A CASE OF DESMOID TUMOUR

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J. Evid. Based Med. Healthc., pISSN- 2349-2562, eISSN- 2349-2570/ Vol. 3/Issue 46/June 09, 2016 Page 2308

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ABDOMINAL FIBROMATOSIS IN A YOUNG FEMALE

PRESENTING WITH ABDOMINAL PAIN

Achu Jacob Philip1_{, Peter Manoharan}2_{, Nivedha Ravy}3

1_{Post Graduate Resident, Department of General Surgery, Pondicherry Institute of Medical Sciences, Puducherry.}

2_{Professor, Department of General Surgery, Pondicherry Institute of Medical Sciences, Puducherry.}

3_{House Surgeon, Department of General Surgery, Pondicherry Institute of Medical Sciences, Puducherry.}

ABSTRACT

Desmoid tumours are rare; they account for about 0.03 percent of all neoplasms and less than 3 percent of all soft tissue tumours. The estimated incidence in the general population is two to four per million population per year. Here reporting a case of a 34-year-old female presenting with pain abdomen and swelling chest, excised mass diagnosed to be a desmoid tumour.

KEYWORDS

Desmoid Tumour.

HOW TO CITE THIS ARTICLE: Philip AJ, Manoharan P, Ravy N. A case of desmoid tumour – abdominal fibromatosis in a young female, presenting with abdominal pain. J. Evid. Based Med. Healthc. 2016; 3(46), 2308-2311.

DOI: 10.18410/jebmh/2016/510

INTRODUCTION: Fibromatosis refers to a group of benign soft tissue tumours which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behaviour with frequent local recurrence.[1,2]

Prognostic Factors: Age, Tumour Size, Tumour Site.

Abdominal Fibromatosis: Arises within abdominal wall of women during or after pregnancy. May be seen with caesarean section scar.

Extra-abdominal Fibromatosis: Arises outside abdomen and abdominal wall, usually in muscles of shoulder, chest wall, back and thigh, equal gender frequencies.

Intra-abdominal Fibromatosis: Mesenteric, Pelvic or Retroperitoneal Locations, Associated with Gardner's Syndrome.

Case Report: A 34-year-old female came with complaints of abdominal pain and pain over the lower aspect of chest on left side for 3 years. She has a swelling over the left lower part of chest for 7 years initially increased in size and stable. No other comorbidities. Gives a history of abdominal surgery for trauma. Haemodynamically stable patient.

Systemic Examination: Within Normal Limits.

Local Examination: She had a swelling in the lower part of chest left side, below the left costal margin, bony hard in

consistency, fixed to the skin, on mobile, no tenderness, surface smooth, no dilated veins over the swelling, no pulsations.

Epigastric scar extending up to left chest, patient gives h/o surgery for abdominal wall mass. Also gives history of placement of mesh. USG guided biopsy was taken and sent for HPE which was suggestive of benign spindle cell lesion-? abdominal fibromatosis.

CECT Thorax: Showed partly well-defined soft tissue density lesion adjacent to the left anterior muscles at the level of 4th_{, 5}th_{, 6}th_{, 7}th_{, 8}th_{, and 9}th_ribs.

Sclerosis with adjacent? periosteal reaction involving the left 7th_{, 8}th_{ribs adjacent to the above-mentioned soft}

tissue densities.

Imaging Features Described Could Represent:

 Multiple chronic haematomas.

 Soft tissue masses (? Desmoids).

 Patient was taken up for excision, Mass was excised in toto and send for HPE.

Histopathology showed low-grade spindle cell lesion with infiltrative nature. Possibility of fibromatosis is considered. Postoperative period was uneventful; sutures were removed on POD 9.

Histopathology Features

Financial or Other, Competing Interest: None. Submission 12-05-2016, Peer Review 21-05-2016, Acceptance 30-05-2016, Published 09-06-2016. Corresponding Author:

Dr. Achu Jacob Philip, Room No. 205, PG Annex,

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USG Guided FNAC from the Lesion

X-ray Chest: Showing Soft Tissue Mass

USG Showing Abdominal Lesion

Plain CT (Tumour Mass on Anterior Wall)

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Post-Operative Wound Site

DISCUSSION: Desmoids are rare and are generally benign. Intra-abdominal desmoids, are usually seen in patients with familial adenomatous polyposis (FAP): patients with FAP and a family history of desmoids tumours have a 25 per cent chance of developing desmoids.[3,4]_{Various biological and}

chemical factors have been reported as playing a role in the growth of desmoids. Desmoid can also arise rarely from abdominal wall. The growth rate of desmoids is unpredictable, and spontaneous regression has been reported. Surgical trauma has been implicated as an aetiological factor in the development of desmoids, which have been reported to occur as early as 3 months and as late as 5 years after surgery.[5] _{In our case, the patient had}

undergone abdominal surgery for mass before her presentation with the abdominal mass.

The recurrence rate after incomplete excision is high, suggesting that surgical trauma may be a causative factor for a new somatic mutation. Some studies have emphasised that hormonal factors (Particularly Oestrogen, Prostaglandins) can influence the growth of desmoids.[2,6]

The incidence of sporadic intra-abdominal desmoid tumours is rare.[7]_{Intra-abdominal desmoids can present as}

suprapubic masses of longstanding duration or as abdominal masses. Presenting symptoms include anaemia, weight loss, malnutrition and ureteric obstruction, abdominal pain, nausea and vomiting, dysphagia, and a raised white blood

cell count. Our patient presented with an abdominal mass of

weeks’ duration.

Although surgical intervention is advocated as the best modality of treatment by many, a variety of other approaches have been described. The objective is to obtain tumour-free margins wherever possible.[7,5]_{The difficulty in}

treatment associated with intra-abdominal desmoids is principally related to their location in the vicinity of strategic organs. This presents a challenge in obtaining tumour-free margins. A positive margin is the leading cause of recurrence, although the extent needed for a clear margin is not defined in literature.[8] _{Wide excision has been}

recommended as the preferred mode of treatment because of the risk of local recurrence after incomplete excision of the tumour, which often invades the surrounding viscera.[9,7,8]_{En bloc excision, along with the adjacent bowel,}

has been recommended as the preferred method of treatment for desmoid tumours of the mesentery. In the present case, a wide-margin excision of mass in toto was done.

Proposed adjuvant therapies for the treatment of desmoid tumours include perioperative use of drugs such as sulindac and toremifene. Other modalities of treatment have been explored in the literature, including radiotherapy, nonsteroidal anti-inflammatory drugs such as sulindac and indomethacin, chemotherapy including vinblastine and methotrexate, and antiestrogens such as tamoxifen, especially in patients where complete resection is not possible.[9,5,6]

CONCLUSION: Desmoid tumours are very rare presentation, mostly arising in patient’s post-surgery or trauma. In our case, patient presented with mass post-surgery, and surgical excision of tumour was done in toto leaving adequate free margin. The chances of recurrence cannot be ruled out. Patient will require regular follow-up. Desmoid tumour is mostly a benign tumour and presenting as an anterior abdominal wall tumour is very rare. Most of such patients will require wide local excision with free margin, and if needed the abdominal can be reinforced with composite mesh to prevent future incisional hernia.

REFERENCES

1. Shields CJ, Winter DC, Kirwan WO, et al. Desmoid tumours. Eur J Surg Oncol 2001;27(8):701–706. 2. Bailey & Love’s Short Practices of Surgery. Desmoid

tumour. CRC Press 2013;26th_edn:969.

3. Courtney M Townsend, Daniel Beauchamp R, Mark Evers B, et al. Sabiston text book of surgery. Elsevier 2012;19th_{edn:1096-1097.}

4. Ballo MT, Zagars GK, Pollack A, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol 1999;17(1):158–167.

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6. Gronchi A, Casali PG, Mariani L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol 2003;21(7):1390– 1397.

7. Lopez R, Kemalyan N, Moseley HS, et al. Problems in diagnosis and management of desmoid tumours. Am J Surg 1990;159(5):450–453.

8. Disher AC, Biswas M, Miller TQ, et al. Atypical desmoids tumor of the abdomen: a case report. J Natl Med Assoc 1993;85(4):309-311.