iprs000042
10.3205/iprs000042
urn:nbn:de:0183-iprs0000421
Research Article
Diagnosis and treatment of extraabdominal desmoid fibromatosis
Diagnostik und Therapie der extraabdominalen Desmoidfibromatose
Ghanem
Ghanem
Mohamed
M
Dr. med.
MBA
Orthopädische Klinik und Poliklinik, Universitätsklinikum Leipzig, Liebigstr. 20, 04103 Leipzig, Germany, Phone: 0341-9723878, Fax: 0341-9723209Department of Orthopaedic Surgery, University Hospital of Leipzig, Germany
mohamed.ghanem@medizin.uni-leipzig.de
author
Heinisch
Heinisch
Antje
A
Department of Orthopaedic Surgery, University Hospital of Leipzig, Germany
author
Heyde
Heyde
Christoph-E.
CE
Department of Orthopaedic Surgery, University Hospital of Leipzig, Germany
author
Freiherr von Salis-Soglio
Freiherr von Salis-Soglio
Georg
G
Department of Orthopaedic Surgery, University Hospital of Leipzig, Germany
author
German Medical Science GMS Publishing House
Düsseldorf
610
desmoid fibromatosis
soft tissue tumors
surgery
local recurrence
extraabdominelle Desmoidfibromatose
Weichteiltumoren
operative Behandlung
Lokalrezidive
2014022420150622
engl
This is an Open Access article distributed under the terms of the Creative Commons Attribution License. You are free: to Share - to copy, distribute and transmit the work, provided the original author and source are credited.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen. Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.
2193-8091
3
GMS Interdisciplinary Plastic and Reconstructive Surgery DGPW
GMS Interdiscip Plast Reconstr Surg DGPW
01
20150622In the initial publication the article was erroneously designated as "Review Article".
Einleitung: Die extraabdominelle Desmoidfibromatose ist eine außerordentlich seltene, als semimaligne eingestufte Bindegewebserkrankung. Auf Grund der Seltenheit, der nicht spezifischen Lokalisation, des unspezifischen Erscheinungsbildes und der hohen Rezidivrate bleiben sowohl die Diagnostik als auch Therapie der extraabdominellen Desmoidfibromatose eine Herausforderung. Anhand einer Aufarbeitung des eigenen Patientengutes sowie einer umfassenden Literaturrecherche soll der aktuelle Wissensstand zu diesem Krankheitsbild dargestellt werden. Material und Methoden: Zwischen August 1998 und Mai 2007 wurden 7 Patienten mit einer extraabdominalen Desmoidfibromatose operativ behandelt. Alle Patienten wurden mittels eines definierten Diagnosealgorithmus einschließlich MRT und Biopsie diagnostiziert. Die Therapie war in allen Fällen operativ. Der Nachuntersuchungszeitraum betrug im Durchschnitt 4 (1–7) Jahre. Zum Nachuntersuchungszeitpunkt erfolgten eine Befragung, eine klinische Untersuchung sowie eine MRT-Kontrolle. Ergebnisse: Die Ergebnisse zeigen, dass wir R0-Resektion in 4 Fällen, R1 in zwei Fällen und Rx in einem Fall erreicht haben. Bei 4 Patienten wurde kein Rezidiv nach der einmaligen Operation in unserer Klinik beobachtet. Bei 2 Patienten wurde jeweils eine einzige Revisionsoperation durchgeführt, wonach beide Patienten rezidivfrei waren. In nur einem Fall waren mehrere Operationen (eine primäre und zwei Revisionsoperationen) notwendig, nach denen kein Rezidiv berichtet wurde. Schlussfolgerungen: Die frühzeitige Diagnosestellung ist für den Erfolg der operativen Behandlung von Bedeutung, hierbei ist ein definierter Diagnosealgorithmus einschließlich einer MRT-Untersuchung und einer Biopsie obligat. Die Operation stellt die Behandlung der Wahl dar. Engmaschige Nachkontrollen sind notwendig, um Rezidive frühzeitig erkennen und dann gegebenenfalls operativ behandeln zu können.
Introduction: The desmoid fibromatosis is a very rare connective tissue disease which is recognized as semimalignant. The aim of this work is to review the relevant literature and to analyze the management of our patient collective. Material and Method: Surgery was performed on 7 patients with extraabdominal desmoid fibromatosis between August 1998 and May 2007. MRI examination as well as biopsy was carried out in all cases. All patients were operated on; the mean follow up was 4 years (1–7). Upon follow up, every patient has undergone clinical and MRI examination. Results: The results show that we have achieved R0 resection in 4 cases and R1 in two cases and Rx in one case. In 4 patients, no recurrence was observed after the single surgery performed in our hospital. In <![CDATA[2 patients</PlainText></TextGroup> a single revision surgery was performed in each case and yielded no further recurrence. In only one case, multiple surgeries (one primary and two revision surgeries) were necessary, after which no recurrence was reported.</Pgraph><Pgraph><Mark1>Conclusion:</Mark1> The early diagnosis of the disease is of utmost importance to the success of the outcome. MRI examination and biopsy are mandatory. Surgery is the therapy of choice. The recurrence rate is high and is linked to the difficulty of recognition of the exact infiltrative extent of the tumour. This necessitates a close follow-up.</Pgraph></Abstract>
<TextBlock linked="yes" name="Introduction">
<MainHeadline>Introduction</MainHeadline><Pgraph>Desmoid fibromatosis is an extremely rare disease, which is known since the 19th Century <TextLink reference="2"></TextLink>, <TextLink reference="28"></TextLink>, <TextLink reference="30"></TextLink>, <TextLink reference="34"></TextLink>, <TextLink reference="40"></TextLink>. Synonymous terms are: desmoid tumor, aggressive fibromatosis, desmoid-type fibromatosis, musculoaponeurotic fibromatosis <TextLink reference="6"></TextLink>, <TextLink reference="12"></TextLink>, <TextLink reference="22"></TextLink>, <TextLink reference="26"></TextLink>, <TextLink reference="27"></TextLink>. It is a neoplasm of connective tissue, which arises from musculoaponeurotic structures. It is locally aggressive and infiltrates the surrounding tissue (Figure 1 <ImgLink imgNo="1" imgType="figure"/>). </Pgraph><Pgraph>The etiology is still largely unknown [2, 6, 18, 37, 40, 41]. Metastases are not described in literature, but frequent local recurrences are reported <TextLink reference="2"></TextLink>, <TextLink reference="5"></TextLink>, <TextLink reference="7"></TextLink>, <TextLink reference="14"></TextLink>, <TextLink reference="16"></TextLink>, <TextLink reference="17"></TextLink>, <TextLink reference="24"></TextLink>, <TextLink reference="45"></TextLink>. With an incidence of 2–5 per million population per year <TextLink reference="20"></TextLink>, <TextLink reference="22"></TextLink>, <TextLink reference="38"></TextLink>, <TextLink reference="43"></TextLink>, desmoid fibromatosis is an extremely rare tumor entity. Desmoid fibromatosis is seen in patients with familial adenomatous polyposis (FAP) with an incidence of 3.5 to 32% <TextLink reference="3"></TextLink>, <TextLink reference="26"></TextLink>, <TextLink reference="27"></TextLink>, <TextLink reference="33"></TextLink>. Desmoid fibromatosis can develop in men and women at any age <TextLink reference="6"></TextLink>, <TextLink reference="29"></TextLink>, <TextLink reference="34"></TextLink>. Females are more frequently affected by this disease <TextLink reference="11"></TextLink>, <TextLink reference="24"></TextLink>, <TextLink reference="25"></TextLink>, <TextLink reference="32"></TextLink>, <TextLink reference="41"></TextLink>, <TextLink reference="45"></TextLink>. There are several ways to classify desmoid fibromatosis. The classification of fibromatosis according to Enzinger and Weiss <TextLink reference="44"></TextLink> is accepted worldwide. Accordingly, fibromatoses can be classified into superficial and deep fibromatoses <TextLink reference="9"></TextLink>, <TextLink reference="40"></TextLink>. Desmoidfibromatoses can also be divided into extra-abdominal, abdominal and intra-abdominal tumors <TextLink reference="18"></TextLink>, <TextLink reference="33"></TextLink> according to their localization. Peter Schulte et al. <TextLink reference="26"></TextLink> proposed a uniform clinical classification of desmoid fibromatoses which summerizes the clinical heterogeneous behaviour (Table 1 <ImgLink imgNo="1" imgType="table"/>). </Pgraph><Pgraph>The aim of this classification was to achieve standardization and to facilitate the development of therapeutic guidelines. The first manifestation of desmoid fibromatosis is very diverse. It is reported in literature that patients usually manifest with a painless extraabdominal swelling. Intra-abdominal desmoid fibromatoses can be occult and remain completely asymptomatic over a long period of time <TextLink reference="12"></TextLink>, <TextLink reference="15"></TextLink>, <TextLink reference="36"></TextLink>, <TextLink reference="41"></TextLink>, <TextLink reference="42"></TextLink>. However desmoid fibromatoses may first manifest <TextLink reference="4"></TextLink>, <TextLink reference="35"></TextLink>, <TextLink reference="39"></TextLink> by pain. Depending on their localisation and extent, they can cause considerable disability and morbidity <TextLink reference="1"></TextLink>, <TextLink reference="5"></TextLink>, <TextLink reference="9"></TextLink>, <TextLink reference="14"></TextLink>, <TextLink reference="24"></TextLink>. MRI (Figure 1 <ImgLink imgNo="1" imgType="figure"/>) is of crucial importance and is considered as the gold standard in the diagnosis <TextLink reference="1"></TextLink>, <TextLink reference="14"></TextLink>, <TextLink reference="24"></TextLink>, <TextLink reference="32"></TextLink>. </Pgraph><Pgraph>The diagnosis is to be confirmed by biopsy. Microscopically, there is a benign appearance with a well differentiated composition of intersecting bundles of fibroblasts, which are separated by collagen deposits (Figure 2 <ImgLink imgNo="2" imgType="figure"/>). </Pgraph><Pgraph>The tissue is mainly composed of collagen with regular extensions in adjacent tissue <TextLink reference="1"></TextLink>, <TextLink reference="11"></TextLink>, <TextLink reference="29"></TextLink>, <TextLink reference="39"></TextLink>. The treatment of choice is surgery. In view of the great tendency for recurrence, a wide resection must be implemented <TextLink reference="5"></TextLink>, <TextLink reference="8"></TextLink> in order to achieve a secure R0 resection <TextLink reference="1"></TextLink>, <TextLink reference="13"></TextLink>, <TextLink reference="25"></TextLink>. A sole or adjuvant radiotherapy should be considered in inoperable cases or in cases of insufficient resection of the tumor <TextLink reference="22"></TextLink>. Drug therapy (hormone therapy, NSAIDs, chemotherapy) is only of secondary importance due to lack of sufficient evidence <TextLink reference="6"></TextLink>, <TextLink reference="24"></TextLink>. </Pgraph><Pgraph>The main problem of this entity of tumors lies in the non-specific appearance and delayed diagnosis as well as the extremely high tendency for recurrence <TextLink reference="5"></TextLink>, <TextLink reference="6"></TextLink>, <TextLink reference="8"></TextLink>, <TextLink reference="21"></TextLink>, <TextLink reference="28"></TextLink>. Based on a literature review and a retrospective analysis of the treated patients in our department, this work deals with the aspects of adequate diagnosis and optimal therapy.</Pgraph></TextBlock>
<TextBlock linked="yes" name="Material and methods">
<MainHeadline>Material and methods</MainHeadline><Pgraph>We retrospectively analyzed seven patients who were treated between August 1998 and May 2007. Only those patients with a sole extraabdominal desmoid fibromatosis were included. The retrospective analysis was performed using a written questionnaire which was specially designed for this study. In addition, all seven patients were subjected to a comprehensive survey and clinical as well as MRI follow-up. The average age of patients at initial presentation to our clinic was 54.6 years (37 to 68 years), the sex ratio was balanced with three women and four men. The most common anatomic location was in the shoulder-arm region (5/7 tumors: 2x shoulder, 2x scapula, 1x axilla). The other two cases presented the tumor at the flank and at the lower leg respectively (Figure 3 <ImgLink imgNo="3" imgType="figure"/> and Figure 4 <ImgLink imgNo="4" imgType="figure"/>). </Pgraph><Pgraph>Five patients presented with a painless and two patients with a painful soft tissue swelling increasing in size. The time interval between the first symptoms and first diagnosis was averaged 4.5 (2–11) months. The diagnostic investigations made in all cases were local ultrasound, X-ray, contrast-enhanced MRI and a biopsy with a subsequent evaluation in the interdisciplinary tumor board. </Pgraph></TextBlock>
<TextBlock linked="yes" name="Results">
<MainHeadline>Results</MainHeadline><Pgraph>We performed surgery on all the seven patients. For practical purpose, we divided these patients into two groups; the first group consisted of four patients and presented primarily in our hospital, whereas the second group consisted of three patients who were previously operated in other hospitals and presented to us with a recurrence.</Pgraph><Pgraph>R0 resection could be performed in three out of the four patients of the first group. In one of these resections the safety margin was only 1 mm. Two months after surgery the patient was free of recurrence. </Pgraph><Pgraph>In a further case of group one, intraoperative frozen section yielded insufficient security boundaries, so that meticulously wider resection was carried out. The resected material was sufficiently enough in sano. At the 18 month postoperative follow-up, the patient was free of recurrence. </Pgraph><Pgraph>In the third case, a resection in sano (1.5 mm safety margin) was performed. 20 months later, the patient developed recurrence. A new R0 resection (safety margin <1cm) followed. 10 months postoperatively, a second recurrence was observed. Despite further R0 resection a third recurrence was observed seven months later. Revision surgery was carried out with a wide safety margin; this patient is now free of recurrence.</Pgraph><Pgraph>Concerning the fourth patient, recurrence was observed during adjuvant radiotherapy interval after an R1 resection. Revision surgery yielded an Rx status. Hormone therapy with tamoxifen has been added. At the follow-up in May 2007 (8 years and 5 months after the last surgery), the patient was free of recurrence. </Pgraph><Pgraph>Regarding the three patients of second group, one patient was reported to having had R1 resection. We performed revision surgery in which still only R1 resection could be achieved. The remaining residual tumor showed no sign of growth by the last follow-up (18 months postoperatively). </Pgraph><Pgraph>In the case of the second patient of the second group, a two-stage resection was performed at another hospital due to a very complex structure of tumor in close proximity to major vascular and nerve cords at the shoulder region. Information on resection status was not available. Because of recurrence, we performed a radical resection (interscapulo-thoracic amputation). This could still only achieve R1 resection, after which the patient was free of recurrence at the follow-up in May 2007 (42 months after the last surgery). </Pgraph><Pgraph>In the case of the third patient in the second group, R1 resection was achieved after surgery in another hospital. Due to recurrence, we performed revision surgery (<TextGroup><PlainText>5 months</PlainText></TextGroup> postoperatively) and achieved an R0 status. Yet again four months later the patient developed recurrence, which in turn could be resected in sano. The safety margin ranged here between 1.1 cm and 3.5 cm. Since then, the patient is free of recurrence. </Pgraph><Pgraph>The overall results show that we have achieved R0 resection in 4 cases and R1 in two cases and Rx in one case. In 4 patients, no recurrence was observed after the single surgery performed in our hospital. In 2 patients a single revision surgery was performed in each case and yielded no further recurrence. In only one case, multiple surgeries (one primary and two revision surgeries) were necessary, after which no recurrence was reported.</Pgraph></TextBlock>
<TextBlock linked="yes" name="Discussion">
<MainHeadline>Discussion</MainHeadline><Pgraph>Extra-abdominal desmoid fibromatoses are usually painless lesions <TextLink reference="39"></TextLink>. The majority of our patients (5/7) presented with a localized painless swelling. Clearly a rough swelling was palpable in all patients. These findings are conforming to the data reported in literature <TextLink reference="12"></TextLink>, <TextLink reference="15"></TextLink>, <TextLink reference="36"></TextLink>, <TextLink reference="42"></TextLink>. Dalén et al. <TextLink reference="5"></TextLink> and Mendenhall et al. <TextLink reference="20"></TextLink> reported a time interval of approximately one year between the first symptoms and the diagnosis. In our hospital, the diagnosis could be made after an average of 4.5 months. Sonography, conventional radiography, CT and nuclear medicine were non-specific and thus not reliable investigative tools <TextLink reference="9"></TextLink>, <TextLink reference="15"></TextLink>, <TextLink reference="19"></TextLink>, <TextLink reference="24"></TextLink>, <TextLink reference="27"></TextLink>, <TextLink reference="32"></TextLink>. In the literature, MRI is the method of choice in the diagnosis of desmoid fibromatosis <TextLink reference="1"></TextLink>, <TextLink reference="6"></TextLink>, <TextLink reference="24"></TextLink>. This method evaluates the exact localization, extension, growth behavior as well as recurrence. The contrast enhanced MRI is therefore essential for preoperative planning and hence was performed in all of our patients <TextLink reference="32"></TextLink>. However, reactive edema within the bone marrow or soft tissue and contrast-induced increased signal can mimic aggressive processes <TextLink reference="36"></TextLink>. Thus, a biopsy is mandatory to confirm the diagnosis. The treatment of choice for extraabdominal desmoid fibromatosis is surgical treatment <TextLink reference="5"></TextLink>. Desmoid fibromatosis has to be surgically treated by wide resection <TextLink reference="8"></TextLink>, with the goal to achieve secure R0 resection <TextLink reference="1"></TextLink>, <TextLink reference="13"></TextLink>, <TextLink reference="25"></TextLink>. The complete surgical removal of the tumor, however, is often difficult to achieve because an intraoperative demarcation between the diffusely infiltrating tumor and the surrounding healthy tissue is rather difficult in many cases. Literature reports point to an appropriate safety margin ranging from 1 cm <TextLink reference="6"></TextLink> to about 5 cm <TextLink reference="39"></TextLink>. The histopathological findings of our analysis have shown that the achieved safety margin was less than 1 cm in all these cases. Thus, recurrence occurred in the four patients despite the fact that an R0 resection was achieved. The safety zone, however, was less than a centimeter. </Pgraph><Pgraph>Leithner et al. <TextLink reference="18"></TextLink> studied 412 patients with extraabdominal primary tumor. While the recurrence rate was 27% after wide or radical resection, it was around 2.5 times higher (72% recurrence rate) after marginal or intralesional resection. Also in 127 patients with a recurrent extraabdominal tumor the microscopic surgical margin was a significant prognostic factor <TextLink reference="18"></TextLink>. This result supports the hypothesis that wide resections of extraabdominal desmoid fibromatosis are associated with a lower recurrence rate. The influence of the extent of resection on the development of local recurrence is still discussed in the literature <TextLink reference="34"></TextLink>. This is primarily due to the fact that the published data rarely refer to a type of desmoid fibromatosis. On the other hand, recurrence is observed even after wide resection. One of our patients developed recurrence 20 months after a wide R0 resection. In this context, only the surgical removal of the primary tumor may be considered as a local trauma and hence as a stimulus for the development of recurrence as described in the literature <TextLink reference="22"></TextLink>. </Pgraph><Pgraph>Further, it is reported in literature that surgical procedures could be combined with radiotherapy. A peri- and postoperative radiation therapy or as an adjunct to surgical treatment is a treatment option for reducing the risk of recurrence, especially in those patients in whom no R0 resection can be achieved <TextLink reference="24"></TextLink>, <TextLink reference="43"></TextLink>. Depending on the location and extent of the tumor, adjuvant radiotherapy must be considered in patients with R1 resection and/or recurrence. Patients with R2 resection should always receive radiotherapy when revision surgery is not possible <TextLink reference="20"></TextLink>. Radiotherapy alone is recommended if the tumor is inoperable <TextLink reference="28"></TextLink>, <TextLink reference="31"></TextLink>, <TextLink reference="38"></TextLink>. Radiotherapy is performed one month after the surgical procedure <TextLink reference="28"></TextLink>. Wide margins and high doses are recommended to achieve adequate local control <TextLink reference="24"></TextLink>. The optimal total dose is 50–5<TextGroup><PlainText>5 Gy</PlainText></TextGroup> <TextLink reference="6"></TextLink>, <TextLink reference="20"></TextLink>. Desmoid fibromatosis responds to cytotoxic chemotherapy. In some patients, symptomatic and permanent regression was observed. However, there are no evidence-based studies which describe a consistent treatment success of extraabdominal desmoid fibromatosis <TextLink reference="6"></TextLink>, <TextLink reference="24"></TextLink>. Chew et al. <TextLink reference="2"></TextLink> state that a waiting and observing strategy might be reasonable in cases of desmoid fibromatosis of extremities in which a radical resection would only be realized by means of an amputation. Even Rock et al. <TextLink reference="31"></TextLink> recommend close observation when recurrent lesions are not progressive until a possible tumor growth makes a further excision necessary. Thus, the risk of observation as a sole treatment should be weighed against the risk of possible morbidities in case of primary resection or resection of recurrent disease <TextLink reference="21"></TextLink>.</Pgraph></TextBlock>
<TextBlock linked="yes" name="Conclusions">
<MainHeadline>Conclusions</MainHeadline><Pgraph>Early diagnosis is essential for the treatment of desmoid fibromatosis, since this improves the chances of success of surgical therapy. Contrast enhanced MRI scan and a biopsy are obligatory as part of the standardized diagnostic work-up. The problem in the treatment of desomid fibromatosis is mainly due to the aggressive infiltrative nature of tumor growth which causes considerable difficulty in properly assessing the boundaries of the tumor intraoperatively. Surgery is usually the treatment of choice and adequate safety margins (>1 cm) should be achieved. Intraoperative frozen section analysis is recommended to assure adequate safety margins. Adjuvant radiotherapy is to be considered in case of narrow R0 resection and in case of R1 resection and recurrences. Radiotherapy is obligatory in case of R2 resections. Radiotherapy as a sole treatment is only considered in inoperable patients or in cases where surgical treatment would be associated with a significant loss of function. Frequent follow-up is necessary to early detect a possible recurrence.</Pgraph></TextBlock>
<TextBlock linked="yes" name="Notes">
<MainHeadline>Notes</MainHeadline><SubHeadline>Competing interests</SubHeadline><Pgraph>The authors declare that they have no competing interests.</Pgraph><SubHeadline>Authorship</SubHeadline><Pgraph>The authors M. Ghanem and A. Heinisch have equally contributed to this work.</Pgraph><SubHeadline>Acknowledgement </SubHeadline><Pgraph>The authors would like to thank the Institute for Pathology of the Leipzig University, Chairman Prof. Dr. C. Wittekind, for their work and for Figure 1. The authors would also like to thank the Department of Radiology of the Leipzig University, Prof. Dr. T. Kahn, for their work and for <TextGroup><PlainText>Figure 2</PlainText></TextGroup>.</Pgraph></TextBlock>
<References linked="yes">
<Reference refNo="1">
<RefAuthor>Biermann JS</RefAuthor>
<RefTitle>Desmoid tumors</RefTitle>
<RefYear>2000</RefYear>
<RefJournal>Curr Treat Options Oncol</RefJournal>
<RefPage>262-6</RefPage>
<RefTotal>Biermann JS. Desmoid tumors. Curr Treat Options Oncol. 2000 Aug;1(3):262-6. DOI: 10.1007/s11864-000-0038-5</RefTotal>
<RefLink>http://dx.doi.org/10.1007/s11864-000-0038-5</RefLink>
</Reference>
<Reference refNo="2">
<RefAuthor>Chew C</RefAuthor>
<RefAuthor>Reid R</RefAuthor>
<RefAuthor>O'Dwyer PJ</RefAuthor>
<RefTitle>Evaluation of the long term outcome of patients with extremity desmoids</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>Eur J Surg Oncol</RefJournal>
<RefPage>428-32</RefPage>
<RefTotal>Chew C, Reid R, O'Dwyer PJ. Evaluation of the long term outcome of patients with extremity desmoids. Eur J Surg Oncol. 2004 May;30(4):428-32. DOI: 10.1016/j.ejso.2004.01.010</RefTotal>
<RefLink>http://dx.doi.org/10.1016/j.ejso.2004.01.010</RefLink>
</Reference>
<Reference refNo="3">
<RefAuthor>Clark SK</RefAuthor>
<RefAuthor>Neale KF</RefAuthor>
<RefAuthor>Landgrebe JC</RefAuthor>
<RefAuthor>Phillips RK</RefAuthor>
<RefTitle>Desmoid tumours complicating familial adenomatous polyposis</RefTitle>
<RefYear>1999</RefYear>
<RefJournal>Br J Surg</RefJournal>
<RefPage>1185-9</RefPage>
<RefTotal>Clark SK, Neale KF, Landgrebe JC, Phillips RK. Desmoid tumours complicating familial adenomatous polyposis. Br J Surg. 1999 Sep;86(9):1185-9. DOI: 10.1046/j.1365-2168.1999.01222.x</RefTotal>
<RefLink>http://dx.doi.org/10.1046/j.1365-2168.1999.01222.x</RefLink>
</Reference>
<Reference refNo="4">
<RefAuthor>Clark TW</RefAuthor>
<RefTitle>Percutaneous chemical ablation of desmoid tumors</RefTitle>
<RefYear>2003</RefYear>
<RefJournal>J Vasc Interv Radiol</RefJournal>
<RefPage>629-34</RefPage>
<RefTotal>Clark TW. Percutaneous chemical ablation of desmoid tumors. J Vasc Interv Radiol. 2003 May;14(5):629-34. DOI: 10.1097/01.RVI.0000064861.87207.3E</RefTotal>
<RefLink>http://dx.doi.org/10.1097/01.RVI.0000064861.87207.3E</RefLink>
</Reference>
<Reference refNo="5">
<RefAuthor>Dalén BP</RefAuthor>
<RefAuthor>Bergh PM</RefAuthor>
<RefAuthor>Gunterberg BU</RefAuthor>
<RefTitle>Desmoid tumors: a clinical review of 30 patients with more than 20 years' follow-up</RefTitle>
<RefYear>2003</RefYear>
<RefJournal>Acta Orthop Scand</RefJournal>
<RefPage>455-9</RefPage>
<RefTotal>Dalén BP, Bergh PM, Gunterberg BU. Desmoid tumors: a clinical review of 30 patients with more than 20 years' follow-up. Acta Orthop Scand. 2003 Aug;74(4):455-9. DOI: 10.1080/00016470310017785</RefTotal>
<RefLink>http://dx.doi.org/10.1080/00016470310017785</RefLink>
</Reference>
<Reference refNo="6">
<RefAuthor>Delloye C</RefAuthor>
<RefAuthor>Viejo-Fuertes D</RefAuthor>
<RefAuthor>Scalliet P</RefAuthor>
<RefTitle>Treatment of aggressive fibromatosis: a multidisciplinary approach</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>Acta Orthop Belg</RefJournal>
<RefPage>199-203</RefPage>
<RefTotal>Delloye C, Viejo-Fuertes D, Scalliet P. Treatment of aggressive fibromatosis: a multidisciplinary approach. Acta Orthop Belg. 2004 Jun;70(3):199-203.</RefTotal>
</Reference>
<Reference refNo="7">
<RefAuthor>Denys H</RefAuthor>
<RefAuthor>De Wever O</RefAuthor>
<RefAuthor>Nusgens B</RefAuthor>
<RefAuthor>Kong Y</RefAuthor>
<RefAuthor>Sciot R</RefAuthor>
<RefAuthor>Le AT</RefAuthor>
<RefAuthor>Van Dam K</RefAuthor>
<RefAuthor>Jadidizadeh A</RefAuthor>
<RefAuthor>Tejpar S</RefAuthor>
<RefAuthor>Mareel M</RefAuthor>
<RefAuthor>Alman B</RefAuthor>
<RefAuthor>Cassiman JJ</RefAuthor>
<RefTitle>Invasion and MMP expression profile in desmoid tumours</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>Br J Cancer</RefJournal>
<RefPage>1443-9</RefPage>
<RefTotal>Denys H, De Wever O, Nusgens B, Kong Y, Sciot R, Le AT, Van Dam K, Jadidizadeh A, Tejpar S, Mareel M, Alman B, Cassiman JJ. Invasion and MMP expression profile in desmoid tumours. Br J Cancer. 2004 Apr;90(7):1443-9. DOI: 10.1038/sj.bjc.6601661</RefTotal>
<RefLink>http://dx.doi.org/10.1038/sj.bjc.6601661</RefLink>
</Reference>
<Reference refNo="8">
<RefAuthor>Duggal A</RefAuthor>
<RefAuthor>Dickinson IC</RefAuthor>
<RefAuthor>Sommerville S</RefAuthor>
<RefAuthor>Gallie P</RefAuthor>
<RefTitle>The management of extra-abdominal desmoid tumours</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>Int Orthop</RefJournal>
<RefPage>252-6</RefPage>
<RefTotal>Duggal A, Dickinson IC, Sommerville S, Gallie P. The management of extra-abdominal desmoid tumours. Int Orthop. 2004 Aug;28(4):252-6. DOI: 10.1007/s00264-004-0571-0</RefTotal>
<RefLink>http://dx.doi.org/10.1007/s00264-004-0571-0</RefLink>
</Reference>
<Reference refNo="9">
<RefAuthor>Edmonds LD</RefAuthor>
<RefAuthor>Ly JQ</RefAuthor>
<RefAuthor>LaGatta LM</RefAuthor>
<RefAuthor>Lusk JD</RefAuthor>
<RefAuthor>Beall DP</RefAuthor>
<RefTitle>Quiz case. Extraabdominal desmoid tumor of the upper arm</RefTitle>
<RefYear>2003</RefYear>
<RefJournal>Eur J Radiol</RefJournal>
<RefPage>312-5</RefPage>
<RefTotal>Edmonds LD, Ly JQ, LaGatta LM, Lusk JD, Beall DP. Quiz case. Extraabdominal desmoid tumor of the upper arm. Eur J Radiol. 2003 Dec;48(3):312-5. DOI: 10.1016/S0720-048X(03)00051-2</RefTotal>
<RefLink>http://dx.doi.org/10.1016/S0720-048X(03)00051-2</RefLink>
</Reference>
<Reference refNo="10">
<RefAuthor>Enneking WF</RefAuthor>
<RefTitle></RefTitle>
<RefYear>1983</RefYear>
<RefBookTitle>Musculoskeletal tumor surgery</RefBookTitle>
<RefPage>90-5</RefPage>
<RefTotal>Enneking WF. Musculoskeletal tumor surgery. New York: Churchill Livingstone; 1983. p. 90-5.</RefTotal>
</Reference>
<Reference refNo="11">
<RefAuthor>Erguvan-Dogan B</RefAuthor>
<RefAuthor>Dempsey PJ</RefAuthor>
<RefAuthor>Ayyar G</RefAuthor>
<RefAuthor>Gilcrease MZ</RefAuthor>
<RefTitle>Primary desmoid tumor (extraabdominal fibromatosis) of the breast</RefTitle>
<RefYear>2005</RefYear>
<RefJournal>AJR Am J Roentgenol</RefJournal>
<RefPage>488-9</RefPage>
<RefTotal>Erguvan-Dogan B, Dempsey PJ, Ayyar G, Gilcrease MZ. Primary desmoid tumor (extraabdominal fibromatosis) of the breast. AJR Am J Roentgenol. 2005 Aug;185(2):488-9. DOI: 10.2214/ajr.185.2.01850488</RefTotal>
<RefLink>http://dx.doi.org/10.2214/ajr.185.2.01850488</RefLink>
</Reference>
<Reference refNo="12">
<RefAuthor>Goldwirth M</RefAuthor>
<RefAuthor>Kollender Y</RefAuthor>
<RefAuthor>Goodwin DR</RefAuthor>
<RefTitle>Desmoid tumour of the hand</RefTitle>
<RefYear>2002</RefYear>
<RefJournal>Hand Surg</RefJournal>
<RefPage>151-2</RefPage>
<RefTotal>Goldwirth M, Kollender Y, Goodwin DR. Desmoid tumour of the hand. Hand Surg. 2002 Jul;7(1):151-2. DOI: 10.1142/S0218810402000923</RefTotal>
<RefLink>http://dx.doi.org/10.1142/S0218810402000923</RefLink>
</Reference>
<Reference refNo="13">
<RefAuthor>Gwynne-Jones DP</RefAuthor>
<RefAuthor>Theis JC</RefAuthor>
<RefAuthor>Jeffery AK</RefAuthor>
<RefAuthor>Hung NA</RefAuthor>
<RefTitle>Long-term follow-up of a recurrent multifocal desmoid tumour treated with tamoxifen: a case report</RefTitle>
<RefYear>2005</RefYear>
<RefJournal>J Orthop Surg (Hong Kong)</RefJournal>
<RefPage>174-7</RefPage>
<RefTotal>Gwynne-Jones DP, Theis JC, Jeffery AK, Hung NA. Long-term follow-up of a recurrent multifocal desmoid tumour treated with tamoxifen: a case report. J Orthop Surg (Hong Kong). 2005 Aug;13(2):174-7.</RefTotal>
</Reference>
<Reference refNo="14">
<RefAuthor>Hosalkar HS</RefAuthor>
<RefAuthor>Fox EJ</RefAuthor>
<RefAuthor>Delaney T</RefAuthor>
<RefAuthor>Torbert JT</RefAuthor>
<RefAuthor>Ogilvie CM</RefAuthor>
<RefAuthor>Lackman RD</RefAuthor>
<RefTitle>Desmoid tumors and current status of management</RefTitle>
<RefYear>2006</RefYear>
<RefJournal>Orthop Clin North Am</RefJournal>
<RefPage>53-63</RefPage>
<RefTotal>Hosalkar HS, Fox EJ, Delaney T, Torbert JT, Ogilvie CM, Lackman RD. Desmoid tumors and current status of management. Orthop Clin North Am. 2006 Jan;37(1):53-63. DOI: 10.1016/j.ocl.2005.08.004</RefTotal>
<RefLink>http://dx.doi.org/10.1016/j.ocl.2005.08.004</RefLink>
</Reference>
<Reference refNo="15">
<RefAuthor>Kadwa S</RefAuthor>
<RefTitle>Juvenile aggressive fibromatosis</RefTitle>
<RefYear>2003</RefYear>
<RefJournal>SADJ</RefJournal>
<RefPage>299-302</RefPage>
<RefTotal>Kadwa S. Juvenile aggressive fibromatosis. SADJ. 2003 Aug;58(7):299-302.</RefTotal>
</Reference>
<Reference refNo="16">
<RefAuthor>Kogelnik HD</RefAuthor>
<RefTitle>Chirurgie versus Radiotherapie für Patienten mit aggressiver Fibromatose oder Desmoidtumoren. Eine vergleichende Übersicht von 22 Arbeiten</RefTitle>
<RefYear>2000</RefYear>
<RefJournal>Strahlenther Onkol</RefJournal>
<RefPage>541-2</RefPage>
<RefTotal>Kogelnik HD. Chirurgie versus Radiotherapie für Patienten mit aggressiver Fibromatose oder Desmoidtumoren. Eine vergleichende Übersicht von 22 Arbeiten. Strahlenther Onkol. 2000;176(11):541-2.</RefTotal>
</Reference>
<Reference refNo="17">
<RefAuthor>Kuhnen C</RefAuthor>
<RefAuthor>Helwing M</RefAuthor>
<RefAuthor>Rabstein S</RefAuthor>
<RefAuthor>Homann HH</RefAuthor>
<RefAuthor>Müller KM</RefAuthor>
<RefTitle>Desmoidfibromatosen (aggressive Fibromatosen). Klinisch-pathologische Korrelationen und Differenzialdiagnose spindelzelliger fibroblastisch/myofibroblastischer Tumoren des Weichgewebes</RefTitle>
<RefYear>2005</RefYear>
<RefJournal>Pathologe</RefJournal>
<RefPage>117-26</RefPage>
<RefTotal>Kuhnen C, Helwing M, Rabstein S, Homann HH, Müller KM. Desmoidfibromatosen (aggressive Fibromatosen). Klinisch-pathologische Korrelationen und Differenzialdiagnose spindelzelliger fibroblastisch/myofibroblastischer Tumoren des Weichgewebes [Desmoid-type fibromatosis (aggressive fibromatosis)]. Pathologe. 2005 Mar;26(2):117-26. DOI: 10.1007/s00292-004-0742-1</RefTotal>
<RefLink>http://dx.doi.org/10.1007/s00292-004-0742-1</RefLink>
</Reference>
<Reference refNo="18">
<RefAuthor>Leithner A</RefAuthor>
<RefAuthor>Gapp M</RefAuthor>
<RefAuthor>Leithner K</RefAuthor>
<RefAuthor>Radl R</RefAuthor>
<RefAuthor>Krippl P</RefAuthor>
<RefAuthor>Beham A</RefAuthor>
<RefAuthor>Windhager R</RefAuthor>
<RefTitle>Margins in extra-abdominal desmoid tumors: a comparative analysis</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>J Surg Oncol</RefJournal>
<RefPage>152-6</RefPage>
<RefTotal>Leithner A, Gapp M, Leithner K, Radl R, Krippl P, Beham A, Windhager R. Margins in extra-abdominal desmoid tumors: a comparative analysis. J Surg Oncol. 2004 Jun;86(3):152-6. DOI: 10.1002/jso.20057</RefTotal>
<RefLink>http://dx.doi.org/10.1002/jso.20057</RefLink>
</Reference>
<Reference refNo="19">
<RefAuthor>Meller J</RefAuthor>
<RefAuthor>Lehmann K</RefAuthor>
<RefAuthor>Altenvoerde G</RefAuthor>
<RefAuthor>Jauho A</RefAuthor>
<RefAuthor>Becker W</RefAuthor>
<RefTitle>F-18-FDG PET zum Nachweis einer aggressiven Fibromatose</RefTitle>
<RefYear>2000</RefYear>
<RefJournal>Nuklearmedizin</RefJournal>
<RefPage>N115-7</RefPage>
<RefTotal>Meller J, Lehmann K, Altenvoerde G, Jauho A, Becker W. F-18-FDG PET zum Nachweis einer aggressiven Fibromatose [F-18-FDG PET for the detection of aggressive fibromatosis]. Nuklearmedizin. 2000 Dec;39(8):N115-7.</RefTotal>
</Reference>
<Reference refNo="20">
<RefAuthor>Mendenhall WM</RefAuthor>
<RefAuthor>Zlotecki RA</RefAuthor>
<RefAuthor>Morris CG</RefAuthor>
<RefAuthor>Hochwald SN</RefAuthor>
<RefAuthor>Scarborough MT</RefAuthor>
<RefTitle>Aggressive fibromatosis</RefTitle>
<RefYear>2005</RefYear>
<RefJournal>Am J Clin Oncol</RefJournal>
<RefPage>211-5</RefPage>
<RefTotal>Mendenhall WM, Zlotecki RA, Morris CG, Hochwald SN, Scarborough MT. Aggressive fibromatosis. Am J Clin Oncol. 2005 Apr;28(2):211-5. DOI: 10.1097/01.coc.0000144817.78549.53</RefTotal>
<RefLink>http://dx.doi.org/10.1097/01.coc.0000144817.78549.53</RefLink>
</Reference>
<Reference refNo="21">
<RefAuthor>Merchant NB</RefAuthor>
<RefAuthor>Lewis JJ</RefAuthor>
<RefAuthor>Woodruff JM</RefAuthor>
<RefAuthor>Leung DH</RefAuthor>
<RefAuthor>Brennan MF</RefAuthor>
<RefTitle>Extremity and trunk desmoid tumors: a multifactorial analysis of outcome</RefTitle>
<RefYear>1999</RefYear>
<RefJournal>Cancer</RefJournal>
<RefPage>2045-52</RefPage>
<RefTotal>Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999 Nov 15;86(10):2045-52. DOI: 10.1002/(SICI)1097-0142(19991115)86:10<2045::AID-CNCR23>3.0.CO;2-F</RefTotal>
<RefLink>http://dx.doi.org/10.1002/(SICI)1097-0142(19991115)86:10<2045::AID-CNCR23>3.0.CO;2-F</RefLink>
</Reference>
<Reference refNo="22">
<RefAuthor>Micke O</RefAuthor>
<RefAuthor>Seegenschmiedt MH</RefAuthor>
<RefAuthor> German Cooperative Group on Radiotherapy for Benign Diseases</RefAuthor>
<RefTitle>Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national Patterns of Care Study</RefTitle>
<RefYear>2005</RefYear>
<RefJournal>Int J Radiat Oncol Biol Phys</RefJournal>
<RefPage>882-91</RefPage>
<RefTotal>Micke O, Seegenschmiedt MH; German Cooperative Group on Radiotherapy for Benign Diseases. Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national Patterns of Care Study. Int J Radiat Oncol Biol Phys. 2005 Mar;61(3):882-91. DOI: 10.1016/j.ijrobp.2004.07.705</RefTotal>
<RefLink>http://dx.doi.org/10.1016/j.ijrobp.2004.07.705</RefLink>
</Reference>
<Reference refNo="23">
<RefAuthor>Nuyttens JJ</RefAuthor>
<RefAuthor>Rust PF</RefAuthor>
<RefAuthor>Thomas CR Jr</RefAuthor>
<RefAuthor>Turrisi AT 3rd</RefAuthor>
<RefTitle>Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articles</RefTitle>
<RefYear>2000</RefYear>
<RefJournal>Cancer</RefJournal>
<RefPage>1517-23</RefPage>
<RefTotal>Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articles. Cancer. 2000 Apr 1;88(7):1517-23. DOI: 10.1002/(SICI)1097-0142(20000401)88:7<1517::AID-CNCR3>3.0.CO;2-9[uid] </RefTotal>
<RefLink>http://dx.doi.org/10.1002/(SICI)1097-0142(20000401)88:7<1517::AID-CNCR3>3.0.CO;2-9[uid]</RefLink>
</Reference>
<Reference refNo="24">
<RefAuthor>Pakos EE</RefAuthor>
<RefAuthor>Tsekeris PG</RefAuthor>
<RefAuthor>Goussia AC</RefAuthor>
<RefTitle>Desmoid tumours of the extremities and trunk: a review of the literature</RefTitle>
<RefYear>2005</RefYear>
<RefJournal>Int Orthop</RefJournal>
<RefPage>210-3</RefPage>
<RefTotal>Pakos EE, Tsekeris PG, Goussia AC. Desmoid tumours of the extremities and trunk: a review of the literature. Int Orthop. 2005 Aug;29(4):210-3. DOI: 10.1007/s00264-005-0641-y</RefTotal>
<RefLink>http://dx.doi.org/10.1007/s00264-005-0641-y</RefLink>
</Reference>
<Reference refNo="25">
<RefAuthor>Park HC</RefAuthor>
<RefAuthor>Pyo HR</RefAuthor>
<RefAuthor>Shin KH</RefAuthor>
<RefAuthor>Suh CO</RefAuthor>
<RefTitle>Radiation treatment for aggressive fibromatosis: findings from observed patterns of local failure</RefTitle>
<RefYear>2003</RefYear>
<RefJournal>Oncology</RefJournal>
<RefPage>346-52</RefPage>
<RefTotal>Park HC, Pyo HR, Shin KH, Suh CO. Radiation treatment for aggressive fibromatosis: findings from observed patterns of local failure. Oncology. 2003;64(4):346-52. DOI: 10.1159/000070292</RefTotal>
<RefLink>http://dx.doi.org/10.1159/000070292</RefLink>
</Reference>
<Reference refNo="26">
<RefAuthor>Peterschulte G</RefAuthor>
<RefAuthor>Lickfeld T</RefAuthor>
<RefAuthor>Möslein G</RefAuthor>
<RefTitle>Das Desmoid-Problem</RefTitle>
<RefYear>2000</RefYear>
<RefJournal>Chirurg</RefJournal>
<RefPage>894-903</RefPage>
<RefTotal>Peterschulte G, Lickfeld T, Möslein G. Das Desmoid-Problem [The desmoid problem]. Chirurg. 2000 Aug;71(8):894-903. DOI: 10.1007/s001040051153</RefTotal>
<RefLink>http://dx.doi.org/10.1007/s001040051153</RefLink>
</Reference>
<Reference refNo="27">
<RefAuthor>Picariello L</RefAuthor>
<RefAuthor>Tonelli F</RefAuthor>
<RefAuthor>Brandi ML</RefAuthor>
<RefTitle>Selective oestrogen receptor modulators in desmoid tumours</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>Expert Opin Investig Drugs</RefJournal>
<RefPage>1457-68</RefPage>
<RefTotal>Picariello L, Tonelli F, Brandi ML. Selective oestrogen receptor modulators in desmoid tumours. Expert Opin Investig Drugs. 2004 Nov;13(11):1457-68. DOI: 10.1517/13543784.13.11.1457</RefTotal>
<RefLink>http://dx.doi.org/10.1517/13543784.13.11.1457</RefLink>
</Reference>
<Reference refNo="28">
<RefAuthor>Pignatti G</RefAuthor>
<RefAuthor>Barbanti-Bròdano G</RefAuthor>
<RefAuthor>Ferrari D</RefAuthor>
<RefAuthor>Gherlinzoni F</RefAuthor>
<RefAuthor>Bertoni F</RefAuthor>
<RefAuthor>Bacchini P</RefAuthor>
<RefAuthor>Barbieri E</RefAuthor>
<RefAuthor>Giunti A</RefAuthor>
<RefAuthor>Campanacci M</RefAuthor>
<RefTitle>Extraabdominal desmoid tumor. A study of 83 cases</RefTitle>
<RefYear>2000</RefYear>
<RefJournal>Clin Orthop Relat Res</RefJournal>
<RefPage>207-13</RefPage>
<RefTotal>Pignatti G, Barbanti-Bròdano G, Ferrari D, Gherlinzoni F, Bertoni F, Bacchini P, Barbieri E, Giunti A, Campanacci M. Extraabdominal desmoid tumor. A study of 83 cases. Clin Orthop Relat Res. 2000 Jun;(375):207-13. DOI: 10.1097/00003086-200006000-00025</RefTotal>
<RefLink>http://dx.doi.org/10.1097/00003086-200006000-00025</RefLink>
</Reference>
<Reference refNo="29">
<RefAuthor>Pritchard DJ</RefAuthor>
<RefAuthor>Nascimento AG</RefAuthor>
<RefAuthor>Petersen IA</RefAuthor>
<RefTitle>Local control of extra-abdominal desmoid tumors</RefTitle>
<RefYear>1996</RefYear>
<RefJournal>J Bone Joint Surg Am</RefJournal>
<RefPage>848-54</RefPage>
<RefTotal>Pritchard DJ, Nascimento AG, Petersen IA. Local control of extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1996 Jun;78(6):848-54.</RefTotal>
</Reference>
<Reference refNo="30">
<RefAuthor>Reitamo JJ</RefAuthor>
<RefAuthor>Scheinin TM</RefAuthor>
<RefAuthor>Häyry P</RefAuthor>
<RefTitle>The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor</RefTitle>
<RefYear>1986</RefYear>
<RefJournal>Am J Surg</RefJournal>
<RefPage>230-7</RefPage>
<RefTotal>Reitamo JJ, Scheinin TM, Häyry P. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg. 1986 Feb;151(2):230-7. DOI: 10.1016/0002-9610(86)90076-0 </RefTotal>
<RefLink>http://dx.doi.org/10.1016/0002-9610(86)90076-0</RefLink>
</Reference>
<Reference refNo="31">
<RefAuthor>Rock MG</RefAuthor>
<RefAuthor>Pritchard DJ</RefAuthor>
<RefAuthor>Reiman HM</RefAuthor>
<RefAuthor>Soule EH</RefAuthor>
<RefAuthor>Brewster RC</RefAuthor>
<RefTitle>Extra-abdominal desmoid tumors</RefTitle>
<RefYear>1984</RefYear>
<RefJournal>J Bone Joint Surg Am</RefJournal>
<RefPage>1369-74</RefPage>
<RefTotal>Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984 Dec;66(9):1369-74.</RefTotal>
</Reference>
<Reference refNo="32">
<RefAuthor>Schlemmer M</RefAuthor>
<RefTitle>Desmoid tumors and deep fibromatoses</RefTitle>
<RefYear>2005</RefYear>
<RefJournal>Hematol Oncol Clin North Am</RefJournal>
<RefPage>565-71, vii-viii</RefPage>
<RefTotal>Schlemmer M. Desmoid tumors and deep fibromatoses. Hematol Oncol Clin North Am. 2005 Jun;19(3):565-71, vii-viii. DOI: 10.1016/j.hoc.2005.03.008</RefTotal>
<RefLink>http://dx.doi.org/10.1016/j.hoc.2005.03.008</RefLink>
</Reference>
<Reference refNo="33">
<RefAuthor>Schulz-Ertner D</RefAuthor>
<RefAuthor>Zierhut D</RefAuthor>
<RefAuthor>Mende U</RefAuthor>
<RefAuthor>Harms W</RefAuthor>
<RefAuthor>Branitzki P</RefAuthor>
<RefAuthor>Wannenmacher M</RefAuthor>
<RefTitle>The role of radiation therapy in the management of desmoid tumors</RefTitle>
<RefYear>2002</RefYear>
<RefJournal>Strahlenther Onkol</RefJournal>
<RefPage>78-83</RefPage>
<RefTotal>Schulz-Ertner D, Zierhut D, Mende U, Harms W, Branitzki P, Wannenmacher M. The role of radiation therapy in the management of desmoid tumors. Strahlenther Onkol. 2002 Feb;178(2):78-83. DOI: 10.1007/s00066-002-0900-4</RefTotal>
<RefLink>http://dx.doi.org/10.1007/s00066-002-0900-4</RefLink>
</Reference>
<Reference refNo="34">
<RefAuthor>Shields CJ</RefAuthor>
<RefAuthor>Winter DC</RefAuthor>
<RefAuthor>Kirwan WO</RefAuthor>
<RefAuthor>Redmond HP</RefAuthor>
<RefTitle>Desmoid tumours</RefTitle>
<RefYear>2001</RefYear>
<RefJournal>Eur J Surg Oncol</RefJournal>
<RefPage>701-6</RefPage>
<RefTotal>Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol. 2001 Dec;27(8):701-6. DOI: 10.1053/ejso.2001.1169</RefTotal>
<RefLink>http://dx.doi.org/10.1053/ejso.2001.1169</RefLink>
</Reference>
<Reference refNo="35">
<RefAuthor>Sikka RS</RefAuthor>
<RefAuthor>Vora M</RefAuthor>
<RefAuthor>Edwards TB</RefAuthor>
<RefAuthor>Szabo I</RefAuthor>
<RefAuthor>Walch G</RefAuthor>
<RefTitle>Desmoid tumor of the subscapularis presenting as isolated loss of external rotation of the shoulder. A report of two cases</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>J Bone Joint Surg Am</RefJournal>
<RefPage>159-64</RefPage>
<RefTotal>Sikka RS, Vora M, Edwards TB, Szabo I, Walch G. Desmoid tumor of the subscapularis presenting as isolated loss of external rotation of the shoulder. A report of two cases. J Bone Joint Surg Am. 2004 Jan;86-A(1):159-64.</RefTotal>
</Reference>
<Reference refNo="36">
<RefAuthor>Smith SE</RefAuthor>
<RefAuthor>Kransdorf MJ</RefAuthor>
<RefTitle>Primary musculoskeletal tumors of fibrous origin</RefTitle>
<RefYear>2000</RefYear>
<RefJournal>Semin Musculoskelet Radiol</RefJournal>
<RefPage>73-88</RefPage>
<RefTotal>Smith SE, Kransdorf MJ. Primary musculoskeletal tumors of fibrous origin. Semin Musculoskelet Radiol. 2000;4(1):73-88.</RefTotal>
</Reference>
<Reference refNo="37">
<RefAuthor>Sørensen A</RefAuthor>
<RefAuthor>Keller J</RefAuthor>
<RefAuthor>Nielsen OS</RefAuthor>
<RefAuthor>Jensen OM</RefAuthor>
<RefTitle>Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1-27 years</RefTitle>
<RefYear>2002</RefYear>
<RefJournal>Acta Orthop Scand</RefJournal>
<RefPage>213-9</RefPage>
<RefTotal>Sørensen A, Keller J, Nielsen OS, Jensen OM. Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1-27 years. Acta Orthop Scand. 2002 Apr;73(2):213-9. DOI: 10.1080/000164702753671830</RefTotal>
<RefLink>http://dx.doi.org/10.1080/000164702753671830</RefLink>
</Reference>
<Reference refNo="38">
<RefAuthor>Spear MA</RefAuthor>
<RefAuthor>Jennings LC</RefAuthor>
<RefAuthor>Mankin HJ</RefAuthor>
<RefAuthor>Spiro IJ</RefAuthor>
<RefAuthor>Springfield DS</RefAuthor>
<RefAuthor>Gebhardt MC</RefAuthor>
<RefAuthor>Rosenberg AE</RefAuthor>
<RefAuthor>Efird JT</RefAuthor>
<RefAuthor>Suit HD</RefAuthor>
<RefTitle>Individualizing management of aggressive fibromatoses</RefTitle>
<RefYear>1998</RefYear>
<RefJournal>Int J Radiat Oncol Biol Phys</RefJournal>
<RefPage>637-45</RefPage>
<RefTotal>Spear MA, Jennings LC, Mankin HJ, Spiro IJ, Springfield DS, Gebhardt MC, Rosenberg AE, Efird JT, Suit HD. Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys. 1998 Feb 1;40(3):637-45. DOI: 10.1016/S0360-3016(97)00845-6 </RefTotal>
<RefLink>http://dx.doi.org/10.1016/S0360-3016(97)00845-6</RefLink>
</Reference>
<Reference refNo="39">
<RefAuthor>Suit H</RefAuthor>
<RefAuthor>Spiro I</RefAuthor>
<RefTitle>Radiation in the multidisciplinary management of desmoid tumors</RefTitle>
<RefYear>2001</RefYear>
<RefJournal>Front Radiat Ther Oncol</RefJournal>
<RefPage>107-19</RefPage>
<RefTotal>Suit H, Spiro I. Radiation in the multidisciplinary management of desmoid tumors. Front Radiat Ther Oncol. 2001;35:107-19. DOI: 10.1159/000061271</RefTotal>
<RefLink>http://dx.doi.org/10.1159/000061271</RefLink>
</Reference>
<Reference refNo="40">
<RefAuthor>Tanaka H</RefAuthor>
<RefAuthor>Harasawa A</RefAuthor>
<RefAuthor>Furui S</RefAuthor>
<RefTitle>Usefulness of MR imaging in assessment of tumor extent of aggressive fibromatosis</RefTitle>
<RefYear>2005</RefYear>
<RefJournal>Radiat Med</RefJournal>
<RefPage>111-5</RefPage>
<RefTotal>Tanaka H, Harasawa A, Furui S. Usefulness of MR imaging in assessment of tumor extent of aggressive fibromatosis. Radiat Med. 2005 Mar;23(2):111-5.</RefTotal>
</Reference>
<Reference refNo="41">
<RefAuthor>Wagstaff MJ</RefAuthor>
<RefAuthor>Raurell A</RefAuthor>
<RefAuthor>Perks AG</RefAuthor>
<RefTitle>Multicentric extra-abdominal desmoid tumours</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>Br J Plast Surg</RefJournal>
<RefPage>362-5</RefPage>
<RefTotal>Wagstaff MJ, Raurell A, Perks AG. Multicentric extra-abdominal desmoid tumours. Br J Plast Surg. 2004 Jun;57(4):362-5. DOI: 10.1016/j.bjps.2004.02.014</RefTotal>
<RefLink>http://dx.doi.org/10.1016/j.bjps.2004.02.014</RefLink>
</Reference>
<Reference refNo="42">
<RefAuthor>Watanabe T</RefAuthor>
<RefAuthor>Suenaga N</RefAuthor>
<RefAuthor>Minami A</RefAuthor>
<RefTitle>Extra-abdominal desmoid around the shoulder joint: one of the differential diagnoses of frozen shoulder</RefTitle>
<RefYear>2004</RefYear>
<RefJournal>J Shoulder Elbow Surg</RefJournal>
<RefPage>90-4</RefPage>
<RefTotal>Watanabe T, Suenaga N, Minami A. Extra-abdominal desmoid around the shoulder joint: one of the differential diagnoses of frozen shoulder. J Shoulder Elbow Surg. 2004 Jan-Feb;13(1):90-4. DOI: 10.1016/S1058-2746(03)00054-5</RefTotal>
<RefLink>http://dx.doi.org/10.1016/S1058-2746(03)00054-5</RefLink>
</Reference>
<Reference refNo="43">
<RefAuthor>Weiss RJ</RefAuthor>
<RefAuthor>Treiber M</RefAuthor>
<RefAuthor>Zahlten-Hinguranage A</RefAuthor>
<RefAuthor>Bernd L</RefAuthor>
<RefTitle>Verbesserung der lokalen Kontrolle bei Patienten mit aggressiver Fibromatose durch die Kombination von Chirurgie und Strahlentherapie</RefTitle>
<RefYear>2002</RefYear>
<RefJournal>Chirurg</RefJournal>
<RefPage>615-21</RefPage>
<RefTotal>Weiss RJ, Treiber M, Zahlten-Hinguranage A, Bernd L. Verbesserung der lokalen Kontrolle bei Patienten mit aggressiver Fibromatose durch die Kombination von Chirurgie und Strahlentherapie [Improving local control in patients with aggressive fibromatosis by combined surgery and radiotherapy]. Chirurg. 2002 Jun;73(6):615-21. DOI: 10.1007/s00104-002-0441-5</RefTotal>
<RefLink>http://dx.doi.org/10.1007/s00104-002-0441-5</RefLink>
</Reference>
<Reference refNo="44">
<RefAuthor>Weiss SW</RefAuthor>
<RefAuthor>Goldblum JR</RefAuthor>
<RefTitle></RefTitle>
<RefYear>2001</RefYear>
<RefBookTitle>Enzinger and Weiss’s Soft Tissue Tumors</RefBookTitle>
<RefPage>320-9</RefPage>
<RefTotal>Weiss SW, Goldblum JR, eds. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. Missouri: Mosby; 2001. p. 320-9.</RefTotal>
</Reference>
<Reference refNo="45">
<RefAuthor>Zollinger HU</RefAuthor>
<RefTitle></RefTitle>
<RefYear>1969</RefYear>
<RefBookTitle>Pathologische Anatomie</RefBookTitle>
<RefPage>465</RefPage>
<RefTotal>Zollinger HU. Pathologische Anatomie. 2. ed. Stuttgart: Georg Thieme Verlag; 1969. p. 465.</RefTotal>
</Reference>
</References>
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<Caption><Pgraph><Mark1>Table 1: DES-Classification according to Peterschulte et al. [26] (E: extraabdominal, AW: abdominal wall, M1: mesenterium without obstruction, M2: mesenterium with obstruction)</Mark1></Pgraph></Caption>
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<Caption><Pgraph><Mark1>Figure 1: Desmoid fibromatosis of the shoulder and axillary region with local aggressive infiltration </Mark1></Pgraph><Pgraph>(Courtesy of the Department of Radiology of the Leipzig University)</Pgraph></Caption>
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<Caption><Pgraph><Mark1>Figure 2: Benign appearance with a well differentiated composition of intersecting bundles of fibroblasts, which are separated by collagen deposits</Mark1></Pgraph><Pgraph>(Courtesy of the Institute of Pathology of Leipzig University)</Pgraph></Caption>
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<Caption><Pgraph><Mark1>Figure 3: Intraoperative appearance</Mark1></Pgraph></Caption>
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<Caption><Pgraph><Mark1>Figure 4: Macroscopic appearance</Mark1></Pgraph></Caption>
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