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Zentralbl Chir 2010; 135(1): 79-82
DOI: 10.1055/s-2008-1076884
Kasuistik

© Georg Thieme Verlag Stuttgart ˙ New York

Sporadic Retroperitoneal Aggressive Fibromatosis: Report of a Case

Sporadische retroperitoneale aggressive Fibromatose: FallberichtG. Ghidirim1 , I. Mishin1 , I. Gagauz1 , M. Vozian1 , G. Zastavnitsky1 , I. Iakovleva1
  • 1First Department of Surgery “N. Anestiadi” and Laboratory of Hepato-Pancreato-Biliary Surgery, Medical University “N. Testemitsanu”, Kishinev, Moldova
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Publication History

Publication Date:
07 May 2009 (online)

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Abstract

We describe herein a case of sporadic retroperitoneal aggressive fibromatosis. A 54-year-old man with a palpable abdominal mass was referred to our hospital for investigation and treatment. Abdominal ultrasonography and computed tomography revealed a solid mass with relatively well-defined borders in the left abdominal retroperitoneum. At surgery, a large tumor (14 × 13 × 11 cm) was found, arising from the retroperitoneal space and involving the wall of jejunum. Complete removal was achieved. Histological examination of the resected specimen revealed the presence of changes consistent with aggressive fibromatosis. The patient was not scheduled for any further treatment. Two years after surgery, the patient is without any signs of recurrent disease. Although extremely rare, aggressive fibromatosis should be considered in the differential diagnosis of retroperitoneal masses. Complete surgical resection with negative pathological margins remains the first line management of these neoplasms. Careful follow-up is indicated because recurrence may occur every time after surgery. 

Zusammenfassung

Wir beschreiben einen Fall von sporadischer retroperitonealer aggressiver Fibromatose. Ein 54-jähriger Mann wurde uns mit einem tastbaren Abdominaltumor zur Abklärung und Behandlung zugewiesen. Bei der Ultraschalluntersuchung und im CT fand sich eine solide Masse mit relativ gut definierten Grenzen im linken Retroperitoneum. Bei der Operation zeigte sich ein großer Tumor (14 × 13 × 11 cm), der vom Retroperitoneum ausging und die Jejunalwand infiltriert hatte. Der ­Tumor wurde komplett entfernt. Die histologische Untersuchung ergab Veränderungen, die mit einer aggressiven Fibromatose vereinbar waren. Eine weitere Therapie wurde nicht ange­schlossen. Zwei Jahre nach der Operation ist der Patient ohne Anzeichen für ein Rezidiv. Obwohl extrem selten sollte die aggressive Fibromatose in die Differenzialdiagnose eines retroperitonealen Tumors eingeschlossen werden. Therapie der Wahl ist die komplette chirurgische Resektion mit negativen pathologischen Grenzen (R 0-Resektion). Eine sorgfältige Nachbeobachtung ist angezeigt, da diese Tumoren jederzeit nach dem Eingriff zum Rezidiv neigen. 

Key words

retroperitoneal fibromatosis - desmoid tumor - surgery

Schlüsselwörter

retroperitoneale Fibromatose - Desmoidtumor - Chirurgie

References

  • 1 Allen P W. The fibromatoses: a clinocopathologic classification based on 140 cases.  Am J Surg Pathol. 1977;  1 255-270
    PubMedGoogle Scholar
  • 2 Anthony T, Rodriguez-Bigas M A, Weber T K et al. Desmoid tumors.  J Am Coll Surg. 1996;  182 369-377
    PubMedGoogle Scholar
  • 3 Ballo M T, Zagars G K, Pollack A et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy.  J Clin Oncol. 1999;  17 158-167
    PubMedGoogle Scholar
  • 4 Bandipalliam P, Balmana J, Syngal S. Comprehensive genetic and endoscopic evaluation may be necessary to distinguish sporadic versus familial adenomatous polyposis-associated abdominal desmoid tumors.  Surgery. 2004;  135 683-689
    CrossrefPubMedGoogle Scholar
  • 5 Brasfield R D, Das Gupta T K. Desmoid tumors of the anterior abdominal wall.  Surgery. 1969;  65 241-246
    PubMedGoogle Scholar
  • 6 Bridge J A, Sreekantaiah C, Mouron B et al. Clonal chromosomal abnormalities in desmoid tumors: implications for histopathogenesis.  Cancer. 1992;  69 430-436
    CrossrefPubMedGoogle Scholar
  • 7 Bruce J M, Bradley 3rd  E L, Satchidanand S K. A desmoid tumor of the pancreas. Sporadic intra-abdominal desmoids revisited.  Int J Pancreatol. 1996;  19 197-203
    PubMedGoogle Scholar
  • 8 Budzynski A, Wysocki A. Retroperitoneal desmoid tumor.  Przegl Lek. 1996;  53 506-507
    PubMedGoogle Scholar
  • 9 Burke A P, Sobin L H, Shekitka K M et al. Intra-abdominal fibromatosis. A pathologic analysis of 130 tumors with comparison of clinical subgroups.  Am J Surg Pathol. 1990;  14 335-341
    PubMedGoogle Scholar
  • 10 Calne R Y, Pollard S G, Jamieson N V et al. Intestinal transplant for recurring mesenteric desmoid tumour.  Lancet. 1993;  342 58-59
    CrossrefPubMedGoogle Scholar
  • 11 Clark S K, Neale K F, Landgrebe J C et al. Desmoid tumours complicating familial adenomatous polyposis.  Br J Surg. 1999;  86 1185-1189
    CrossrefPubMedGoogle Scholar
  • 12 Corbel L, Souissi M, Chretien Y et al. Desmoid tumor of the mesentery. An uncommon cause of ureteral obstruction.  J Urol (Paris). 1991;  97 337-340
    PubMedGoogle Scholar
  • 13 Easter D W, Halasz N A. Recent trends in the management of desmoid tumors. Summary of 19 cases and review of the literature.  Ann Surg. 1989;  210 765-769
    PubMedGoogle Scholar
  • 14 Enzinger F M, Weiss S W. Extraabdominal fibromatosis (extraabdominal desmoid). In: Enzinger FM, Weiss SW, eds. Soft Tissue Tumors. 3rd edn. St. Louis, Mosby 1995: 210–219
    Google Scholar
  • 15 Kiel K D, Suit H D. Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors).  Cancer. 1984;  54 2051-2055
    CrossrefPubMedGoogle Scholar
  • 16 Klein W A, Miller H H, Anderson M et al. The use of indomethacin, sulindac, and tamoxifen for the treatment of desmoid tumours associated with familial polyposis.  Cancer. 1987;  60 2863-2868
    CrossrefPubMedGoogle Scholar
  • 17 Kofoed H, Kamby C, Anagnostaki L. Aggressive fibromatosis.  Surg Gynecol Obstet. 1985;  149 215-218
    PubMedGoogle Scholar
  • 18 Kulaylat M N, Karakousis C P, Keaney C M et al. Desmoid tumour: a pleomorphic lesion.  Eur J Surg Oncol. 1999;  25 487-497
    CrossrefPubMedGoogle Scholar
  • 19 Latchford A R, Sturt N J, Neale K et al. A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis.  Br J Surg. 2006;  93 1258-1264
    CrossrefPubMedGoogle Scholar
  • 20 Lewis J J, Boland P J, Leung D H et al. The enigma of desmoid tumors.  Ann Surg. 1999;  229 866-872
    CrossrefPubMedGoogle Scholar
  • 21 Lopez R, Kemalyan N, Moseley H S et al. Problems in diagnosis and management of desmoid tumors.  Am J Surg. 1990;  159 450-453
    CrossrefPubMedGoogle Scholar
  • 22 Lynch H T, Fitzgibbons Jr R. Surgery, desmoid tumors, and familial adenomatous polyposis: case report and literature review.  Am J Gastroenterol. 1996;  91 2598-2601
    PubMedGoogle Scholar
  • 23 Middleton S B, Phillips R K. Surgery for large intra-abdominal desmoid tumors: report of four cases.  Dis Colon Rectum. 2000;  43 1759-1762
    CrossrefPubMedGoogle Scholar
  • 24 Miralbell R, Suit H D, Mankin H J. Fibromatoses from postsurgical surveillance to combined surgical and radiation therapy.  Int J Radiat Oncol Biol Phys. 1990;  18 535-540
    PubMedGoogle Scholar
  • 25 Mohos E, Kovacs T, Brittig F et al. Desmoid tumors in three patients.  Magy Seb. 2001;  54 387-392
    PubMedGoogle Scholar
  • 26 Morris J A, Johnson D L, Rimmer J A et al. Identical-twin small-bowel transplant for desmoid tumour.  Lancet. 1995;  345 1577-1578
    PubMedGoogle Scholar
  • 27 Nishida S, Levi D, Kato T et al. Ninety-five cases of intestinal transplantation at the University of Miami.  J Gastrointest Surg. 2002;  6 233-239
    CrossrefPubMedGoogle Scholar
  • 28 Ooi B S, Lee C N, Ti T K et al. Retroperitoneal fibromatosis presenting as acute duodenal obstruction.  Aust N Z J Surg. 2001;  71 74-76
    PubMedGoogle Scholar
  • 29 Penna C, Tiret E, Parc R et al. Operation and abdominal desmoid tumors in familial adenomatous polyposis.  Surg Gynecol Obstet. 1993;  177 263-268
    PubMedGoogle Scholar
  • 30 Piquet P, Delpero J R, Pol B et al. Vascular reconstruction after extended resection of a retroperitoneal fibromatosis.  Surgery. 1990;  107 346-349
    PubMedGoogle Scholar
  • 31 Plukker J T, van Oort I, Vermey A et al. Aggressive fibromatosis (non-familial desmoid tumour): therapeutic problems and the role of adjuvant radiotherapy.  Br J Surg. 1995;  82 510-514
    CrossrefPubMedGoogle Scholar
  • 32 Posner M C, Shiu M H, Newsome J L et al. The desmoid tumor. Not a benign disease.  Arch Surg. 1989;  124 191-196
    CrossrefPubMedGoogle Scholar
  • 33 Reitamo J J. The desmoid tumor. IV. Choice of treatment, results, and complications.  Arch Surg. 1983;  118 1318-1322
    PubMedGoogle Scholar
  • 34 Reitamo J J, Scheinin T M, Hayry P. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor.  Am J Surg. 1986;  151 230-237
    CrossrefPubMedGoogle Scholar
  • 35 Shields C J, Winter D C, Kirwan W O et al. Desmoid tumours.  Eur J Surg Oncol. 2001;  27 701-706
    CrossrefPubMedGoogle Scholar
  • 36 Smith A J, Lewis J J, Merchant N B et al. Surgical management of intra-abdominal desmoid tumours.  Br J Surg. 2000;  87 608-613
    CrossrefPubMedGoogle Scholar
  • 37 Sportiello D J, Hoogerland D L. A recurrent pelvic desmoid tumor successfully treated with tamoxifen.  Cancer. 1991;  67 1443-1446
    CrossrefPubMedGoogle Scholar
  • 38 Spree E, Niemann U, Wenisch H. Sporadical extraperitoneal desmoid tumors – review and report on 4 cases.  Zentralbl Chir. 2005;  130 449-453
    Article in Thieme ConnectPubMedGoogle Scholar
  • 39 Weiss E S, Burkart A L, Yeo C J. Fibromatosis of the remnant pancreas after pylorus-preserving pancreaticoduodenectomy.  J Gastrointest Surg. 2006;  10 679-688
    CrossrefPubMedGoogle Scholar
  • 40 Weiss R J, Treiber M, Zahlten-Hinguranage A et al. Improving local control in patients with aggressive fibromatosis by combined surgery and radiotherapy.  Chirurg. 2002;  73 615-621
    CrossrefPubMedGoogle Scholar

Igor MishinM.D., Ph.D. 

str. Muncheshty 52, ap. 60

2001 Kishinev

Moldova

Phone: +3 73 / 22 / 8 32 / 4 65

Fax: +3 73 / 22 / 5 22 / 0 08

Email: mishin_igor@mail.ru

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