Desmoid tumours of the anterior abdominal wall

European Journal of Surgical Oncology 1999; 25: 398–400

Desmoid tumours of the anterior abdominal wall R. J. Sutton and J. M. Thomas The Royal Marsden Hospital, London, UK

Aims: To review the surgical management and outcomes for large desmoid tumours of the abdominal wall. Methods: Seven patients with large desmoid tumours of the anterior abdominal wall were treated by wide local excision and reconstruction with two layers of Marlex∇ mesh (Bard, Galway, Ireland). Results: No patient having initial surgery at this hospital has either a significant residual functional deficit or developed a recurrence. Conclusions: Large desmoid tumours of the abdominal wall are safely and adequately managed with abdominal wall resection followed by mesh reconstruction. Key words: desmoid; fibromatosis; abdominal wall.

Introduction When the desmoid tumour, or aggressive fibromatosis, presents in the anterior abdominal wall its size, site, propensity to local recurrence and reconstructive considerations create a surgical challenge. Desmoid tumours are rare, accounting for less than 3% of soft-tissue tumours, or 0.03% of all neoplasms,1 and in our series about 9% occur in the anterior abdominal wall. Surgical experience in the literature is limited, although it is agreed that whenever possible the tumour should be widely excised,2,3 despite an estimated local recurrence rate of 25–65%.4 We have reviewed the seven patients treated at this unit over the last 8 years to evaluate treatment.

Patients and methods This is a retrospective study of seven patients who were referred to one Consultant Surgeon at the Royal Marsden Hospital between 1989 and 1997 with a desmoid tumour of the anterior abdominal wall (Table 1). Six patients were women, mean age 28.5 years (range 16–37 years): three had previous pregnancies, and one was only 1 year post-partum at presentation. The only male patient was aged 19 years at presentation and had undergone a total colectomy the previous year for familial adenomatous polyposis (FAP). In two cases the tumour was found to have arisen within a surgical scar. One patient was referred with a recurrent desmoid tumour, which arose within 1 year of her initial surgery. Correspondence to: Mr J. M. Thomas, Department of Academic Surgery, The Royal Marsden Hospital, Fulham Road, London SW3 6JJ, UK. 0748–7983/99/040398+03 $12.00/0

Four had the diagnosis confirmed prior to referral; three by open biopsy (one became infected) and one by Trucut core biopsy (Travenol Laboratories, Deerfield, Illinois, USA). The remaining two cases were confirmed by Tru-cut core biopsy. All patients had a pre-operative computed tomography (CT) scan, as well as a rigid sigmoidoscopy to exclude FAP. Prior to referral hormone manipulation had been tried unsuccessfully in two patients (one with tamoxifen 20 mg o.d., and one with toremifene 200 mg o.d.). One of these patients had also received a short course of chemotherapy (vincristine and methotrexate), also with no effect. The tumour involved the anterior abdominal wall alone in four patients, but adjacent structures were involved (diaphragm, inguinal ligament, liver and stomach) in the other three. The median maximum tumour diameter of the resected specimens was 11.7 cm (range 4–15 cm). Whenever possible the tumour was widely excised with a margin of 1 cm of macroscopically normal tissue. In those cases where the tumour involved adjacent structures, clearance was achieved by continuing the dissection to resect the involved segment of that structure, such as an antral gastrectomy. Invariably the tumour is inseparable from the underlying peritoneum, which must be sacrificed for clearance. Whenever possible the greater ommentum was sutured to the margins of the defect to produce a ‘neo-peritoneum’ and to separate gut from Marlex∇ mesh (Bard, Galway, Ireland). Reconstruction was performed with two layers of Marlex∇ mesh, the first layer being sutured to the margins of the defect ‘drum tight’ and the second layer to normal tissues beyond the defect, such that there was no overlap of suture lines. All operations were performed under antibiotic cover, which was continued until the suction drains were removed.  1999 W.B. Saunders Company Limited

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Desmoid tumours of the abdominal wall Table 1. Data of individual cases Patient

Age (years) Sex Method of diagnosis

1

2

3

4

5

6

7

23 F Referred with recurrent disease

37 F Tru-cut core biopsy + CT scan None

19 M Tru-cut core biopsy + CT scan None

27 F Incision biopsy + CT scan None

16 F Incision biopsy + CT scan Toremifene

31 F Tru-cut core biopsy+ CT scan

12×6×9

9.5×8.5×7

10×6×5

14×5×5

13×11.5×11/5

Stomach, liver, pancreas 36

None

Inguinal ligament

None None

19

36

36

Pre-operative treatment

Previous surgery

Tumour size (cm) Other organ involvement

4×3×2

37 F Incision biopsy + MRI Tamoxifen Vincristine Methotrexate 4×4×3

None

None

72

48

Follow-up (months)

None

14

MRI, magnetic resonance imaging.

Results There were no peri-operative complications. The median follow-up was 3.5 years (range 14 months to 6 years). Only one patient developed a tumour recurrence (also the only case referred with recurrent disease). This measured 4 cm in diameter and was re-excised with a 2-cm margin. The patient is now disease-free at 6 years and had a successful pregnancy and normal vaginal delivery 1 year post-surgery. No patient has either an abdominal wall weakness or an incisional hernia and no patient complains of pain or discomfort. One patient who required a gastric antrectomy to clear the disease has an intermittent problem with gastric emptying. Discussion The desmoid tumour is a benign soft-tissue neoplasm. It is an unencapsulated, locally invasive fibroblastic proliferation arising in fascia or aponeurosis and may cause severe local morbidity or even death by infiltration of surrounding structures.4 It is strongly associated with female sex, FAP and surgical trauma. Twenty-five per cent arise in a surgical scar.2 Between 73 and 87% of desmoid tumours occur in women2 and they are especially common in young women during, or shortly after, pregnancy.5 The reason for this is not clear, though Reitamo et al. found that fertile female patients with desmoid tumours had a significant predisposition to oestrogen predominance and a deviation from progesterone dominance. The oestrogen receptor was present in 33% of tumours assayed and the density was significantly higher than in healthy control tissues.6 FAP has been shown to be associated with germ line mutations of the adenomatous polyposis coli (APC) gene on chromosome 5.7 Different mutations in this gene may produce different clinical pictures, such as the development

of extra-colonic manifestations including osteomas, epidermal cysts and desmoid tumours.8 This combination of FAP with extra-colonic disease is commonly referred to as Gardener’s syndrome. Mutations between codons 1445 and 1578 on the APC gene are associated with the development of desmoid tumours,9 though this is found uncommonly in sporadic disease.10 The overall incidence of desmoid tumours in FAP is 8%.11 The diagnosis can therefore be suspected if the patient is young and female, has FAP or if there is a history of rapid growth. It can be confirmed almost invariably by Tru-cut core biopsy with an accuracy of greater than 90%.12,13 Open incision biopsy has a complication rate of 17%.14 Although desmoid tumours lack the ability to metastasize, eradication is difficult due to insidious local extension, which leads to a risk of local recurrence after wide excision, the majority of which should manifest within the first 2 years.15,16 However, this characteristic of the disease is poorly understood as recurrence is by no means inevitable after local excision, even when there is known to be residual disease.6,17 In our experience about 40% of patients who recur enter a ‘plateau phase’, often after a period of rapid growth.18 Furthermore, spontaneous regression has been reported,5 most notably in post-menopausal women.19 In this series, all pathological margins were clear of disease. In the event of a margin being microscopically involved the authors would not advocate re-excision but would await events and widely excise any palpable recurrence. The case for adjuvant radiotherapy in this setting is unproven20,21 and in any event the proximity and tolerance of underlying intraperitoneal structures is a relative contraindication. This experience demonstrates that large desmoid tumours of the abdominal wall can be treated successfully by wide surgical excision and reconstruction using two layers of Marlex∇ mesh. For patients having initial surgery at this hospital there has been no tumour recurrence.

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R. J. Sutton and J. M. Thomas References

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