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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 27  |  Issue : 1  |  Page : 36-38

Recalcitrant desmoid tumor of the abdominal wall: A reconstructive dilemma


1 Department of Plastic Surgery, AIIMS, Jodhpur, Rajasthan, India
2 Department of Surgery, AIIMS, Jodhpur, Rajasthan, India

Date of Web Publication4-Jan-2019

Correspondence Address:
Dr. Prakash Chandra Kala
Department of Plastic Surgery, AIIMS, Jodhpur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjps.tjps_64_18

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  Abstract 


Desmoid tumors are slow-growing benign tumors. These tumors infiltrate surrounding tissues but not malignant and associated with women of fertile age, especially during and after pregnancy. We report a young male patient with a recurrent desmoid tumor of the anterior abdominal wall who underwent resection four times earlier. The patient had a history of four-time operation over the same site for the same disease. Preoperative evaluation included thorough clinical examination with abdominal ultrasound. The histology revealed a desmoid tumor. Primary surgical resection and immediate reconstruction of abdominal defect were performed.

Keywords: Desmoid tumors, primary surgical resection, vertical rectus abdominis myocutaneous


How to cite this article:
Kala PC, Sahu RK, Choudhary R, Puranik A. Recalcitrant desmoid tumor of the abdominal wall: A reconstructive dilemma. Turk J Plast Surg 2019;27:36-8

How to cite this URL:
Kala PC, Sahu RK, Choudhary R, Puranik A. Recalcitrant desmoid tumor of the abdominal wall: A reconstructive dilemma. Turk J Plast Surg [serial online] 2019 [cited 2019 Aug 18];27:36-8. Available from: http://www.turkjplastsurg.org/text.asp?2019/27/1/36/249405




  Introduction Top


Desmoid tumors constitute a less aggressive form of fibromatosis, originating from muscle and aponeurosis. The term desmoid was coined by Muller in 1838 and is derived from the Greek word desmos, which means tendon-like. They account for <3% of all soft tissue neoplasms.[1] These tumors are associated with women in fertile age, especially during and after pregnancy or trauma/operations.[2] Abdominal wall is the most common location of desmoid tumors. They do not metastasize, but they have tendency to recur with an incidence of 50%. Repetitive surgery makes abdominal wall reconstruction difficult.


  Case Report Top


A 26-year-old male patient presented with a painless mass in the lower abdomen for 2 months which was gradually increasing in size. The patient had a history of three recurrences. The average duration between recurrences was 6 months. In this patient, the last surgical intervention was performed 10 months ago. No other surgery was performed in the body. On examination, multiple lower abdominal wall masses involving right iliac regions were observed and the largest one was 6x6 cm in size. Main mass was fixed to the anterior abdominal wall, nontender, globular in shape measuring 6x6 cm with smooth surface, and firm in consistency. Skin in the lower half was scared due to previous operations [Figure 1]. Ultrasonography (USG) demonstrated a large solid heterogeneous hypoechoic mass showing internal vascularity, few anechoic areas of necrosis confined to anterior abdominal wall. No enlarged lymph node or ascites were seen. No focal lesion was noted in the bone or liver. Computed tomography or magnetic resonance imaging was not advised as USG already explained its local extent.
Figure 1: Preoperative photographs showing growth with extensive lower abdominal scaring

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After preoperative workup, complete excision of the tumor with wide surgical margins (5 cm) was done. Abdominal wall down to the peritoneum was removed, resulting in a large wall defect of about 12x12 cm which was very difficult to close without flap surgery [Figure 2]. Skin of the right lilac and suprapubic area was scared due to previous surgeries for recurrence of disease. It was challenging to dissect rectus in inferior part. We plan vertical rectus abdominis myocutaneous (VRAM) flap based on inferior epigastric artery. It was not possible to elevate flap on the same side rectus muscle. Hence, contralateral rectus muscle base vertical skin island was elevated and rotated over the defect. Donor-site defect was reinforced with polypropylene mesh [Figure 3]. Wound closed after keeping vacuum suction drain beneath the subcutaneous space. Excised mass was heavy weighing 500 g. On cut section, it was gritty texture and glistening white. The postoperative course was uneventful, and the patient was discharged on the 10th postoperative day [Figure 4]. Histopathology reports were consistent with desmoid tumor with negative surgical margins. There was no recurrence in 1-year follow-up, but there is no definite time limit for recurrence; however, in this case, duration this is almost double in follow-up, and there is no recurrence time.
Figure 2: Excised specimen with wide adequate margins

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Figure 3: Final defect after excision and mesh covering in the defect with flap marking

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Figure 4: Early postoperative photograph with well-settled flap

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  Discussion Top


Abdominal desmoid tumor occurs in the abdominal wall in 40-50% of cases.[3] History, tumor location within the previous operative site, and USG findings supoort tumor recurrence. Definitive diagnosis must be established with histopathologic analysis. Desmoid tumor usually presents as a firm mass with ill-defined margins and no distinct capsule. On cut surface, they are gritty, glistening white, and trabeculated resembling scar tissue. Histologically, desmoid tumors consist of elongated fibroblasts and myofibroblasts.[4] Although they do not metastasize, their infiltrative growth and tendency to recur even after macroscopic complete excision cause real challenge in desmoid tumor management. Surgery must be radical with the intent of achieving free margins. This may leave extensive parietal defect. However, recurrence still can occur in 20%–30% of cases after complete resection. The median time to recurrence reported in the literature is 14–17 months.[5] This leads to repeated operations. Whether primary or recurrent desmoid tumor, the integrity of the abdominal wall must be restored. Anatomical reconstruction by direct suture is not always possible. Often prosthetic material is necessary to cover the defect. We used a double layer prolene mesh for intraperitoneal placement of viscera. After fixation the mesh, abdominal wall defect is covered with a VRAM flap. The effectiveness of radiotherapy, chemotherapy, hormone therapy, and anti-inflammatory treatment is not proven yet. These therapies are recommended in patients with inoperable tumors, local recurrences, or incompletely excised lesions. Metastatic disease has not been reported with desmoid tumor. Sulindac, indomethacin, and tamoxifen along with varieties of antineoplastic medications have been used to treat these patients with variable results.


  Conclusion Top


Surgery when possible is still the treatment of choice, whether in primary or recurrent abdominal wall desmoid tumor. The quality of resection is a main prognosis factor. Radical surgery results in significant loss of substance in the abdominal wall which can be managed using many reconstruction techniques. Repair by a double layer mesh covered by a pedicled fascial or musculofascial flap seems to be a simple and efficient method.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chen CB, Chiou YY, Chen CH, Chou YH, Chiang JH, Chang CY, et al. Sonographic and computed tomography findings of intra-abdominal desmoid tumor. J Chin Med Assoc 2010;73:393-5.  Back to cited text no. 1
    
2.
Kumar V, Khanna S, Khanna AK, Khanna R. Desmoid tumors: Experience of 32 cases and review of the literature. Indian J Cancer 2009;46:34-9.  Back to cited text no. 2
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3.
Arshad AR, Normala B. Surgical management of large desmoid tumour of the anterior abdominal wall. Asian J Surg 2008;31:90-5.  Back to cited text no. 3
    
4.
Lahat G, Nachmany I, Itzkowitz E, Abu-Abeid S, Barazovsky E, Merimsky O, et al. Surgery for sporadic abdominal desmoid tumor: Is low/no recurrence an achievable goal? Isr Med Assoc J 2009;11:398-402.  Back to cited text no. 4
    
5.
Stoeckle E, Coindre JM, Longy M, Binh MB, Kantor G, Kind M, et al. A critical analysis of treatment strategies in desmoid tumours: A review of a series of 106 cases. Eur J Surg Oncol 2009;35:129-34.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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