Journal of Surgery (ISSN: 2575-9760)

case report

Massive Abdominal Wall Desmoid Tumor Following Deep Dorsal Vein Arterialization: A Case Report

Soroush Shahrokh1, Javad Nickbakht2, Housain Shahrokh2, Mark Reza Laftavi3*

1Rowan University School of Osteopathic Medicine, New Jersey, USA.

2Iran University of Medical Sciences School of Medicine, Tehran, Iran

3State University of New York at Upstate, Syracuse, New York, USA

*Corresponding author: Mark Reza Laftavi, State University of New York at Upstate, Syracuse, New York USA. Tel: +1-3154647329; Email: laftavim@upstate.edu

Received Date: 24 January, 2019; Accepted Date: 06 March, 2019; Published Date: 11 March, 2019

Abstract

Desmoid Tumor (DT), also called aggressive fibromatosis, is a non-malignant tumor of the mesenchymal stem cells. Although DTs are not malignant and do not metastasize, they are locally aggressive tumors and are hence referred to as a “benign malignancy.” Due to the variable natural history of DTs, there is no standard treatment modalities. However, surgical resection with microscopic negative margins (R0) is considered the mainstay therapy for a DT.

There is scarce literature discussing this rare tumor. Here, we report a case of a 30-year-old male patient who presented with an extremely large abdominal wall DT, with the size of 15x16x20 cm. The tumor had developed beneath the incision site of previous surgery. The tumor was removed with microscopic negative surgical margins. In a one-year follow-up, patient had no complaints and there were no signs of tumor recurrence. His MRI did not show regrowth of the tumor.


Figure 1: CT-scan of the patient’s abdominal DT. CT scan was performed prior to surgery with and without contrast. The CT scan shows the presence of a large abdominal tumor. There is a 154x118 mm tumor soft tissue mass lesion on the left side of the abdominal wall with subtle enhancement. The differential diagnosis based on the CT scan was a solitary fibroma tumor, abdominal fibromatosis or soft tissue sarcoma.



Figure 2: The patient’s abdominal mass immediately prior to surgical resection and two weeks after surgery. The DT, due to its large size, was causing cosmetic concerns and difficulty sleeping for the patient.



Figure 3: The raw DT that was recovered from the patient immediately following surgery. The DT was extremely large, with the size of 15x112x8 cm, one of the largest cases of abdominal fibromatosis reported in the literature.



Figure 4: The histologic sample biopsy. The microscopic examination of the biopsy shows “neoplastic component with a proliferation of benign-looking spindle fibers with a fascicular architecture”. The cells show a vesicular nucleus with inconspicuous nucleoli and eosinophilic cytoplasm separated by bundles of collagen. No mitotic figures or necrosis were present in the microscopic exam of the biopsy slides. According to the presentation and the microscopic features of the biopsy, the final diagnosis was made to be a DT.


  1. Kasper B1, Ströbel P, Hohenberger P (2011) Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist 16: 682-693.
  2. Howard JH, Pollock RE (2016) Intra-Abdominal and Abdominal Wall Desmoid Fibromatosis. Oncol Ther 4: 57-72.
  3. Li J, Wang CY (2008) TBL1-TBLR1 and beta-catenin recruit each other to Want target-gene promoter for transcription activation and oncogenesis. Nat Cell Biol 10: 160-169.
  4. de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F (2009) Desmoid tumors: need for an individualized approach. Expert Rev Anticancer Ther 9: 525-535.
  5. Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan MF (1999) The Enigma of Desmoid Tumors. Annals of Surgery 229: 866-873.
  6. Hosalkar HS, Torbert JT, Fox EJ, Delaney TF, Aboulafia AJ, et al. (2000) Musculoskeletal desmoid tumors. J Am Acad Orthop Surg 16: 188-198.
  7. Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd (2000) Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articles. Cancer 88: 1517-1523.
  8. Lazar AJ, Tuvin D, Hajibashi S, Habeeb S, Bolshakov S, et al. (2008) Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol 173: 1518-1527.
  9. Wu C, Amini-Nik S, Nadesan P, Stanford WL, Alman BA (2010) Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells. Cancer Res 70: 7690-7698.
  10. Amary MF, Pauwels P, Meulemans E, Roemen GM, Islam L, et al. (2007) Detection of beta-catenin mutations in paraffin-embedded sporadic desmoid-type fibromatosis by mutation-specific restriction enzyme digestion (MSRED): an ancillary diagnostic tool. Am J Surg Pathol 31: 1299-1309.
  11. Alman BA, Li C, Pajerski ME, Diaz-Cano S, Wolfe HJ (1997) Increased beta-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors). The American Journal of Pathology 151: 329-334.
  12. Tejpar S, Nollet F, Li C, Wunder JS, Michils G, et al. (1999) Predominance of beta-catenin mutations and beta-catenin dysregulation in sporadic aggressive fibromatosis (desmoid tumor). Oncogene 18: 6615-6620.
  13. Carothers AM, Rizvi H, Hasson RM, Heit YI, Davids JS, et al. (2012) Mesenchymal stromal cell mutations and wound healing contribute to the etiology of desmoid tumors. Cancer Res 72: 346-355.
  14. Cheon SS, Cheah AY, Turley S, Nadesan P, Poon R, et al. (2002) β-Catenin stabilization dysregulates mesenchymal cell proliferation, motility, and invasiveness and causes aggressive fibromatosis and hyperplastic cutaneous wounds. Proceedings of the National Academy of Sciences of the United States of America 99: 6973-6978.
  15. Eastley NC, Hennig IM, Esler CP, Ashford RU (2015) Nationwide trends in the current management of desmoid (aggressive) fibromatosis. Clin Oncol (R Coll Radiol) 27: 362-368.
  16. Salas S, Dufresne A, Bui B, Blay JY, Terrier P, et al. (2011) 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 29: 3553-3558.
  17. Huang K, Wang CM, Chen JG, Du CY, Zhou Y, et al. (2014) Prognostic factors influencing event-free survival and treatments in desmoid-type fibromatosis: analysis from a large institution. Am J Surg 207: 847-854.
  18. Janssen ML, van Broekhoven DL, Cates JM, Bramer WM, Nuyttens JJ, et al. (2017) Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis. Br J Surg 104: 347-357.
  19. McCrea PD, Turck CW, Gumbiner B (1991) A homolog of the armadillo protein in Drosophila (plakoglobin) associated with E-cadherin. Science 254: 1359-1361.
  20. Hegde MR, Roa BB (2006) Detecting mutations in the APC gene in familial adenomatous polyposis (FAP). Curr Protoc Hum Genet 2006.
  21. Singer AJ, Clark RA (1999) Cutaneous Wound Healing. The New England Journal of Medicine 341: 738-746.
  22. Wanjeri JK, Opeya CJ (2011) A massive abdominal wall desmoid tumor occurring in a laparotomy scar: a case report. World J Surg Oncol 9: 35.
  23. Waller A, Findeis S, Lee MJ (2016) Familial Adenomatous Polyposis. J Pediatr Genet 5: 78-83.
  24. Gounder MM, Lefkowitz RA, Keohan ML, D'Adamo DR, Hameed M, et al. (2011) Activity of Sorafenib against desmoid tumor/deep fibromatosis. Clin Cancer Res 17: 4082-4090.
  25. Penel N, Chibon F, Salas S (2017) Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol 29: 268-274.
  26. Harati K, Jaenisch A, Behr B, Goertz O, Harati A, et al. (2017) Effect of surgical margins on prognosis in aggressive fibromatosis: A single-institutional analysis of 90 patients. Oncol Lett 14: 5129-5134.
  27. Wilkinson MJ, Chan KE, Hayes AJ, Strauss DC (2014) Surgical outcomes following resection for sporadic abdominal wall fibromatosis. Ann Surg Oncol 21: 2144-2149.