercostal muscle). The defect on the right chest wall was then closed with a 10×10 cm Dacron patch. Although the mass was in close proximity to the lower-inner quadrant of the right breast, all surgical margins were negative with the intraoperative frozen section and the right mastectomy was avoided. Then the right breast was closed in the skin flap plasty. Patient was transferred to the intensive care unit and moved to general care after 24 hours and she was discharged to home after a week. She is currently under follow-up.
Extra-abdominal desmoids are frequently referred to as fibromatoses that are mesenchymal neoplasms and develop from the connective tissues, fasciae and aponeuroses.
For the treatment of this recurrent tumour, an aggressive surgical resection strategy with a safe margin (2–3 cm) remains the standard therapeutic anoeuvre, which has been strongly advocated by surgeons dealing with fibromatosis of the chest wall that involves the breast [6, 7]. The reasons that support this aggressive surgical approach include the potentials of fibromatosis to grow aggressively locally and to invade into the surrounding structures, thus having a high rate of local recurrence when incompletely excised with positive surgical margins. Some surgeons, however, disagree with such a wide excision and prefer more conservative resection [8–10]. Their concerns are about the less optimal cosmetic outcome and the risk of loss of function. Further, there have not been any reports showing fibromatosis can metastasize. Presence of residual tumor cannot be clearly shown to impact disadvantageously on five-year disease free or overall survival . In our case, we chose to perform a wide en bloc resection to avoid future recurrence, based on negative frozen section on the surgical margin. Thus, we were able to avoid mastectomy. A flap plasty and cosmetology of breast were performed. This is particularly beneficial to the young woman who was going to get married in the next year and her quality of life was not adversely affected.
For a large recurrent fibromatosis involving the chest wall and breast, wide en bloc resection is necessary to prevent future recurrence but shall avoid mastectomy if possible. This surgical management is particularly useful for unmarried young women.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We greatly appreciate the assistance of the staff of the Department of Thoracic Surgery, West-China Hospital, Sichuan University, and thank them for their efforts.
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