|Desmoid tumors of the abdominal wall: A case report.|
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|PMID: 12890284 Owner: NLM Status: Publisher|
|BACKGROUND: Desmoid tumors are slow growing deep fibromatoses with aggressive infiltration of adjacent tissue but without any metastatic potential. CASE PRESENTATION: We report on two female patients with desmoid tumor of the abdominal wall who underwent primary resection. Both patients had a history of an earlier abdominal surgery. Preoperative evaluation included abdominal ultrasound, magnetic resonance imaging and computed tomography. The histology in both cases revealed a desmoid tumor. CONCLUSION: Complete surgical resection is the first line management of this tumor entity.|
|Marcus Overhaus; Pan Decker; Hans Peter Fischer; Hans Jochen Textor; Andreas Hirner|
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|Type: JOURNAL ARTICLE Date: 2003-Jul-9|
|Title: World journal of surgical oncology Volume: 1 ISSN: 1477-7819 ISO Abbreviation: World J Surg Oncol Publication Date: 2003 Jul|
|Created Date: 2003-Jul-31 Completed Date: - Revised Date: -|
Medline Journal Info:
|Nlm Unique ID: 101170544 Medline TA: World J Surg Oncol Country: -|
|Languages: ENG Pagination: 11 Citation Subset: -|
|Department of General-, Visceral-, Thoracic- and Vascular Surgery, Sigmund Freud Str, 25, 53105 Bonn, University of Bonn, Germany. firstname.lastname@example.org|
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Journal ID (nlm-ta): World J Surg Oncol
Publisher: BioMed Central, London
Copyright ? 2003 Overhaus et al; licensee BioMed Central Ltd. This is an Open Access article: verbatim copying and redistribution of this article are permitted in all media for any purpose, provided this notice is preserved along with the article's original URL.
Received Day: 16 Month: 4 Year: 2003
Accepted Day: 9 Month: 7 Year: 2003
collection publication date: Year: 2003
Electronic publication date: Day: 9 Month: 7 Year: 2003
Volume: 1First Page: 11 Last Page: 11
Publisher Id: 1477-7819-1-11
PubMed Id: 12890284
|Desmoid tumors of the abdominal wall: A case report|
|Marcus Overhaus1||Email: email@example.com|
|Pan Decker1||Email: Decker@mutterhaus.de|
|Hans Peter Fischer2||Email: firstname.lastname@example.org|
|Hans Jochen Textor3||Email: email@example.com|
|Andreas Hirner1||Email: firstname.lastname@example.org|
1Department of General-, Visceral-, Thoracic- and Vascular Surgery, Sigmund Freud Str. 25, 53105 Bonn, University of Bonn, Germany
2Department of Pathology, Sigmund Freud Str. 25, 53105 Bonn, University of Bonn, Germany
3Department of Radiology, Sigmund Freud Str. 25, 53105 Bonn, University of Bonn, Germany
Desmoid tumors are benign myofibroblastic neoplasms originating from the muscle aponeurosis and classified as deep fibromatoses . They constitute 3% of all soft tissue tumors and 0.03% of all neoplasms . Despite their aggressive local infiltration, desmoid tumors lack a metastatic potential . However, because of this local infiltration and compression of surrounding structures, a high recurrence rate exists and in anatomic locations with restricted access to surgical resection desmoid tumors can lead to death . Especially in patients with familial adenomatous polyposis (FAP) undergoing colectomy desmoid tumors are the leading cause of morbidity and mortality .
Molecular studies demonstrated desmoid tumors in FAP as clonal neoplasms arising from germline mutation or changes in the APC alleles [6-8]. Cytogenic data verified clonal chromosome aberrations in deep-seated sporadic extra-abdominal fibromatoses and lesions of the abdominal wall and therefore provide additional evidence for the neoplastic nature of these lesions .
Main locations of desmoid tumor occurrence are the proximal extremities, the abdominal wall and the mesenteric intestine in patients with FAP . In sporadic cases, they occur in localisations of trauma, and scars or irradiation. The therapeutic management of these tumors is still controversial.
A 28-year-old female recognized a left lower abdominal wall tumor. In her history she reported an appendectomy and two pregnancies with spontaneous birth in 1994 and 1999. On clinical admission, a painless tumor was investigated without any contact to hip bone structures. Analysed blood parameters were within normal range and tumor markers were negative.
Preoperative computed tomography scan (CT, figure 1) and magnet-resonance-tomography (MRT, figure 2) revealed a left lower abdominal wall tumor of unknown dignity. The MRT morphology demonstrated a fibrotic tumor, originating from the transversal and internal oblique abdominal muscle fascia. The resection of the tumor with excision of the oblique internal abdominal muscle followed and the defect was covered with a Bard Composix-Mesh? (C. R. Bard, Inc., 730 Central Aves Murray Hill, New Jersey, 07974, USA) and major omentum.
The postoperative course was uneventful and the patient was discharged at the 8th postoperative day.
A 37-year-old female recognized an initially painless tumor in the right lower abdominal wall. In her history, the patient reported on a laparoscopic resection of an ovarian-cyst in 1993 and two pregnancies (1995 and 1997) with caesarean section. On admission, a palpable demarcated fixed tumor in the right lower third of the lateral abdominal wall was investigated. Analysed blood parameters were within normal range and tumor markers negative.
The CT revealed an ovarian-tumor, combined with a right abdominal wall tumor of unknown dignity. A metastasis of the ovarian-tumor, a lymphoma or a sarcoma was discussed as differential diagnosis. The MRT demonstrated the abdominal wall tumor originating from the abdominal transversal muscle and internal abdominal oblique muscle fascia. First, extirpation of the right ovary was performed. The resection of the abdominal wall tumor included the excision of the internal abdominal oblique muscle, replaced by a Bard Composix-Mesh? and covered with major omentum.
The postoperative course was uneventful and the patient was discharged at the 9th postoperative day.
Microscopic examination of the resected specimen in both the cases revealed the spindle-cellular tumors immigrated through muscle-tissues consisting of partially intact musclefibers surrounded by spindle like elements. Tumor-cells had a pale eosinophilic cytoplasm and chromatin structures and were embedded in collagen network interrupted by fibrotic sections (Figure 3). The immunohistochemistry revealed a minority of spindled tumor cells, expressing smooth muscle cell actin. Lower than 3% of the tumor-cells expressed the proliferative Ki 67 antigen. A desmoid tumor was diagnosed in both cases (figure 4).
Sections from the ovary in case 2 revealed a demarcated, non capsulated, spindle-cellular, collagen rich tumor a diagnosis of an ovarian fibroma without signs of malignancy was made.
Desmoid tumors are benign deep fibromatoses, originating from fascia and muscle aponeurosis with an infiltrating growth . They are primary located abdominally or intraabdominally , whereas only sporadic cases describe a localisation within the thorax wall  or retroperitoneally . The desmoid tumor is often associated with female gender, FAP  and occasionally with surgical trauma . It has a higher prevalence in women who experienced pregnancy . Depending on the tumors size, on the therapy and on the negative resection margins, recurrence occurs in up to 45% .
CT scan localizes the tumor and excludes metastasis (figure 1). MRT reveals the tumor's hypointensity on T1 and demonstrates variable signal intensity on T2 weighted imaging, depending on the accumulation of mucoid structures (figure 2). Therefore, a differentiation from other solid tumors is impossible using 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 (figure 3). Characteristically, there is a diffuse cell infiltration of adjacent tissue structures. Immunohistochemical response for actin can be partially positive and immunohistochemical muscle cell markers delimit desmoid tumors from fibrosarcoma .
The therapy of choice is still controversial . Anti-inflammatory treatment , hormone-therapy  and chemotherapy  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. In a comparative analysis, a significantly better local recurrence control was described with radiation and combined surgical resection in comparison to resection only . However, these results of radiation therapy can only be achieved due to a higher complication rate . Other studies show a tumor progress after radiation therapy  and a high local recurrence rate .
Due to the germline mutations and chromosomal aberrations of the APC alleles Bright-Thomas et al. performed a pre-clinical study of gene transfer for the treatment of desmoid disease in FAP . Despite the success of transgene expression, further work is needed in animal models of desmoid disease to assess the clinical effects of gene therapy.
Therefore, the most effective treatment of accessible and smaller desmoid tumors is still the resection with negative margins, although it may not prevent local recurrence . However, massive mesenteric lesions often occur in FAP patients after colectomy  and non surgical treatment has variable and unpredictable objective efficacy [10,21,28,29]. Middleton et al. reported on successful short-term surgery in four cases of large intra-abdominal tumors in which non-surgical treatment was inefficient . In conclusion, there remains a need for a wider range of therapeutic options, especially in the management of these massive tumors.
Surgery always aims at radical tumor resection with free margins, which, depending on localisation of surgery, may leave major soft tissue defects behind [28,30,31]. Although abdominal wall integrity after full-thickness surgery can be restored with direct sutures [4,32], reconstruction with synthetic materials is a common technique in major abdominal wall defects . Albeit in our study the small tumor resection with tumor free margins and reconstruction of the abdominal wall was performed with a Bard Composix-Mesh? (figure 5), recent literature data recommend distant or free muscle flaps for greater abdominal wall defect coverage that is not accessible to local flaps [30,32]. Additionally, prosthetic material is more susceptible to bacterial infection and other complications [34,35], although new developed material showed encouraging experimental results .
In conclusion, the treatment of desmoid tumors remains enigmatic. Non-surgical treatment resulted in diverse and unpredictable outcome and is considered to be an opportunity in patients with unresectable lesions or for adjuvant therapy. Radical resection with clear margins remains the principal determinant of outcome with the risk of local recurrence.
computed tomography scan (CT), magnet-resonance-tomography (MRT), familial adenomatous polyposis (FAP)
MO participated in the preparation of the manuscript, literature search and drafted the manuscript.
PD was responsible for the operations, follow-up of the patients and data preparation and conceived of the study.
HPF carried out the tissue preparation, (immuno)histology and edited the manuscript for its scientific content.
HJT accomplished and analysed the CT scans and MRT and approved the final version of the manuscript.
AH participated in its design and coordination.
All authors read and approved the final manuscript.
|Fletcher CDM. Myofibroblastic tumours: an update.Verh Dtsch Ges Path 1998;82:75–82. [pmid: 10095420]|
|Kiel KD,Suit HD. Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors)Cancer 1984;54:2051–2055. [pmid: 6488135]|
|Lewis JJ,Boland PJ,Leung DH,Woodruff JM,Brennan MF. The enigma of desmoid tumorsAnn Surg 1999;229:866–872. [pmid: 10363901] [doi: 10.1097/00000658-199906000-00014]|
|Merchant NB,Lewis JJ,Woodruff JM,Leung DH,Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcomeCancer 1999;86:2045–2052. [pmid: 10570430] [doi: 10.1002/(SICI)1097-0142(19991115)86:10<2045::AID-CNCR23>3.3.CO;2-6]|
|Nugent KP,Spigelman AD,Phillips RK. Life expectancy after colectomy and ileorectal anastomosis for familial adenomatous polyposisDis Colon Rectum 1993;36:1059–1062. [pmid: 8223060]|
|Dangel A,Meloni AM,Lynch HT,Sandberg AA. Deletion (5q) in a desmoid tumor of a patient with Gardner's syndromeCancer Genet Cytogenet 1994;78:94–98. [pmid: 7987814] [doi: 10.1016/0165-4608(94)90053-1]|
|Miyaki M,Konishi M,Kikuchi-Yanoshita R,Enomoto M,Tanaka K,Takahashi H,Muraoka M,Mori T,Konishi F,Iwama T. Coexistence of somatic and germ-line mutations of APC gene in desmoid tumors from patients with familial adenomatous polyposisCancer Res 1993;53:5079–5082. [pmid: 8221638]|
|Palmirotta R,Curia MC,Esposito DL,Valanzano R,Messerini L,Ficari F,Brandi ML,Tonelli F,Mariani-Costantini R,Battista P. Novel mutations and inactivation of both alleles of the APC gene in desmoid tumorsHum Mol Genet 1995;4:1979–1981. [pmid: 8595425]|
|de Wever I,Dal Cin P,Fletcher CD,Mandahl N,Mertens F,Mitelman F,Rosai J,Rydholm A,Sciot R,Tallini G,Van den Berghe H,Vanni R,Willen H. Cytogenetic, clinical, and morphologic correlations in 78 cases of fibromatosis: a report from the CHAMP Study Group. CHromosomes And MorphologyMod Pathol 2000;13:1080–1085. [pmid: 11048801]|
|Lynch HT,Fitzgibbons R, Jr. Surgery, desmoid tumors, and familial adenomatous polyposis: case report and literature reviewAm J Gastroenterol 1996;91:2598–2601. [pmid: 8946994]|
|Hasegawa SL,Fletcher CDM. Fibromatosis in the adultAdv Pathol 1996;9:259–275.|
|Gacouin A,Desrues B,Lecoz A,Quinquenel ML,Ramee MP,Delaval P. [Desmoid tumor of the thoracic wall]Rev Mal Respir 1993;10:554–556. [pmid: 8122023]|
|Budzynski A,Wysocki A. [Retroperitoneal desmoid tumor]Przegl Lek 1996;53:506–507. [pmid: 8819361]|
|Soravia C,Berk T,McLeod RS,Cohen Z. Desmoid disease in patients with familial adenomatous polyposisDis Colon Rectum 2000;43:363–369. [pmid: 10733118]|
|De Cian F,Delay E,Rudigoz RC,Ranchere D,Rivoire M. Desmoid tumor arising in a cesarean section scar during pregnancy: monitoring and managementGynecol Oncol 1999;75:145–148. [pmid: 10502442] [doi: 10.1006/gyno.1999.5539]|
|Gansar GF,Markowitz IP,Cerise EJ. Thirty years of experience with desmoid tumors at Charity HospitalAm Surg 1987;53:318–319. [pmid: 3579044]|
|Nuyttens JJ,Rust PF,Thomas CR, Jr,Turrisi AT,III. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articlesCancer 2000;88:1517–1523. [pmid: 10738207] [doi: 10.1002/(SICI)1097-0142(20000401)88:7<1517::AID-CNCR3>3.3.CO;2-0]|
|Casillas J,Sais GJ,Greve JL,Iparraguirre MC,Morillo G. Imaging of intra- and extraabdominal desmoid tumorsRadiographics 1991;11:959–968. [pmid: 1749859]|
|Mentzel T,Katenkamp D. [Myofibroblastic tumors. Brief review of clinical aspects, diagnosis and differential diagnosis]Pathologe 1998;19:176–186. [pmid: 9648142] [doi: 10.1007/s002920050271]|
|Shields CJ,Winter DC,Kirwan WO,Redmond HP. Desmoid tumoursEur J Surg Oncol 2001;27:701–706. [pmid: 11735163] [doi: 10.1053/ejso.2001.1169]|
|Waddell WR,Kirsch WM. Testolactone, sulindac, warfarin, and vitamin K1 for unresectable desmoid tumorsAm J Surg 1991;161:416–421. [pmid: 2035759]|
|Wilcken N,Tattersall MH. Endocrine therapy for desmoid tumorsCancer 1991;68:1384–1388. [pmid: 1831400]|
|Patel SR,Evans HL,Benjamin RS. Combination chemotherapy in adult desmoid tumorsCancer 1993;72:3244–3247. [pmid: 8242548]|
|Goy BW,Lee SP,Eilber F,Dorey F,Eckardt J,Fu YS,Juillard GJ,Selch MT. The role of adjuvant radiotherapy in the treatment of resectable desmoid tumorsInt J Radiat Oncol Biol Phys 1997;39:659–665. [pmid: 9336146] [doi: 10.1016/S0360-3016(97)00334-9]|
|Rock MG,Pritchard DJ,Reiman HM,Soule EH,Brewster RC. Extra-abdominal desmoid tumorsJ Bone Joint Surg Am 1984;66:1369–1374. [pmid: 6501332]|
|Pritchard DJ,Nascimento AG,Petersen IA. Local control of extra-abdominal desmoid tumorsJ Bone Joint Surg Am 1996;78:848–854. [pmid: 8666602]|
|Bright-Thomas RM,Agrawal A,Hargest R. Preclinical studies of gene transfer for the treatment of desmoid disease in familial adenomatous polyposisBr J Surg 2002;89:1563–1569. [pmid: 12445067] [doi: 10.1046/j.1365-2168.2002.02277.x]|
|Middleton SB,Phillips RK. Surgery for large intra-abdominal desmoid tumors: report of four casesDis Colon Rectum 2000;43:1759–1762. [pmid: 11156464]|
|Okuno SH,Edmonson JH. Combination chemotherapy for desmoid tumorsCancer 2003;97:1134–1135. [pmid: 12569616] [doi: 10.1002/cncr.11189]|
|Brenner P,Rammelt S. Abdominal wall and foot reconstruction after extensive desmoid tumor resection with free tissue transferLangenbecks Arch Surg 2002;386:592–597. [pmid: 11914936] [doi: 10.1007/s00423-002-0277-y]|
|Sutton RJ,Thomas JM. Desmoid tumours of the anterior abdominal wallEur J Surg Oncol 1999;25:398–400. [pmid: 10419711] [doi: 10.1053/ejso.1999.0664]|
|Rohrich RJ,Lowe JB,Hackney FL,Bowman JL,Hobar PC. An algorithm for abdominal wall reconstructionPlast Reconstr Surg 2000;105:202–216. [pmid: 10626993]|
|Bauer JJ,Salky BA,Gelernt IM,Kreel I. Repair of large abdominal wall defects with expanded polytetrafluoroethylene (PTFE)Ann Surg 1987;206:765–769. [pmid: 3689012]|
|Disa JJ,Klein MH,Goldberg NH. Advantages of autologous fascia versus synthetic patch abdominal reconstruction in experimental animal defectsPlast Reconstr Surg 1996;97:801–806. [pmid: 8628775]|
|Leber GE,Garb JL,Alexander AI,Reed WP. Long-term complications associated with prosthetic repair of incisional herniasArch Surg 1998;133:378–382. [pmid: 9565117] [doi: 10.1001/archsurg.133.4.378]|
|Disa JJ,Chiaramonte MF,Girotto JA,Klein MH,Goldberg NH. Advantages of autologous fascia versus synthetic patch abdominal reconstruction in experimental animal defectsPlast Reconstr Surg 2001;108:2086–2087. [pmid: 11743406] [doi: 10.1097/00006534-200112000-00040]|
Keywords: Desmoid, fibromatosis, abdominal, genotype, familial adenomatous polyposis, clonal neoplasm, surgery..
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