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Gigantic recurrent abdominal desmoid tumour: a case report

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Abstract

Deeply seated fibromatosis or desmoid tumour (DT) is a rare entity characterized by benign proliferation of fibroblasts. Although non-malignant, this tumour can be life-threatening due to its invasive property and high recurrence rate. We report a unique case of a huge recurrent abdominal DT (36 cm in diameter, 25 kg in weight) that caused pressure necrosis and sloughing of the overlying anterior abdominal wall, and produced a large fungating mass protruding outside the abdomen. Although preoperative neoadjuvant therapies were all ineffective, radical surgical removal of the tumour was successful. The procedure was followed by an excellent clinical recovery and the patient is still alive with no evidence of recurrent disease after a 6-year follow-up. We recommend surgical removal of DT regardless of its size and in particular in cases where there is no or inadequate response to neoadjuvant therapy.

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References

  1. Alman BA, Pajerski ME, Diaz-Cano S, Corboy K, Wolfe HJ (1997) Aggressive fibromatosis (desmoid tumor) is a monoclonal disorder. Diagn Mol Pathol 6:98–101

    Article  PubMed  CAS  Google Scholar 

  2. Cormio G, Cormio L, Marzullo A, Pansini F, Traficante A, Selvaggi FP, Selvaggi L (1997) Fibromatosis of the female pelvis. Ann Chir Gynaecol 86:84–86

    PubMed  CAS  Google Scholar 

  3. Knudsen AL, Bulow S (2001) Desmoid tumour in familial adenomatous polyposis. A review of literature. Fam Cancer 1:111–119

    Article  PubMed  CAS  Google Scholar 

  4. Reitamo JJ, Scheinin TM, Hayry P (1986) The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 151:230–237

    Article  PubMed  CAS  Google Scholar 

  5. Rodriguez-Bigas MA, Mahoney MC, Karakousis CP, Petrelli NJ (1994) Desmoid tumors in patients with familial adenomatous polyposis. Cancer 74:1270–1274

    Article  PubMed  CAS  Google Scholar 

  6. Nursal TZ, Abbasoglu O (2003) Sporadic hereditary pancreatic desmoid tumor: a new entity? J Clin Gastroenterol 37:186–188

    Article  PubMed  Google Scholar 

  7. Ferenc T, Sygut J, Kopczynski J, Mayer M, Latos-Bielenska A, Dziki A, Kulig A (2006) Aggressive fibromatosis (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background. Pol J Pathol 57:5–15

    PubMed  Google Scholar 

  8. Kulaylat MN, Karakousis CP, Keaney CM, McCorvey D, Bem J, Ambrus JL Sr (1999) Desmoid tumour: a pleomorphic lesion. Eur J Surg Oncol 25:487–497

    Article  PubMed  CAS  Google Scholar 

  9. Gwynne-Jones DP, Theis JC, Jeffery AK, Hung NA (2005) Long-term follow-up of a recurrent multifocal desmoid tumour treated with tamoxifen: a case report. J Orthop Surg (Hong Kong) 13:174–177

    CAS  Google Scholar 

  10. Chao AS, Lai CH, Hsueh S, Chen CS, Yang YC, Soong YK (2000) Successful treatment of recurrent pelvic desmoid tumour with tamoxifen: case report. Hum Reprod 15:311–313

    Article  PubMed  CAS  Google Scholar 

  11. Seoud M, Abbas J, Kaspar H, Khalil A, Geara F (2005) Long-term survival following aggressive surgery and radiotherapy for pelvic fibromatosis. Int J Gynecol Cancer 15:1112–1114

    Article  PubMed  CAS  Google Scholar 

  12. Poritz LS, Blackstein M, Berk T, Gallinger S, McLeod RS, Cohen Z (2001) Extended follow-up of patients treated with cytotoxic chemotherapy for intra-abdominal desmoid tumors. Dis Colon Rectum 44:1268–1273

    Article  PubMed  CAS  Google Scholar 

  13. Weiss SW, Goldblum JR (2001) Fibromatoses. In: Strauss M (ed) Soft tissue tumors. Mosby, St. Louis, pp 309–346

    Google Scholar 

  14. Hayry P, Reitamo JJ, Totterman S, Hopfner-Hallikainen D, Sivula A (1982) The desmoid tumor. II. Analysis of factors possibly contributing to the etiology and growth behavior. Am J Clin Pathol 77:674–680

    PubMed  CAS  Google Scholar 

  15. Reitamo JJ (1983) The desmoid tumor. IV. Choice of treatment, results, and complications. Arch Surg 118:1318–1322

    PubMed  CAS  Google Scholar 

  16. Lasser P, Elias D, Contesso G, Genin J, Mankarios H, Rougier P (1993) Desmoid tumors or intra-abdominal fibromatoses. Ann Chir 47:352–359

    PubMed  CAS  Google Scholar 

  17. Khorsand J, Karakousis CP (1985) Desmoid tumors and their management. Am J Surg 149:215–218

    Article  PubMed  CAS  Google Scholar 

  18. Schnitzler M, Cohen Z, Blackstein M, Berk T, Gallinger S, Madlensky L, McLeod R (1997) Chemotherapy for desmoid tumors in association with familial adenomatous polyposis. Dis Colon Rectum 40:798–801

    Article  PubMed  CAS  Google Scholar 

  19. Spear MA, Jennings LC, Mankin HJ, Spiro IJ, Springfield DS, Gebhardt MC, Rosenberg AE, Efird JT, Suit HD (1998) Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys 40:637–645

    Article  PubMed  CAS  Google Scholar 

  20. Goy BW, Lee SP, Eilber F, Dorey F, Eckardt J, Fu YS, Juillard GJ, Selch M T (1997) The role of adjuvant radiotherapy in the treatment of resectable desmoid tumors. Int J Radiat Oncol Biol Phys 39:659–665

    Article  PubMed  CAS  Google Scholar 

  21. Hamilton L, Blackstein M, Berk T, McLeod RS, Gallinger S, Madlensky L, Cohen Z (1996) Chemotherapy for desmoid tumours in association with familial adenomatous polyposis: a report of three cases. Can J Surg 39:247–252

    PubMed  CAS  Google Scholar 

  22. Easter DW, Halasz NA (1989) Recent trends in the management of desmoid tumors. Summary of 19 cases and review of the literature. Ann Surg 210:765–769

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Histopathology, Faculty of Medicine, Menoufia University, Shebin El-Kom, Egypt

    E. A. Rakha & M. A. Kandil

  2. Department of Surgery, Faculty of Medicine, Menoufia University, Shebin El-Kom, Egypt

    M. G. El-Santawe

Authors
  1. E. A. Rakha
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  2. M. A. Kandil
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  3. M. G. El-Santawe
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Correspondence to E. A. Rakha.

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Rakha, E.A., Kandil, M.A. & El-Santawe, M.G. Gigantic recurrent abdominal desmoid tumour: a case report. Hernia 11, 193–197 (2007). https://doi.org/10.1007/s10029-006-0165-4

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  • DOI: https://doi.org/10.1007/s10029-006-0165-4

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