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 Table of Contents  
REVIEW ARTICLE
Year : 2016  |  Volume : 2  |  Issue : 2  |  Page : 127-131

Resection of a large intra-abdominal desmoid tumor requiring abdominal wall reconstruction: A case report and review of literature


Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA

Date of Submission23-Mar-2016
Date of Acceptance10-May-2016
Date of Web Publication28-Dec-2016

Correspondence Address:
David S Strosberg
395 W. 12th Avenue, Suite 654, Columbus, OH 43210
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2455-5568.196869

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  Abstract 


Abdominal desmoid tumors (ADTs) are rare, fast-growing, nonmalignant tumors of the soft tissue. ADTs have no known metastatic potential; however, they are locally aggressive and may result in local tissue destruction. The diagnosis of an ADT should be considered in a female patient with a prior history of pregnancy and previous abdominal surgery who presents with a painless intra-abdominal mass. We present the case of a 23 cm intra-ADT excision in a 40-year-old African American female requiring abdominal wall reconstruction with component separation. We also discuss a comprehensive review of the literature including current treatment methods and prognostic outcomes in patients following the resection of ADTs. An individualized multi-modality treatment approach employing both surgical and medical therapy to achieve histologically negative margins appears to be the most effective treatment strategy to reduce the recurrence rate of ADTs.
The following core competencies are addressed in this article: Patient care and medical knowledge.

Keywords: Component separation, desmoid tumor, fibromatosis, soft tissue sarcoma, Wnt/beta-catenin


How to cite this article:
Chen JZ, Strosberg DS, Dettorre R, Mikami D. Resection of a large intra-abdominal desmoid tumor requiring abdominal wall reconstruction: A case report and review of literature. Int J Acad Med 2016;2:127-31

How to cite this URL:
Chen JZ, Strosberg DS, Dettorre R, Mikami D. Resection of a large intra-abdominal desmoid tumor requiring abdominal wall reconstruction: A case report and review of literature. Int J Acad Med [serial online] 2016 [cited 2019 Jul 23];2:127-31. Available from: http://www.ijam-web.org/text.asp?2016/2/2/127/196869




  Introduction Top


Abdominal desmoid tumors (ADTs) are rare, fast-growing, nonmalignant tumors of the soft tissue. ADTs have no known metastatic potential; however, they are locally aggressive and may result in local tissue destruction. We present the case of a 23 cm intra-ADT excision in a 40-year-old African American female requiring abdominal wall reconstruction with component separation, and a comprehensive review of the literature including current treatment methods and prognostic outcomes in patients following the resection of ADTs.


  Case Presentation Top


A 40-year-old African American female with a prior surgical history of a cesarean section and uterine fibroid removal presented to the outpatient clinic complaining of a painless abdominal wall mass. The patient denied obstructive symptoms. Computed tomography of the abdomen demonstrated a soft tissue mass measuring 18 cm × 14 cm × 16 cm [Figure 1], with biopsy suggestive of desmoid pathology. The decision was made for surgical excision.
Figure 1: Computed tomography of intra-abdominal desmoid tumor

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A midline vertical incision was made from just below the umbilicus to the pubic tubercle. Upon entering the abdomen, a large mass was encountered which encompassed the majority of the abdomen [Figure 2]. The right portion of the mass was noted to be infiltrating the anterior abdominal wall, and the right rectus muscle and the inferior portion was firmly attached to the uterus. The mass was circumferentially dissected and freed from all peritoneal attachments and the uterus. Methylene blue did not identify any intraoperative ureteral or bladder injuries. The final specimen was 23 cm in diameter [Figure 3].
Figure 2: Intraoperative excision of the demoid tumor

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Figure 3: Final excision of the desmoid tumor, measuring 23 cm × 17 cm × 15 cm

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As a portion of the patient's fascia was excised with the specimen, a separation of components was performed to reconstruct her abdominal wall with a tension-free repair. The fascia the patient's left side was reapproximated to the posterior fascial layer and the peritoneum on the patient's right side. No prosthetic or biologic mesh was required for abdominal wall reconstruction. Before leaving the OR, the pathologist confirmed that microscopically negative margins were achieved.

The postoperative course was uneventful, and the patient was discharged on the 4th day. The patient was seen in clinic and reported no further complaints. The patient had no evidence of recurrence at 9 months follow-up.


  Discussion Top


Incidence and presentation

ADTs are rare, fast-growing, nonmalignant tumors originating from soft tissue of the musculoaponeurosis.[1] In general, they account for 0.03% of all neoplasms and 3% of all soft tissue tumors.[2] They are more common among females and generally occur in patients between the ages of 15 and 60.[3] There is a close association between the prevalence of ADTs and increased estrogen states such as occur with pregnancy.[4] Pregnant women and women who have a prior history of pregnancy appear to be at increased risk of developing ADTs.[5] ADTs tends to be fast-growing in women of childbearing age. Reitamo et al. reported the growth rate of ADTs to be twice as rapid in fertile women and more than 4 times as high in middle-aged women compared to younger girls.[6]

ADTs most commonly present as an asymptomatic mass, and occasionally may present with pain and weight loss.[7] Although ADTs lack the ability to dedifferentiate or metastasize to distal organ sites, their rapid growth rate may cause local compressive effects including intestinal obstruction, bowel ischemia, and small intestinal perforation.[8],[9]

Pathogenesis

Correlation between aberrant beta-catenin expression and vascular endothelial growth factor overexpression appears to play an important role in the neoplastic progression of sporadic ADTs.[10] Tumor recurrence appears to be associated with a history of prior surgical trauma. This is believed to be due to aberrations in the expression of the molecular signaling pathway Wnt/beta-catenin, which regulates myofibroblast proliferation.[11],[12] Retrospective studies indicate that as many as 30–40% of patients will experience a recurrence of locally aggressive ADTs after the initial surgery.[13] Cases have been reported of ADTs appearing in surgical scars following a cesarean section.[14] This supports the theory that inherent abnormalities in myofibroblast growth regulation during the wound healing process is an underlying cause of sporadic ADTs.[15],[16] Estrogen predominance also increases predisposition to occurrences of ADT; this suggests that hormonal estrogen, trauma, and pregnancy are all contributory factors toward the development of sporadic ADTs.[17]

Staging

No consensus exists for staging intra-abdominal ADTs. Church et al. proposed a staging technique based on tumor size, growth rate, and clinical presentation at the Cleveland Clinic [Table 1].[18] Such staging techniques would separate patients into clinically useful groups who would benefit from surgical treatment and medical therapy. Quintini et al. proposed a prognostic stratification system for familial adenomatous polyposis-associated ADTs based on tumor size (maximum diameter), the severity of pain (based on the need for narcotic pain medication), and total parenteral nutrition requirement. Five years survival rate was found to be 95% for Stage I, 100% for Stage II, 89% for Stage III, and 76% for Stage IV ADT.[19]
Table 1: Staging system for desmoid tumors

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While asymptomatic and moderately-sized tumors (Stage I–III) were more common among the 101 patients based on the Cleveland Clinic's categorical staging technique, patients with large symptomatic tumors benefit more from medical intervention.[20]

Treatment

Surgery

Surgical resection with the aim of achieving negative histological margins remains the first-line therapy in the treatment of fast-growing symptomatic ADTs.[21] In large ADTs, adequate surgical resection may require extensive abdominal wall reconstruction to prevent abdominal wall hernias. Abdominal wall integrity can often be restored with primary closure. However, abdominal wall reconstruction with synthetic or biologic materials and/or the use of free muscle flaps may be required for major abdominal wall defects.[22] Bauer et al. reported on the use of polytetrafluoroethylene (PTFE) prosthetic patches to repair abdominal wall incisional hernias. Among patients who required abdominal wall closures, 43% of these patients had a prior primary repair failure. However, following abdominal wall closure with PTFE patching, recurrent herniation occurred in only 10.7% of patients.[23] Conversely, the use of autologous fascia has several considerable advantages over the use of synthetic prosthetic patches, namely reduced presence of wound contamination. Autologous fascia can be revascularized and incorporated as living tissue, hence, the lower rate of wound infections as compared to expanded PTFE.[24]

Chemotherapy

Systemic therapy with chemo pharmaceuticals has been shown to stabilize tumor growth, and in certain cases obviate the need for surgery. Francis et al. reported on 52 patients who were treated with neoadjuvant protocols. Of the patients who had resectable tumors, tamoxifen and Celebrex obviated surgery in 30% of cases while overall only 13% of patients developed a recurrence. This treatment plan demonstrated excellent local control and obviates the need for aggressive surgical management [Figure 4].[25]
Figure 4: Algorithm for treatment of unresectable desmoid tumors (adapted from Francis et al.)

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Radiotherapy

Radiation therapy is an effective therapeutic treatment option in patients who are not ideal surgical candidates, and for obtaining local control of tumor growth in patients with recurrent ADTs.[26] Acker et al. reviewed the treatment of forty patients with histologically confirmed ADTs, and found that doses in the range of 50–55 Gy offer the greatest chance of local control, equal to that obtained with higher doses as reported previously.[27],[28],[29]

Multimodal therapy

In spite of the best medical and surgical therapy, recurrence rates for ADTs have been shown to be between 20% and 30% with a low risk of mortality. Spear et al. reported on the 5-year outcome examining 107 patients with fibromatosis. Surgery alone was used to treat 51 tumors, radiation alone was used to treat 15 tumors and surgery and radiation was used concomitantly to treat 41 tumors. Surgical treatment alone resulted in a 5 years actuarial control rate of 69%. Radiation alone resulted in 5 years actuarial control rate of 93%. Surgical and radiation resulted in a 5 years actuarial control rate of 72%. In a multivariate analysis, margin status (positive vs. negative) was a significant predictor for failure rate. Radiation is highly recommended in situ ations wherein negative margins are not achieved surgically and also in cases of tumor recurrences.[30]

Huang et al. examined a total of 151 patients with ADTs, who underwent treatment with surgical resection over a 20 years period. Of those patients, 31 (20.5%) developed local tumor recurrence, however, no patient deaths were directly attributable to the desmoid tumor (DT).[31] Salas et al. performed a retrospective review of 426 patients who were treated for sporadic ADTs to evaluate outcomes following surgical and radiation therapy. The absence of residual tumor after local treatment was observed in 323 (75.8%) of patients.[13] Ballo et al. reported on the therapeutic value of surgical resection in combination with adjuvant therapy for the treatment of ADTs. Of the 189 patients who were treated with surgical resection alone or surgical resection in combination with radiation therapy, recurrence occurred in 58 patients (31%). More than 90% of those patients experienced a recurrence within 5 years while only four patients (7%) developed recurrence of DT after 5 years; meanwhile, no deaths were directly attributable to the DT in this series study.[32]

Kotiligam et al. conducted a prospective study on 189 patients at the MD Anderson Cancer Center comparing the efficacy of surgery along and in combination with systemic chemotherapy and radiation therapy. Patients were stratified based on marginal pathology, macroscopic residual disease, and local infiltration. Kotiligam et al. reported improved outcomes with the individualized multi-modal approach, reporting a 5-year recurrence rate of 20% compared to 30% for a prior control group.[33]


  Conclusion Top


The diagnosis of a DT should be strongly considered in female patients between the ages of 15 and 60 with a prior history of pregnancy and abdominal surgery. Recurrence following surgical treatment and medical therapy is common and up to 90% of recurrence occurs within 5 years. No consensus exists for staging of intra-abdominal ADTs. An individualized multi-modality treatment approach employing both surgical and medical therapy to achieve histologically negative margins appears to be the most effective treatment strategy to reduce the recurrence rate of ADTs.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Shih LY, Wei CK, Lin CW, Tseng CE. Postoperative retroperitoneal desmoid tumor mimics recurrent gastrointestinal stromal tumor: A case report. World J Gastroenterol 2012;18:6172-6.  Back to cited text no. 1
    
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Church JM. Mucosal ischemia caused by desmoid tumors in patients with familial adenomatous polyposis: Report of four cases. Dis Colon Rectum 1998;41:661-3.  Back to cited text no. 8
    
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Salas S, Chibon F, Noguchi T, Terrier P, Ranchere-Vince D, Lagarde P, et al. Molecular characterization by array comparative genomic hybridization and DNA sequencing of 194 desmoid tumors. Genes Chromosomes Cancer 2010;49:560-8.  Back to cited text no. 11
    
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Tejpar S, Michils G, Denys H, Van Dam K, Nik SA, Jadidizadeh A, et al. Analysis of Wnt/Beta catenin signalling in desmoid tumors. Acta Gastroenterol Belg 2005;68:5-9.  Back to cited text no. 12
    
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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. 13
    
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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. 14
    
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Kulaylat MN, Karakousis CP, Keaney CM, McCorvey D, Bem J, Ambrus JL Sr. Desmoid tumour: A pleomorphic lesion. Eur J Surg Oncol 1999;25:487-97.  Back to cited text no. 15
    
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Cheon SS, Cheah AY, Turley S, Nadesan P, Poon R, Clevers H, et al. Beta-catenin stabilization dysregulates mesenchymal cell proliferation, motility, and invasiveness and causes aggressive fibromatosis and hyperplastic cutaneous wounds. Proc Natl Acad Sci U S A 2002;99:6973-8.  Back to cited text no. 16
    
17.
Häyry P, Reitamo JJ, Tötterman S, Hopfner-Hallikainen D, Sivula A. The desmoid tumor. II. Analysis of factors possibly contributing to the etiology and growth behavior. Am J Clin Pathol 1982;77:674-80.  Back to cited text no. 17
    
18.
Church J, Berk T, Boman BM, Guillem J, Lynch C, Lynch P, et al. Staging intra-abdominal desmoid tumors in familial adenomatous polyposis: A search for a uniform approach to a troubling disease. Dis Colon Rectum 2005;48:1528-34.  Back to cited text no. 18
    
19.
Quintini C, Ward G, Shatnawei A, Xhaja X, Hashimoto K, Steiger E, et al. Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: A single center review of 154 patients. Ann Surg 2012;255:511-6.  Back to cited text no. 19
    
20.
Church J, Lynch C, Neary P, LaGuardia L, Elayi E. A desmoid tumor-staging system separates patients with intra-abdominal, familial adenomatous polyposis-associated desmoid disease by behavior and prognosis. Dis Colon Rectum 2008;51:897-901.  Back to cited text no. 20
    
21.
Shido Y, Nishida Y, Nakashima H, Katagiri H, Sugiura H, Yamada Y, et al. Surgical treatment for local control of extremity and trunk desmoid tumors. Arch Orthop Trauma Surg 2009;129:929-33.  Back to cited text no. 21
    
22.
Overhaus M, Decker P, Fischer HP, Textor HJ, Hirner A. Desmoid tumors of the abdominal wall: A case report. World J Surg Oncol 2003;1:11.  Back to cited text no. 22
    
23.
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. 23
    
24.
Disa JJ, Chiaramonte MF, Girotto JA, Klein MH, Goldberg NH. Advantages of autologous fascia versus synthetic patch abdominal reconstruction in experimental animal defects. Plast Reconstr Surg 2001;108:2086-7.  Back to cited text no. 24
    
25.
Francis WP, Zippel D, Mack LA, DiFrancesco LM, Kurien E, Schachar NS, et al. Desmoids: A revelation in biology and treatment. Ann Surg Oncol 2009;16:1650-4.  Back to cited text no. 25
    
26.
de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F. Desmoid tumors: Need for an individualized approach. Expert Rev Anticancer Ther 2009;9:525-35.  Back to cited text no. 26
    
27.
Acker JC, Bossen EH, Halperin EC. The management of desmoid tumors. Int J Radiat Oncol Biol Phys 1993;26:851-8.  Back to cited text no. 27
    
28.
Biasco G, Pantaleo MA, Nobili E, Monti C. Spontaneous regression of a desmoid intraabdominal tumor in a patient affected by familial adenomatous polyposis. Am J Gastroenterol 2004;99:1621-2.  Back to cited text no. 28
    
29.
Miralbell R, Suit HD, Mankin HJ, Zuckerberg LR, Stracher MA, Rosenberg AE. Fibromatoses: From postsurgical surveillance to combined surgery and radiation therapy. Int J Radiat Oncol Biol Phys 1990;18:535-40.  Back to cited text no. 29
    
30.
Spear MA, Jennings LC, Mankin HJ, Spiro IJ, Springfield DS, Gebhardt MC, et al. Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys 1998;40:637-45.  Back to cited text no. 30
    
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Huang K, Fu H, Shi YQ, Zhou Y, Du CY. Prognostic factors for extra-abdominal and abdominal wall desmoids: A 20-year experience at a single institution. J Surg Oncol 2009;100:563-9.  Back to cited text no. 31
    
32.
Ballo MT, Zagars GK, Pollack A, Pisters PW, Pollack RA. Desmoid tumor: Prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol 1999;17:158-67.  Back to cited text no. 32
    
33.
Kotiligam D, Lazar AJ, Pollock RE, Lev D. Desmoid tumor: A disease opportune for molecular insights. Histol Histopathol 2008;23:117-26.  Back to cited text no. 33
    


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