Desmoid tumor surgery

Desmoid tumor Microchapters
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Case #1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Overview

The goals of surgery include tumor removal and functional restoration at the tumor site. Standard surgical goal is wide local excision with a grossly negative microscopic margin followed by reconstruction of defect with skin graft, rotational muscle flap or free muscle flap. Abdominal wall resection may be required to close the defect and minimize the risk of hernias. Incomplete tumorremoval or involved excision margins may lead to local recurrence in 25% to 40% of patients. Hence, because of high recurrence risk post-surgery, imaging of the tumor site with ultrasound or MRI scans may be recommended to closely monitor patient’s health on follow up visits after surgery.

Surgery

• Surgical resection is not recommended among patients with advanced or metastatic malignancy
• Desmoid tumor does not have any propensity for metastasis although it may aggressively invade structures
• The optimal management for aggressive fibromatosis depends on tumor location and extent

Goals of surgery

• The goals of surgery are twofold:
  • To remove the tumor
  • To restore function at the site of the tumor
• Surgical resection may be offered although the likelihood of local recurrence after surgery is high, particularly if margins are positive
• Wide local excision with a grossly negative microscopic margin followed by reconstruction of the defect is the standard surgical goal
  • Reconstruction may include:
    • Skin graft (from patient's own thigh or buttocks to cover and protect the area where the tumor was removed)
    • Rotational muscle flap (taken from patient's own muscle near the surgery site, and rotated to fill the area where the tumor was removed)
    • Free muscle flap (muscle is taken from somewhere else in the patient's own body, relocated, and then reconnected with the blood vessels in the area where the tumor was removed)
• Full-thickness resection of the tumor-containing abdominal wall with a grossly negative microscopic margin has to be performed when the lesion closely approximates or involves the peritoneum
• Abdominal wall resection may be required to close the defect and minimize the risk of hernias after resection of abdominal wall desmoids
• As intraabdominal desmoids usually involve the mesentery, so the resection generally requires concomitant bowel resection
• Surgeon must take care not to compromise the superior mesenteric artery or vein during resection
• Given the propensity of desmoids to recur, reconstruction should allow for the possibility of future resections and reconstructions
• Intraperitoneal organs or adjacent bony structures involved by tumor must be resected as well
• Incomplete tumor removal or involved excision margins may lead to local recurrence
• It is estimated that 25 to 40% of patients who undergo surgery can have a local recurrence
• Because of high recurrence rate even after being successfully treated, it is necessary for the patient to keep seeing a physician regularly for follow up visits
• Imaging of the tumor site with ultrasound or MRI scans may be recommended to closely monitor patient’s health on follow up visits

Successful outcome after laparoscopic surgery for sporadic colonic desmoid tumor with β-catenin mutation: a case report. Magnetic resonance imaging findings showed the desmoid tumor (arrows) with lower signal intensity on T1-weighted image (a) and high signal intensity on T2-weighted image (b and c). Note that the desmoid tumor was next to contrast-filled transverse colon. (a and b) Axial plane. (c) Coronal plane.Source: Gunji S. et al, Department of Surgery, Kyoto University Graduate School of Medicine, 54 Shogoin- Kawara-cho, Sakyo-ku, Kyoto, 606-8507, Japan