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 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 3  |  Issue : 2  |  Page : 178-181

Denovo childhood desmoid tumor


Department of Surgery, King Abdullah Medical City, Meccah, Saudi Arabia

Date of Web Publication13-Dec-2013

Correspondence Address:
Timor A Alshee
Department of Surgery, King Abdullah Medical City, Meccah
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.122981

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  Abstract 

Desmoid fibromatosis is characterized by local aggressive growth without any tendency of metastasis. These very rare tumors can develop in any musculo-aponeurotic structure and they can be found in all regions of the human body. We present a case of a 15-year-old girl who presented with left lower abdominal mass of 4 months duration. The mass was firm, non-tender and fixed to the left lower abdominal wall. Abdominal computed tomography (CT) showed a focal, but non-demarcated mass on the anterior abdominal wall musculature. Following CT guided tru-cut biopsy and histological examination, a diagnosis of desmoid tumor was made. The tumor was completely excised and the 20 cm Χ 16 cm defect closed with a physic mesh. There was no tumor recurrence at 18 months follow-up. Desmoid tumor commonly affects abdominal wall muscles of females. Following proper evaluation and histological diagnosis complete excision of the tumor is attended with good outcome.

Keywords: Aggressive fibromatosis, childhood, desmoid


How to cite this article:
Alshee TA, Zahrani AA, Shafi SM. Denovo childhood desmoid tumor. Arch Int Surg 2013;3:178-81

How to cite this URL:
Alshee TA, Zahrani AA, Shafi SM. Denovo childhood desmoid tumor. Arch Int Surg [serial online] 2013 [cited 2024 Mar 1];3:178-81. Available from: https://www.archintsurg.org/text.asp?2013/3/2/178/122981


  Introduction Top


The clinical behavior and prognosis of desmoid tumor are very diverse and depend upon the anatomic location and proximity to vitally important organs. A correlation with the familial intestinal polyposis could be shown. [1] Approximately, 10-25% of patients with polyposis present intra- or extra-abdominal desmoid tumors. [2] Suggested risk factors for desmoid are previous surgery, pregnancy and hormonal treatment with estrogen. The estrogen dependence of the tumors explains the stimulation of their growth by estrogen high flux during pregnancy. The recurrence of desmoid tumors ranges from 20% to 60% in large retrospective studies. However, in many cases, a recurrence of the aggressive tumors after resection is described. [3] Salas et al. showed three unfavorable prognostic factors comprising age less than 37 years, tumor size >7 cm and extra-abdominal tumor location. [4] A clear difference in progression free survival was demonstrated according to the number of prognostic factors present. Kumar et al. described a recurrence rate of 25% in a group of 32 patients. [5] The most important factor for recurrence was a tumor size of >5 cm. [5] In this paper, we present a 15-year-old girl who was evaluated and treated for desmoids tumor of the abdominal wall. We will also discuss the risk factors, prognostic factors and treatment options of desmoid tumor.


  Case Report Top


A 15-year-old female patient presented to the out-patient clinic of the surgical oncology department of our hospital with a left lower quadrant abdominal mass of 4 months duration, which was gradually increasing in size. Patient gave no history of previous trauma, surgery or pregnancy. She was a known case of insulin dependent diabetes mellitus and her family history was unremarkable. She had been injecting insulin on her anterior arms for 3 years.

Upon physical examination, the mass was firm, non-tender and fixed to the left lower abdominal wall. Other systemic examination did not reveal any abnormality. The patient had complete blood counts, renal and liver function tests and the result were within normal limits.

Abdominal computed tomography (CT) scan showed a focal, but non-demarcated mass of 10.0 cm × 7.0 cm involving the whole anterior abdominal wall musculature [Figure 1]. The mass was abutting the external lilac vessels and local infiltration could not be ruled out. There was no evidence of intra-abdominal metastasis. Abdominal magnetic resonance imaging (MRI) [Figure 2] showed similar findings to those of abdominal CT. An ultrasound guided tru-cut biopsy was performed and the diagnosis of desmoid tumor was confirmed by typical histologically findings [Figure 3].
Figure 1: Computed tomography image showing the extent of the tumor

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Figure 2: Magnetic resonance imaging images showing the proximity of the tumor to the vital structures

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Figure 3: Histopathology of the lesion with characteristic features of desmoid

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The patient was admitted and prepared for surgical excision with blood investigations and pre-anesthesia check-up. The mass was excised completely [Figure 4]. Lymph nodes around the external iliac vessels were sent for frozen section, which came as negative for infiltration. Removal of the mass resulted in an abdominal wall defect 20 cm × 16 cm in width, which was repaired using a physic mesh [Figure 5]. She passed the post-operative period smoothly and was discharged home on the 5 th post-operative day. She was followed-up for 18 months with no recurrence.
Figure 4: Specimen of the completely excised tumor

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Figure 5: Repair of the abdominal wall defect with the physicmesh

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  Discussion Top


Desmoid tumor, also known as aggressive fibromatosis, is a rare soft-tissue tumor. For those cases localized in the anterior abdominal wall, radical resection and reconstruction with a mesh is indicated. Because of the rarity of the disease, randomized trials are lacking, but in reported retrospective series, it is clear that although it is considered a benign lesion, local recurrence is not uncommon. [6]

Desmoid tumor is often associated with female gender, familial adenomatous polyposis [7] and occasionally with surgical trauma. [8] It has a higher prevalence in women who experienced pregnancy. [9] Depending upon the tumor size, on the therapy and on the negative resection margins, recurrence occurs in up to 45%. [10]

CT scan localizes the tumor and excludes metastasis. MRI reveals the tumor's hypo-intensity on T1 and demonstrates variable signal intensity on T2 weighted imaging, depending upon the accumulation of mucoid material. Therefore, differentiation from other solid tumors is impossible using the morphological criteria. Histology is the only evidentiary method, which demonstrates long fascicles of spindle cells of variable cell-density with few mitoses and absence of atypical nucleus-separations. [11]

The therapy of choice is still controversial [12] anti-inflammatory treatment, [13] hormone-therapy [14] and chemotherapy [15] were not shown to be effective. These therapies are limited to patients, in whom resection is technically impossible because of a widespread tumor infiltration. The effectiveness and indication of initial and adjuvant radiation is not proven yet.

Surgery always aims at radical tumor resection with free margins, which depending upon localization of surgery, may leave major soft-tissue defects behind. [16],[17] Although abdominal wall integrity after full-thickness surgery can be restored with direct sutures, [18] reconstruction with synthetic materials is a common technique in major abdominal wall defects as performed in our patient. [19]


  Conclusion Top


Desmoid tumors in females are often associated with pregnancy or occur post-partum. The most common sites are in the abdominal muscles, but they have been reported in other areas of the body. Surgical resection is the treatment of choice and is usually curative. Radical resection with clear margins remains the principal determinant of outcome. Our patient had no risk factors for the development of desmoid tumor and in spite of having all the bad prognostic factors; she had a recurrence free follow-up at 18 months. We concluded that more research needs to be carried out to search for other risk factors or prognostic factors in desmoid tumors.

 
  References Top

1.Mulik V, Griffiths AN, Beattie RB. Desmoid tumours with familial adenomatous polyposis in pregnancy. J Obstet Gynaecol 2003;23:307-8.  Back to cited text no. 1
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2.Sinha A, Gibbons DC, Phillips RK, Clark S. Surgical prophylaxis in familial adenomatous polyposis: Do pre-existing desmoids outside the abdominal cavity matter? Fam Cancer 2010;9:407-11.  Back to cited text no. 2
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3.Galeotti F, Facci E, Bianchini E. Desmoid tumour involving the abdominal rectus muscle: Report of a case. Hernia 2006;10:278-81.  Back to cited text no. 3
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4.Salas S, Dufresne A, Bui B, Blay JY, Terrier P, Ranchere-Vince D, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: A wait-and-see policy according to tumor presentation. J Clin Oncol 2011;29:3553-8.  Back to cited text no. 4
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5.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. 5
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6.Bertani E, Chiappa A, Testori A, Mazzarol G, Biffi R, Martella S, et al. Desmoid tumors of the anterior abdominal wall: Results from a monocentric surgical experience and review of the literature. Ann Surg Oncol 2009;16:1642-9.  Back to cited text no. 6
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7.Soravia C, Berk T, McLeod RS, Cohen Z. Desmoid disease in patients with familial adenomatous polyposis. Dis Colon Rectum 2000;43:363-9.  Back to cited text no. 7
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8.De Cian F, Delay E, Rudigoz RC, Ranchère D, Rivoire M. Desmoid tumor arising in a cesarean section scar during pregnancy: Monitoring and management. Gynecol Oncol 1999;75:145-8.  Back to cited text no. 8
    
9.Gansar GF, Markowitz IP, Cerise EJ. Thirty years of experience with desmoid tumors at Charity Hospital. Am Surg 1987;53:318-9.  Back to cited text no. 9
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10.Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3 rd . Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articles. Cancer 2000;88:1517-23.  Back to cited text no. 10
    
11.Casillas J, Sais GJ, Greve JL, Iparraguirre MC, Morillo G. Imaging of intra- and extra-abdominal desmoid tumors. Radiographics 1991;11:959-68.  Back to cited text no. 11
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12.Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol 2001;27:701-6.  Back to cited text no. 12
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13.Waddell WR, Kirsch WM. Testolactone, sulindac, warfarin, and vitamin K1 for unresectable desmoid tumors. Am J Surg 1991;161:416-21.  Back to cited text no. 13
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14.Wilcken N, Tattersall MH. Endocrine therapy for desmoid tumors. Cancer 1991;68:1384-8.  Back to cited text no. 14
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15.Patel SR, Evans HL, Benjamin RS. Combination chemotherapy in adult desmoid tumors. Cancer 1993;72:3244-7.  Back to cited text no. 15
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16.Brenner P, Rammelt S. Abdominal wall and foot reconstruction after extensive desmoid tumor resection with free tissue transfer. Langenbecks Arch Surg 2002;386:592-7.  Back to cited text no. 16
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17.Sutton RJ, Thomas JM. Desmoid tumours of the anterior abdominal wall. Eur J Surg Oncol 1999;25:398-400.  Back to cited text no. 17
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18.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. 18
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19.Bauer JJ, Salky BA, Gelernt IM, Kreel I. Repair of large abdominal wall defects with expanded polytetrafluoroethylene (PTFE). Ann Surg 1987;206:765-9.  Back to cited text no. 19
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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