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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Mesenteric desmoid tumour presenting with recurrent abdominal abscess and duodenal fistula: A case report and review of literature</title><author><name sortKey="Huang, Kai" sort="Huang, Kai" uniqKey="Huang K" first="Kai" last="Huang">Kai Huang</name><affiliation><nlm:aff id="aff0005">Department of Surgery, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author><author><name sortKey="Stuart, Heather" sort="Stuart, Heather" uniqKey="Stuart H" first="Heather" last="Stuart">Heather Stuart</name><affiliation><nlm:aff id="aff0005">Department of Surgery, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author><author><name sortKey="Lyapichev, Kirill" sort="Lyapichev, Kirill" uniqKey="Lyapichev K" first="Kirill" last="Lyapichev">Kirill Lyapichev</name><affiliation><nlm:aff id="aff0010">Department of Pathology and Laboratory Medicine, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author><author><name sortKey="Rosenberg, Andrew E" sort="Rosenberg, Andrew E" uniqKey="Rosenberg A" first="Andrew E." last="Rosenberg">Andrew E. Rosenberg</name><affiliation><nlm:aff id="aff0010">Department of Pathology and Laboratory Medicine, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author><author><name sortKey="Livingstone, Alan S" sort="Livingstone, Alan S" uniqKey="Livingstone A" first="Alan S." last="Livingstone">Alan S. Livingstone</name><affiliation><nlm:aff id="aff0005">Department of Surgery, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">28666150</idno><idno type="pmc">5491487</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5491487</idno><idno type="RBID">PMC:5491487</idno><idno type="doi">10.1016/j.ijscr.2017.06.007</idno><date when="2017">2017</date><idno type="wicri:Area/Pmc/Corpus">002278</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002278</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Mesenteric desmoid tumour presenting with recurrent abdominal abscess and duodenal fistula: A case report and review of literature</title><author><name sortKey="Huang, Kai" sort="Huang, Kai" uniqKey="Huang K" first="Kai" last="Huang">Kai Huang</name><affiliation><nlm:aff id="aff0005">Department of Surgery, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author><author><name sortKey="Stuart, Heather" sort="Stuart, Heather" uniqKey="Stuart H" first="Heather" last="Stuart">Heather Stuart</name><affiliation><nlm:aff id="aff0005">Department of Surgery, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author><author><name sortKey="Lyapichev, Kirill" sort="Lyapichev, Kirill" uniqKey="Lyapichev K" first="Kirill" last="Lyapichev">Kirill Lyapichev</name><affiliation><nlm:aff id="aff0010">Department of Pathology and Laboratory Medicine, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author><author><name sortKey="Rosenberg, Andrew E" sort="Rosenberg, Andrew E" uniqKey="Rosenberg A" first="Andrew E." last="Rosenberg">Andrew E. Rosenberg</name><affiliation><nlm:aff id="aff0010">Department of Pathology and Laboratory Medicine, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author><author><name sortKey="Livingstone, Alan S" sort="Livingstone, Alan S" uniqKey="Livingstone A" first="Alan S." last="Livingstone">Alan S. Livingstone</name><affiliation><nlm:aff id="aff0005">Department of Surgery, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</nlm:aff></affiliation></author></analytic><series><title level="j">International Journal of Surgery Case Reports</title><idno type="eISSN">2210-2612</idno><imprint><date when="2017">2017</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><title>Highlights</title><p><list list-type="simple"><list-item id="lsti0005"><label>•</label><p>Mesenteric desmoid is a rare benign tumor, usually asymptomatic, but can grow aggressively and cause complications.</p></list-item><list-item id="lsti0010"><label>•</label><p>Recurrent intralesional abscess formation even after drainage and medical therapy should lead to the assumption of fistulization to the adjacent bowel.</p></list-item><list-item id="lsti0015"><label>•</label><p>Treatment should be guided by a multidisciplinary team, and operation intervention should be performed by surgeons familiar with desmoid tumor biology.</p></list-item></list></p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Agha, R A" uniqKey="Agha R">R.A. 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<front><journal-meta><journal-id journal-id-type="nlm-ta">Int J Surg Case Rep</journal-id><journal-id journal-id-type="iso-abbrev">Int J Surg Case Rep</journal-id><journal-title-group><journal-title>International Journal of Surgery Case Reports</journal-title></journal-title-group><issn pub-type="epub">2210-2612</issn><publisher><publisher-name>Elsevier</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">28666150</article-id><article-id pub-id-type="pmc">5491487</article-id><article-id pub-id-type="publisher-id">S2210-2612(17)30259-6</article-id><article-id pub-id-type="doi">10.1016/j.ijscr.2017.06.007</article-id><article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject></subj-group></article-categories><title-group><article-title>Mesenteric desmoid tumour presenting with recurrent abdominal abscess and duodenal fistula: A case report and review of literature</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Huang</surname><given-names>Kai</given-names></name><email>hankiefd@gmail.com</email><xref rid="aff0005" ref-type="aff">a</xref><xref rid="cor0005" ref-type="corresp">⁎</xref></contrib><contrib contrib-type="author"><name><surname>Stuart</surname><given-names>Heather</given-names></name><xref rid="aff0005" ref-type="aff">a</xref></contrib><contrib contrib-type="author"><name><surname>Lyapichev</surname><given-names>Kirill</given-names></name><xref rid="aff0010" ref-type="aff">b</xref></contrib><contrib contrib-type="author"><name><surname>Rosenberg</surname><given-names>Andrew E.</given-names></name><xref rid="aff0010" ref-type="aff">b</xref></contrib><contrib contrib-type="author"><name><surname>Livingstone</surname><given-names>Alan S.</given-names></name><xref rid="aff0005" ref-type="aff">a</xref></contrib></contrib-group><aff id="aff0005"><label>a</label>Department of Surgery, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</aff><aff id="aff0010"><label>b</label>Department of Pathology and Laboratory Medicine, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA</aff><author-notes><corresp id="cor0005"><label>⁎</label>Corresponding author. <email>hankiefd@gmail.com</email></corresp></author-notes><pub-date pub-type="pmc-release"><day>16</day><month>6</month><year>2017</year></pub-date><pmc-comment> PMC Release delay is 0 months and 0 days and was based on .</pmc-comment>
<pub-date pub-type="collection"><year>2017</year></pub-date><pub-date pub-type="epub"><day>16</day><month>6</month><year>2017</year></pub-date><volume>37</volume><fpage>119</fpage><lpage>123</lpage><history><date date-type="received"><day>20</day><month>4</month><year>2017</year></date><date date-type="rev-recd"><day>1</day><month>6</month><year>2017</year></date><date date-type="accepted"><day>4</day><month>6</month><year>2017</year></date></history><permissions><license license-type="CC BY-NC-ND" xlink:href="http://creativecommons.org/licenses/by-nc-nd/4.0/"><license-p>This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).</license-p></license></permissions><abstract abstract-type="author-highlights" id="abs0005"><title>Highlights</title><p><list list-type="simple"><list-item id="lsti0005"><label>•</label><p>Mesenteric desmoid is a rare benign tumor, usually asymptomatic, but can grow aggressively and cause complications.</p></list-item><list-item id="lsti0010"><label>•</label><p>Recurrent intralesional abscess formation even after drainage and medical therapy should lead to the assumption of fistulization to the adjacent bowel.</p></list-item><list-item id="lsti0015"><label>•</label><p>Treatment should be guided by a multidisciplinary team, and operation intervention should be performed by surgeons familiar with desmoid tumor biology.</p></list-item></list></p></abstract><abstract id="abs0010"><sec><title>Introduction</title><p>Desmoid tumors are locally destructive but histologically benign. Their management involves close observation and surgical, medical, or hormonal treatment.</p></sec><sec><title>Presentation of the case</title><p>A 36-year-old male was admitted for abdominal pain and fever. A CT scan showed fluid collections and air within a mesenteric mass. Diagnostic laparotomy was performed with drainage of the abscess and biopsy of the mass. The pathology suggested a desmoid tumor. His fever and abdominal pain persisted. An endoscopy was performed, which demonstrated a fistula track in the third part of the duodenum. After a multidisciplinary discussion, consensus was to pursue surgical intervention. The patient underwent an en bloc resection of the tumor including a portion of the wall of the third part of the duodenum. The final pathology confirmed a desmoid tumor with a fistula track to the duodenum. The patient had a re-laparotomy on POD2 for intra-abdominal bleeding but was discharged without further events on POD7. He had no evidence of recurrence on follow-up at 11 months.</p></sec><sec><title>Discussion</title><p>Desmoid tumors are rarely complicated by abscess formation or fistulization. The management of intra-abdominal desmoids in this setting is challenging, as patients are often symptomatic and unresponsive to medical management. Percutaneous drainage and antibiotics are often initiated as first-line treatment, followed by surgery or medical therapy after evaluation of resectability and tumor stage.</p></sec><sec><title>Conclusion</title><p>Rare complications can arise with intra-abdominal desmoid tumors. Principles of infection control should be applied in combination with optimization of oncologic outcome. A multidisciplinary approach helps to achieve both these objectives.</p></sec></abstract><kwd-group id="kwd0005"><title>Keywords</title><kwd>Desmoid tumor</kwd><kwd>Fistula</kwd><kwd>Abscess formation</kwd><kwd>Treatment</kwd><kwd>Case report</kwd></kwd-group></article-meta></front><body><sec id="sec0005"><label>1</label><title>Introduction</title><p>The work presented in this case study has been reported in line with the SCARE criteria <xref rid="bib0005" ref-type="bibr">[1]</xref>.</p><p>Desmoid tumors, also known as aggressive fibromatosis, are locally destructive but histologically benign fibroblastic neoplasms that originate from any musculoaponeurotic or fascial structure in the body. They can occur sporadically or in association with familial adenomatous polyposis (FAP). Desmoids are categorized anatomically as intra-abdominal, abdominal wall, and extra-abdominal. Intra-abdominal desmoids typically occur in the intestinal mesentery and can cause complications such as obstruction, perforation, abscess formation, and hemorrhage <xref rid="bib0010" ref-type="bibr">[2]</xref>, <xref rid="bib0015" ref-type="bibr">[3]</xref>, <xref rid="bib0020" ref-type="bibr">[4]</xref>. This case describes a mesenteric desmoid that fistulized into the duodenum causing chronic abscess formation. It is rare for mesenteric fibromatosis to present with fistula and few cases have been previously reported <xref rid="bib0025" ref-type="bibr">[5]</xref>, <xref rid="bib0030" ref-type="bibr">[6]</xref>. This case report discusses the optimal treatment of this situation and provides a literature review of the topic.</p><sec id="sec0010"><label>1.1</label><title>Patient information</title><p>A 36-year-old Caucasian male walked into a community hospital emergency room with acute upper abdominal pain continuing for three hours, along with nausea and low-grade fever. He denied a change in bowel habits, rectal bleeding, or weight loss. He was a healthy non-smoker, with no relevant medical or surgical history, no medications, and no family history of malignancy. On physical examination, the abdomen was soft and non-distended, with minimal left lower quadrant (LLQ) tenderness to palpation and no palpable mass. A CT scan showed a mesenteric mass with intra-abdominal abscess. He underwent a diagnostic laparotomy at the community hospital with intra-operative drainage of the abscess and biopsy of the mesenteric mass. The final pathology showed a desmoid tumor. His postoperative course was unremarkable, and he was discharged home several days later to complete a course of oral antibiotics.</p><p>Three weeks later, he developed LLQ pain, fever, and decreased drainage from the left pericolic drain. He presented to the community hospital where the CT again showed an intra-abdominal abscess containing air within the mesentery mass (<xref rid="fig0005" ref-type="fig">Fig 1</xref>). The drain was repositioned under ultrasound guidance. However, he developed a contrast-induced nephropathy from the CT and had a prolonged hospital stay for ongoing renal monitoring. The patient’s family requested transfer to our tertiary hospital.<fig id="fig0005"><label>Fig. 1</label><caption><p>A (axial view) B (coronal view), left lower quadrant mesenteric mass containing fluid collection and air locules, measuring 6.6 × 6.1 cm and about the cephalad and lateral component 6.6 × 6.9 cm. (long white arrow points to tumor, and short white arrow points to fistula track).</p></caption><alt-text id="at0040">Fig. 1</alt-text><graphic xlink:href="gr1"></graphic></fig></p><p>Following transfer, two additional abdominal drains were placed in an attempt to achieve infectious source control. He was maintained on broad-spectrum antibiotics but continue to have low-grade fevers. An upper and lower endoscopy was performed to assess for polyposis syndrome. During the upper endoscopy, a fistula track was found in the third portion of the duodenum, and a clip was placed just distal to this site. Both endoscopes were negative for polyps.</p><p>The patient’s case was reviewed by a multidisciplinary sarcoma tumor board, and the outside pathology materials were reviewed. Failure of conservative management to improve the patient’s condition and the finding of a neoplastic fistula led to the consensus to move forward with operative intervention. The patient underwent an en bloc resection of the tumor. A portion of the wall of the third part of the duodenum and a section of jejunum were removed with primary closure of the duodenum and side-to-side anastomosis of the jejunum. The tumor measured 12 cm and involved the small bowel mesentery and a portion of the jejunum, but the superior mesenteric artery and vein were uninvolved. There was no additional disease in the abdomen.</p><p>On postoperative day one, the patient’s hemoglobin dropped from 10 to 7 g/L. He was tachycardic and required ongoing blood products and crystalloid to maintain his urine output. He was taken back to the operating room on postoperative day two for exploratory laparotomy. There was a large clot in the tumor bed. A small mesenteric vein was tied, and the staple line was oversewn to achieve hemostasis. His postoperative course was unremarkable from this point forward, and he was discharged after tolerating a diet on postoperative day seven.</p><p>Three months postoperatively, the patient had gained weight and resumed all normal activity. He was started on Celebrex by the medical oncology team. At his most recent follow-up at 11 months, he had no evidence of recurrence on the CT scan.</p></sec><sec id="sec0015"><label>1.2</label><title>Pathological findings</title><p>The final pathology showed a firm ill-defined mass measuring 12 × 9 x 7 cm. A fistula track to the duodenum was identified measuring 1.5 cm in diameter. The tumor involved more than 80% of the resected mesentery and appeared to invade the wall of the small bowel. All margins were negative with the closest deep posterior margin 2 mm away from the tumor (<xref rid="fig0010" ref-type="fig">Fig 2</xref>). The tumor was composed of uniform fibroblasts associated with undulating collagen fibers, consistent with fibromatosis. The fascicles of the tumor infiltrated into the mesenteric fat, and the fistula tract into the tumor was lined by granulation tissue (<xref rid="fig0015" ref-type="fig">Fig 3</xref>).<fig id="fig0010"><label>Fig. 2</label><caption><p>A (gross specimen), B (Formalin fixed specimen). A portion of small intestine in relation to mesenteric fibromatosis with a fistula formation (black arrow points to the fistula track).</p></caption><alt-text id="at0045">Fig. 2</alt-text><graphic xlink:href="gr2"></graphic></fig><fig id="fig0015"><label>Fig. 3</label><caption><p>A. Fibromatosis with fistula tract into the tumor lined by granulation tissue with inflammation. (white arrow points to the fistula track) B. The fascicles of tumor infiltrate into the mesenteric fat. C. The tumor is composed of broad fascicle of uniform fibroblasts associated with undulating collagen fibers.</p></caption><alt-text id="at0050">Fig. 3</alt-text><graphic xlink:href="gr3"></graphic></fig></p></sec></sec><sec id="sec0020"><label>2</label><title>Discussion</title><p>Intra-abdominal desmoid tumors are the least common type of desmoids. When present, they predominately affect the small bowel mesentery (80%) and, less frequently, the transverse colon, retroperitoneum, omentum, and the ligmentum teres <xref rid="bib0035" ref-type="bibr">[7]</xref>. The majority of patients are asymptomatic until the tumor is quite large because of their slow growth. Clinical presentation can vary depending on their size and location. Desmoids can be misdiagnosed as gastrointestinal stromal tumors (GISTs), solitary fibrous tumors (SFTs), inflammatory myofibroblastic tumors (IMTs), sclerosing mesenteritis, retroperitoneal fibrosis, or secondary malignancy, such as lymphoma <xref rid="bib0040" ref-type="bibr">[8]</xref>. Therefore, a histopathological diagnosis is imperative before treatment. A CT-guided percutaneous biopsy or EUS guided biopsy are less invasive and, therefore, preferred over surgical biopsy.</p><p>Mesenteric desmoid tumors can cause complications such as bowel obstruction, bleeding, perforation, or rarely compression of the ureters or splenic vein <xref rid="bib0015" ref-type="bibr">[3]</xref>, <xref rid="bib0020" ref-type="bibr">[4]</xref>, <xref rid="bib0045" ref-type="bibr">[9]</xref>, <xref rid="bib0050" ref-type="bibr">[10]</xref>. There are also case reports of uncommon presentations such as aortic rupture or abscess formation associated with desmoid tumors <xref rid="bib0055" ref-type="bibr">[11]</xref>, <xref rid="bib0060" ref-type="bibr">[12]</xref>. Abscess formation inside the desmoid tumor is rare, and the etiopathogenesis is not completely clear. Some researchers hypothesize that mesenteric tumors compress the vasculature leading to intestinal ischemia with bacterial transposition causing subsequent necrosis and abscess formation <xref rid="bib0020" ref-type="bibr">[4]</xref>, <xref rid="bib0055" ref-type="bibr">[11]</xref>, <xref rid="bib0065" ref-type="bibr">[13]</xref>, <xref rid="bib0070" ref-type="bibr">[14]</xref>, <xref rid="bib0075" ref-type="bibr">[15]</xref>. Fistulization to adjacent intestine can result, forming a nidus for recurrent abscess formation. In this case, a fistula in the third portion of the duodenum was identified preoperatively, which facilitated surgical planning. Endoscopy should be performed in patients with intra-abdominal desmoid to rule out an associated polyposis syndrome. In the setting of a chronic infectious process, endoscopy serves a dual purpose in also determining if a fistula exist. If the fistula in this case had been distal in the small bowel, a resection could be more easily performed. It was important to know the exact location in this case as a proximal could have warranted a Whipple resection to remove the fistula.</p><p>Owing to the rarity of the disease and a lack of understanding of tumor biology, medical and surgical treatment of intra-abdominal desmoids has not been standardized. The treatment in the NCCN guidelines is based on several retrospective series. Surgery is an option for resectable intra-abdominal desmoid tumor, especially if symptomatic <xref rid="bib0080" ref-type="bibr">[16]</xref>. However, mesenteric desmoid tumors pose particular challenges because of their propensity to grow around the superior mesenteric artery at the root of the mesentery <xref rid="bib0085" ref-type="bibr">[17]</xref>. The infiltrating nature of this tumor often leads to positive resection margins, heavy intra-operative bleeding, or extensive resection of small bowel. Smith et al. reported a series of twenty-four patients with intra-abdominal desmoid tumor that underwent attempted surgical resection <xref rid="bib0090" ref-type="bibr">[18]</xref>. Eight (33%) were found to be unresectable. Of the patients that were resected, five of sixteen (31%) suffered substantial post-operative morbidities, including small bowel ischemia, short bowel syndrome, and chronic severe lower extremity lymphedema. Three of these patients died postoperatively. Despite aggressive surgical management, there was no difference in overall survival between completed resected and unresected patients (73% at 10 years). Alternatives to surgery include NSAIDS (primarily Sulindac) with or without antiestrogens, which are often used in patients with asymptomatic, uncomplicated disease <xref rid="bib0095" ref-type="bibr">[19]</xref>, <xref rid="bib0100" ref-type="bibr">[20]</xref>, <xref rid="bib0105" ref-type="bibr">[21]</xref>. Cytotoxic chemotherapy (doxorubicin, dacarbazine) is limited to patients with large, unresectable intra-abdominal disease that failed to respond to Sulindac and/or anti-estrogen therapy.</p><p>In 2005, the Collaborative Group of the Americas on Inherited Colorectal Cancer (CGA-ICC) proposed a staging system that graded intra-abdominal desmoid tumors by symptoms, tumor size, and growth rate (<xref rid="tbl0005" ref-type="table">Table 1</xref>) <xref rid="bib0095" ref-type="bibr">[19]</xref>. The system was designed based on the current understanding of the natural history of desmoids and worked to stratify patients according to the clinical severity of the tumor. Guidelines for management were proposed based on stage, with early stage tumors treated with NSAIDS or local resection and later stage tumors treated with multimodal medical management. Potentially morbid surgery for late stage tumors was a “last resort if patients have progressed or deteriorated on medical therapy.” Cristiano et al. reported a series of 154 intra-abdominal desmoid tumors in patients with FAP that were classified by the CGA-ICC staging system <xref rid="bib0085" ref-type="bibr">[17]</xref>. In their study, patients with Stage I and II disease were managed by observation and surgical resection, respectively. This group had the most favorable prognosis, with almost all patients alive 15 years after diagnosis. Patients with Stage III disease were managed exclusively with combination medical therapy, including NSAIDS, antiestrogens, and chemotherapy (vinblastine/methotrexate). In contrast, Stage IV patients often required urgent surgical management with combined chemotherapy and radiation, when appropriate. Despite treatment, many Stage III and IV patients suffered a high complication rate and increased mortality, with 5-year overall survival rates of 89% and 76%, respectively. Patients at higher risk of mortality included those who suffered from severe pain, had rapidly growing tumors or tumors larger than 10 cm, or had septic complications, such as fistulae or abscesses.<table-wrap id="tbl0005" position="float"><label>Table 1</label><caption><p>Desmoid tumor staging system from the Collaborative Group of the Americas on Inherited Colorectal Cancer (CGA-ICC) <xref rid="bib0095" ref-type="bibr">[19]</xref>.</p></caption><alt-text id="at0055">Table 1</alt-text><table frame="hsides" rules="groups"><thead><tr><th align="left">Stage</th><th align="left"></th></tr></thead><tbody><tr><td align="left">I</td><td align="left">Asymptomatic, <10 cm maximum diameter, and not growing</td></tr><tr><td align="left">II</td><td align="left">Mild symptomatic,<xref rid="tblfn0005" ref-type="table-fn">*</xref> <10 cm maximum diameter, and not growing</td></tr><tr><td align="left">III</td><td align="left">Moderately symptomatic,<xref rid="tblfn0010" ref-type="table-fn">†</xref> or bowel/ureteric obstruction, or 10–20 cm, or slowly growing</td></tr><tr><td align="left">IV</td><td align="left">Severely symptomatic,<xref rid="tblfn0015" ref-type="table-fn">‡</xref> septic complications such as fistula and abscess, or >20 cm or rapidly growing</td></tr></tbody></table><table-wrap-foot><fn id="tblfn0005"><label>*</label><p id="npar0005">Mildly symptomatic = sensation of mass, pain, but no restriction.</p></fn></table-wrap-foot><table-wrap-foot><fn id="tblfn0010"><label>†</label><p id="npar0010">Moderately symptomatic = sensation of mass, pain; restrictive but not hospitalized.</p></fn></table-wrap-foot><table-wrap-foot><fn id="tblfn0015"><label>‡</label><p id="npar0015">Severely symptomatic = sensation of mass, pain; restrictive, and hospitalized.</p></fn></table-wrap-foot></table-wrap></p><p>Intra-abdominal desmoids are commonly more difficult to resect without serious morbidity compared with extra-abdominal and abdominal wall desmoid tumors. Surgery may be technically challenging, and the risk to the patient is high, particularly when these tumors occur in patients with FAP <xref rid="bib0110" ref-type="bibr">[22]</xref>. A period of close observation, with serial imaging, can be initiated on asymptomatic patients for an interval of 3–6 months, if symptoms permit <xref rid="bib0115" ref-type="bibr">[23]</xref>. In progressive or symptomatic disease, medical management or surgery are appropriate options <xref rid="bib0120" ref-type="bibr">[24]</xref>. Surgery is an alternative in readily resectable disease; however, the risk of surgery and recurrence should be considered. Occasionally, surgical management becomes the only resort in patients with chronic tumor-related complications, such as in this case where the patient developed chronic abscess formation despite wide drainage and medical treatment (<xref rid="tbl0010" ref-type="table">Table 2</xref>).<table-wrap id="tbl0010" position="float"><label>Table 2</label><caption><p>Summary of literature of intra-abdominal Desmoids complicated by abscess formation <xref rid="bib0075" ref-type="bibr">[15]</xref>.</p></caption><alt-text id="at0060">Table 2</alt-text><table frame="hsides" rules="groups"><thead><tr><th align="left">Report</th><th align="left">Year</th><th align="left">No. of pts</th><th align="left">FAP related</th><th align="left">1st treatment</th><th align="left">2nd treatment</th></tr></thead><tbody><tr><td align="left">Maldjian et al.</td><td align="left">1995</td><td align="left">3</td><td align="left">3</td><td align="left">Percutaneous drainage + antibiotics</td><td align="left">Surgical resection (2)</td></tr><tr><td align="left">Cholongitas et al.</td><td align="left">2006</td><td align="left">1</td><td align="left">None</td><td align="left">Percutaneous drainage + antibiotics</td><td align="left">Surgical resection (1)</td></tr><tr><td align="left">Ebrahimi et al.</td><td align="left">2008</td><td align="left">1</td><td align="left">None</td><td align="left">Antibiotics</td><td align="left">Surgical resection (1)</td></tr><tr><td align="left">Peled et al.</td><td align="left">2012</td><td align="left">1</td><td align="left">None</td><td align="left">Antibiotics</td><td align="left">Surgical resection (1)</td></tr><tr><td align="left">Giovanni et al.</td><td align="left">2013</td><td align="left">1</td><td align="left">1</td><td align="left">Percutaneous drainage + antibiotics</td><td align="left">Tamoxifen therapy and chemo (1)</td></tr></tbody></table></table-wrap></p><p>Overall, the principles of managing intra-abdominal desmoid tumors should include the following:<list list-type="simple"><list-item id="lsti0020"><label>1.</label><p>Care should be guided by a multidisciplinary team experienced in the management of desmoid tumors.</p></list-item><list-item id="lsti0025"><label>2.</label><p>Preoperative imaging and endoscopy are essential in operative planning.</p></list-item><list-item id="lsti0030"><label>3.</label><p>Operative intervention should be performed by surgeons familiar with desmoid tumor biology to maximize oncologic outcome and minimize patient morbidity.</p></list-item></list></p></sec><sec id="sec0025"><label>3</label><title>Conclusion</title><p>Intra-abdominal desmoid tumors can produce bowel ischemia, obstruction, perforation, and, in this case, fistulization. Fistulization should be considered in patients with chronic abscess formation after drainage and medical therapy. Multidisciplinary discussion should be conducted before pursuing definitive treatment, and a thorough work up should be completed to appropriately stage the patient and classify desmoid related complications <xref rid="bib0115" ref-type="bibr">[23]</xref>.</p></sec><sec id="sec0030"><title>Conflict of interest</title><p>None of the authors report a conflict of interest regarding this case report.</p></sec><sec id="sec0035"><title>Sources of funding</title><p>None declared.</p></sec><sec id="sec0040"><title>Ethical approval</title><p>Not applicable.</p></sec><sec id="sec0045"><title>Consent</title><p>Informed consent for the publication of this work has been obtained.</p></sec><sec id="sec0050"><title>Author contribution</title><p>Kai Huang: Concept and design of study, data collection, data interpretation and analysis, drafting, revision, approval of final manuscript.</p><p>Heather Stuart: Surgical oncology fellow, who performed operation and follow-up, data collection, interpretation and analysis, revision of final manuscript.</p><p>Alan Livingstone: Surgeon (performed operation and follow-up), Concept and design of study, revision, approval of final manuscript.</p><p>Kirill Lyapichev and Andrew Rosenberg: Pathologists (performed the histological examination and provided the H.E slides), data collection, data interpretation and analysis, approval of final manuscript.</p></sec><sec id="sec0055"><title>Guarantor</title><p>Alan S. 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