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 ¤  Abstract
 ¤ Introduction
 ¤ Case Report
 ¤ Discussion
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 Table of Contents     
UNUSUAL CASE
Year : 2018  |  Volume : 14  |  Issue : 3  |  Page : 247-249
 

Port site desmoid tumour following laparoscopic cholecystectomy: A case report


Department of General Surgery, Division of Surgical Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission21-Oct-2017
Date of Acceptance04-Nov-2017
Date of Web Publication6-Jun-2018

Correspondence Address:
Prof. Ganga Ram Verma
Department of General Surgery, Division of Surgical Gastroenterology, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmas.JMAS_209_17

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 ¤ Abstract 

Desmoid tumours are locally aggressive tumours occurring either spontaneously or in familial conditions. History of trauma is invariably present with surgical trauma being a common cause. Port site desmoid tumours are extremely rare conditions. Inadequate treatment results in high recurrence rate and substantial morbidity. Reconstruction, if required, by the appropriate technique is vital to avoid an incisional hernia. Adjuvant therapy may be useful in large locally advanced or recurrent tumours. We describe a young female with large port site desmoid tumour following laparoscopic cholecystectomy managed with wide local excision and mesh placement.


Keywords: Laparoscopic cholecystectomy, mesh repair, port-site desmoid, wide local excision


How to cite this article:
Krishnamurthy G, Jha VC, Verma GR. Port site desmoid tumour following laparoscopic cholecystectomy: A case report. J Min Access Surg 2018;14:247-9

How to cite this URL:
Krishnamurthy G, Jha VC, Verma GR. Port site desmoid tumour following laparoscopic cholecystectomy: A case report. J Min Access Surg [serial online] 2018 [cited 2020 Jan 25];14:247-9. Available from: http://www.journalofmas.com/text.asp?2018/14/3/247/222434



 ¤ Introduction Top


Desmoid tumours are rare benign yet locally aggressive tumours of mesenchymal origin. It is characterised by abnormal proliferation of myofibroblast and accounts for 0.03% of all neoplasms.[1] It has a female preponderance of reproductive age and known to develop following surgical trauma.[2] The mechanism of their development is unknown and failure to recognise their malignant potential renders the desmoids tumours susceptible to inadequate treatment and high recurrence rate.

Development of desmoids at the port site following minimally access surgery is an extremely rare phenomenon. We present a case of a young female with a desmoid tumour in the abdominal wall at the anterior axillary port site following laparoscopic cholecystectomy. The rare occurrence of the tumour following laparoscopic cholecystectomy makes it an interesting association, and in view of its malignant potential, warrants awareness.


 ¤ Case Report Top


A 34-year-old female presented with a gradually increasing painless parietal swelling over the right lumbar region for the past 2 years. She had noticed a rapid increase in size for the past 6 months. Abdominal examination revealed a firm, well-defined 10 cm × 8 cm lump arising from the anterior abdominal wall occupying right hypochondrium and the right lumbar region. Two months before the onset of the swelling, she underwent laparoscopic cholecystectomy. The right lumbar port site scar was found exactly over the summit of the lump. There was no family history of desmoid tumour. Contrast enhanced computed tomography (CECT) confirmed a homogenous mass of 9 cm × 7 cm × 10 cm arising from the right rectus abdominis muscle indenting the parietal peritoneum [Figure 1]a. Core needle biopsy confirmed the presence of spindle and stellate tumour cells embedded in the dense stroma, consistent with a desmoid tumour. The patient underwent wide local excision of abdominal wall muscles and parietal peritoneum. The defect was repaired with composite mesh and covered with native skin and subcutaneous tissue [Figure 1]b. She had an uneventful postoperative recovery.
Figure 1: (a) Desmoid tumour (*) arising from the anterior abdominal wall abutting the peritoneum with no involvement of the underlying structures. (b) Intra-operative picture showing the composite mesh placed over omentum to cover the defect following the excision of the desmoid tumour

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Histopathological examination revealed spindle- and stellate-shaped tumour cells embedded in dense fibrous stroma showing myxoid degeneration and focal hyalinisation suggestive of desmoid [Figure 2].
Figure 2: (a) Gross Specimen and the (b) cut section of the tumour. (c) Spindle-shaped cells with dense stroma, suggestive of desmoid tumour (H and E, ×40)

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She was started on tamoxifen 20 mg daily. At 20 months follow-up, the patient is doing well. There is no evidence of recurrence or an incisional hernia.


 ¤ Discussion Top


Desmoid tumour develops from abnormal fibroblastic proliferation and characterised by abundant collagen surrounding poorly circumscribed bundles of elongated spindle-shaped cells.[1] They are locally aggressive tumours and do not metastasise to distant organs. In general, most desmoids are slow growing, but 10% may progress rapidly as in the index case.[3] The patient had noticed a rapid increase in size 6 months before her presentation.

Most of the tumours occur sporadically, but in 5% of cases, familial and association with familial adenomatous polyposis have been described.[4] The mechanisms of the development and regulation of growth of desmoids tumours are unknown. Female sex hormones might be acting as a catalyst in the proliferation of fibroblasts as reflected by its tendency to develop in childbearing females, the possible role of anti-estrogenic drugs in management and the appearance of similar lesions in laboratory animals by administration of oestrogen.[5],[6]

Previous history of trauma, often surgical in nature may trigger the development of desmoids in as high as 28% of cases.[7] Wound healing and the fibroblastic proliferation takes place within 2 weeks of trauma. The resultant mesenchymal cellular proliferation may get dysregulated and acquire invasiveness from genetic or chance mutations, leading to aggressive fibromatosis also called desmoid.[3] The present case is the fourth case of trocar site desmoids after laparoscopic surgery.[8],[9]

The common sites of involvement include proximal extremities and abdominal wall. The tumour appears as homogenous soft-tissue mass of variable attenuation and enhancement on CECT. The imaging besides confirming the site of origin also helps in planning the extent of resection.[1] Fine-needle aspiration has very poor specificity in diagnosing a desmoid tumour. Hence, core needle biopsy is the recommended for pre-operative tissue diagnosis.[10] Histologically, these tumours appear benign in nature but may behave aggressively in their natural course.

Wide excision with negative margins remains the first-line of management in abdominal wall desmoid tumours. Abdominal wall reconstruction can be achieved with primary suturing, mesh repair or myocutaneous flap depending on the extent of the defect.[11] Pedunculated desmoids projecting inside the abdomen can be excised by laparoscopic method [7] obviating the need of mesh or flap reconstruction. Hormonal therapy with tamoxifen or non-steroidal anti-inflammatory drugs has proved effective occasionally. Radiotherapy between 40 and 60 Gy has also been reported as an effective treatment with low toxicity in residual or recurrent tumours.[12]


 ¤ Conclusion Top


Trivial trauma from trocar may trigger uncontrolled fibroblastic proliferation leading to the development of desmoids in the anterior abdominal wall. CECT scan and core needle biopsy clinch the diagnosis. Wide excision with negative margins is the cornerstone in the management of abdominal wall desmoids. Large abdominal defect needs reconstruction with mesh to avoid an incisional hernia. Adjuvant treatment may be useful in large locally advanced or recurrent desmoids.

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.
Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol 2001;27:701-6.  Back to cited text no. 1
[PUBMED]    
2.
Lynch HT, Fitzgibbons R Jr. Surgery, desmoid tumors, and familial adenomatous polyposis: Case report and literature review. Am J Gastroenterol 1996;91:2598-601.  Back to cited text no. 2
[PUBMED]    
3.
Righetti AE, Jacomini C, Parra RS, de Almeida AL, Rocha JJ, Féres O, et al. Familial adenomatous polyposis and desmoid tumors. Clinics (Sao Paulo) 2011;66:1839-42.  Back to cited text no. 3
    
4.
Gurbuz AK, Giardiello FM, Petersen GM, Krush AJ, Offerhaus GJ, Booker SV, et al. Desmoid tumours in familial adenomatous polyposis. Gut 1994;35:377-81.  Back to cited text no. 4
[PUBMED]    
5.
Sturt NJ, Clark SK. Current ideas in desmoid tumours. Fam Cancer 2006;5:275-85.  Back to cited text no. 5
[PUBMED]    
6.
Wilcken N, Tattersall MH. Endocrine therapy for desmoid tumors. Cancer 1991;68:1384-8.  Back to cited text no. 6
[PUBMED]    
7.
Lopez R, Kemalyan N, Moseley HS, Dennis D, Vetto RM. Problems in diagnosis and management of desmoid tumors. Am J Surg 1990;159:450-3.  Back to cited text no. 7
[PUBMED]    
8.
Suárez Artacho G, Jiménez Rodríguez R, Díaz Pavón JM, Sánchez Gil J, Vázquez Monchul J. Desmoid tumor arising in a laparoscopic trocar site after cholectomy. Rev Esp Enferm Dig 2009;101:814-5.  Back to cited text no. 8
    
9.
Meshikhes AW, Al-Zahrani H, Ewies T. Laparoscopic excision of abdominal wall desmoid tumor. Asian J Endosc Surg 2016;9:79-82.  Back to cited text no. 9
[PUBMED]    
10.
Dalén BP, Meis-Kindblom JM, Sumathi VP, Ryd W, Kindblom LG. Fine-needle aspiration cytology and core needle biopsy in the preoperative diagnosis of desmoid tumors. Acta Orthop 2006;77:926-31.  Back to cited text no. 10
    
11.
Rohrich RJ, Lowe JB, Hackney FL, Bowman JL, Hobar PC. An algorithm for abdominal wall reconstruction. Plast Reconstr Surg 2000;105:202-16.  Back to cited text no. 11
[PUBMED]    
12.
Sakorafas GH, Nissotakis C, Peros G. Abdominal desmoid tumors. Surg Oncol 2007;16:131-42.  Back to cited text no. 12
[PUBMED]    


    Figures

  [Figure 1], [Figure 2]



 

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