Skip to main content
. 2022 Mar 2;15(3):e247935. doi: 10.1136/bcr-2021-247935

Table 1.

Summary of desmoid tumour cases described in the literature

Year Author Age Gender Presentation Intraoperative findings and organ involvement Tumour size (cm) Operation Outcome
2021 Tchangai et al5 40 F Abdominal distension, constipation, difficulty voiding, weight loss Firm multilobed mass that contracted adhesions with the pelvic colon, right ureter and rectum. 40 Laparotomy and resection through a combined perineal approach. Alive
2021 Park et al6 23 F Epigastric pain Mass involving distal pancreas, invasive to the posterior wall of the antrum of the stomach and transverse colon and fourth portion of the duodenum. 10 Distal pancreatectomy, splenectomy and combined partial resection of the stomach, transverse colon and fourth portion of the duodenum. Alive
2021 Pop et al7 38 F Abdominal pain, asthenia, loss of appetite The mass localised at the level of the jejunal mesentery, in tight contact with the duodenum and the mesenteric vessels. 7 ‘En bloc’ resection of the tumour, together with the involved enteral loops followed by end-to-end anastomosis of the jejunum. Alive
2021 Mitrovic et al8 37 F Epigastric pain, palpable mass in the right hemiabdomen Well-marginated mass that invaded the wall of the caecum and small bowel mesentery near the terminal ileum. 11 Hemicolectomy with partial resection of the terminal ileum with latero-lateral ileocolonic anastomosis. Alive
2021 Omori et al9 46 M Fever, right lower quadrant pain The scar tissue related to the abscess was attached to the lateral wall of the ascending colon and spread to the retroperitoneum. A portion had infiltrated the muscularis propria of the ascending colon and had formed a fistula. 5.4 Right colectomy with abscess resection. Alive
2021 Laurens et al10 56 F Nausea, distended abdomen Mass adhered to a loop of small bowel, a short segment of transverse colon and three sections of omentum. ‘Grapefruit’ Laparotomy and mass excision and bowel anastomoses. Alive
2020 Jin et al11 28 F (pregnant at 32 weeks) Abdominal pain Left side of the uterus. 30 Resection. Alive
2020 Deshpande et al12 44 F Abdominal discomfort Multiple soft tissue masses attached to the wall of small intestine and invading the surrounding mesentery. 11 Resection of a large segment of small intestine. Alive
2020 Khanna et al13 45 M Periumbilical pain, nausea, vomiting, loss of appetite Large, heterogeneous mass arising from the body of the pancreas. The mass was predominantly solid with a small cystic component abutting the stomach and duodenum with loss of intervening fat planes. There was moderate to severe narrowing of the portal venous confluence and adjacent segments of the splenic vein and superior mesenteric vein. 12 Radical resection of the mass with distal pancreatectomy, splenectomy, partial gastrectomy, duodenectomy of the fourth portion of duodenum and resection of portal vein with interposition of deep femoral vein graft from superior mesenteric vein to portal vein. Alive
2020 Omi et al14 47 F Left back pain Adherence of the tumour to the rectum and left ureter but no dissemination or metastasis. The tumour was located at the left obturator fossa, involved the rectum and left ureter and was fixed to the left pelvic sidewall due to infiltration to the left parametrium. 3 Laparoscopic resection of the left adnexa, left parametrium and combined resection of the left ureter and rectum. Alive
2020 Sierra-Davidson et al15 27 F Left upper quadrant pain, palpable mass (post-Roux-en-Y bypass surgery) The mass arose from the proximal small bowel and extended from the root of the mesentery to within 1 cm of the bowel wall. The mass was firmly adherent to the Roux limb, as well as the jejunojejunostomy and distal portion of the biliopancreatic limb. The distal small bowel was not involved. 16 The mass was resected en bloc with the entire Roux limb and the jejunojejunostomy. The RYGB was reconstructed in an antecolic antegastric fashion. The gastrojejunostomy and jejunojejunostomy were then recreated. Alive
2020 Mahnashi et al16 48 M Abdominal pain (epigastrium and left hypochondrium), discomfort The mass arose from the jejunal wall with a highly vascular smooth surface and a well-defined margin at the gastrojejunostomy anastomosis. The mass had a large feeding arterial supply arising from the mesentery of the small bowel. Another mass was found distal to the gastrojejunostomy. 15.8 Laparoscopic exploration converted to laparotomy. Surgical resection was performed, and the gastrojejunal anastomosis was disconnected from the distal stomach pouch. A Roux-en-Y gastric bypass was done. Alive
2019 Asenov et al17 27 M Right lower quadrant and suprapubic pain Tumour involved the jejunum in its proximal third. The affected loop was situated near the ileocecal confluence. Not specified Laparotomy and resection of the small bowel. Alive
2019 Ebeling et al18 59 M Right lower quadrant pain, abdominal distension, urinary urgency Mass from mid-jejunum that displaced the bowel but did not invade adjacent structures. 18 Exploratory laparotomy and mass resection—segmental enterectomy with primary stapled anastomosis. Alive
2019 Mastoraki et al19 36 M Abdominal pain, palpable mass in left abdomen
  1. 14 years prior, the initial tumour was intra-abdominal and adhered to the left colonic flexure, major gastric curvature and spleen.

  2. Recurrence: epigastric mass which adhered to the transverse colon and lower duodenal segment, and seemed to communicate via tissue bridging with a second abdominal lesion along the lower third of the right kidney.

12.5
  1. Initial tumour 14 years prior required tumour resection along with left colectomy, splenectomy and partial gastrectomy.

  2. Tumour recurrence: exploratory laparotomy—unresectable, a jejunojejunal bypass was performed. Traditional therapeutic interventions proved insufficient, and the patient was treated medically thereafter.

Alive
2018 Stickar et al20 76 M Fever, abdominal distension, palpable mass in hypogastrium Mass arose from the jejunum, 20 cm from the duodenojejunal angle. 15 Intestinal resection lateral–lateral mechanical anastomosis. Alive
2018 Akbulut et al21 46 F Postprandial nausea and vomiting The mass originated from the pancreatic body, adhering to the prepyloric antrum of the stomach and forming a conglomerated structure with the fourth part of the duodenum and proximal jejunal loops. 12 Laparotomy with conglomerated fourth part of the duodenum, proximal jejunum, distal pancreas and the spleen were removed en-bloc. End-to-end anastomosis was formed between the third part of duodenum and proximal jejunum. Alive
2018 Burke et al22 46 M Upper abdominal discomfort, dyspepsia, early satiety The mass projected from the mesenteric fat and infiltrated surrounding structures including the small bowel and caecum. It had encased around the SMA and SMV, compromising blood supply to the small bowel. 5.6 Laparotomy with excision of this mesenteric mass—an extended right hemicolectomy, small bowel resection, jejuno-jejunal anastomosis and an ileo-colic anastomosis. Alive
2018 Lee et al23 46 M Left lower quadrant pain, palpable mass in the left upper abdomen The mass arose from the retroperitoneum, closely related to the pancreas tail. The boundary between the mass and adjacent pancreas parenchyma was indistinct. 21.5 Laparoscopic spleen-preserving distal pancreatectomy without preoperative biopsy due to a risk of rupture. Alive
2018 Ogawa24 35 F Abdominal pain The tumours were extensively integrated throughout the rectus abdominis and transverse abdominal muscles. 15 Detectable tumours were resected resulting complete removal of her rectus abdominis muscle, including the anterior and posterior sheaths and parts of her transverse abdominal muscle. Partial colectomy for transverse colon that strongly adhered to some tumours. Alive
2017 Jafri et al25 54 F Dysphagia, weight loss Irregular, ill-defined mass at the head of the pancreas causing complete bile and pancreatic duct obstruction. 5.2 Whipple with end-to-end pancreaticojejunostomy, cholecystectomy, an end-to-side choledochojejunostomy, a wedge liver biopsy of segment 3, a biopsy of the superior pancreatic lymph node and a retrocolic end-to-end gastrojejunostomy. Alive
2017 Lu et al26 47 F Abdominal pain Posterior wall of the antrum. 4.5 Distal gastrectomy (Billroth-I). Alive
2016 Nagata et al27 49 M Ileus symptoms Recurrent mesenteric tumours with adhesion to the intestinal tract and peritoneum. 12 Initial: duodenojejunostomy. Current: surgically unresectable. Alive
2015 Sugrue et al28 71 M Abdominal pain The mass originated from the jejunal mesentery with a 25 cm loop of jejunum firmly adherent to the mass approximately 15–20 cm from the ligament of Treitz. Left-sided tumour adherent to the left abdominal wall, mesentery, omentum and small bowel. 24 The piece of the jejunum that was adherent to the mass was resected, and the mass was removed en bloc from the abdominal cavity with primary, side-to-side stapled small bowel anastomosis. Alive
2015 Bn et al29 24 M Abdominal pain, distention The tumour extended from L2–L5 to posterior part of abdominal wall. 16.4 Laparotomy with mass excised and mesentery repaired. Alive
2014 D et al30 29 M Swelling in the right side of umbilicus Intraperitoneal cavity with retroperitoneal extension. 6 Exploratory laparotomy with excision of mass in ileal mesentery—excised along with 20 cm of ileum and end to end anastomosis in two layers. Alive
2014 Fleetwood et al31 60 M Right upper quadrant pain The mass was inseparably adherent to the small bowel and the mesentery. 13 Laparotomy and resection en bloc with the mass. Alive
2013 Monneur et al32 21 M Abdominal pain, vomiting, pyrosis, constipation, increase of abdomen volume Intra-abdominal tumour close to the stomach (origin difficult to assess). 3.2 No operation—medically treated. Alive
2012 Peled et al33 32 M Abdominal pain, fever A large submucosal mass obstructed the appendix orifice. The appendix was dilated. A large abscess was attached to the lateral wall of the cecum along the appendix. 8 Right hemicolectomy. Alive
2010 Basdanis et al34 52 M Acute epigastric pain, obstructive ileum The large mesenteric mass occupied the left lateral abdominal quadrant and hypogastrium. Not specified Laparotomy with resection of tumour. Alive
2011 Chang et al35 50 M Abdominal pain The tumour arose from the antimesenteric side of the ileum 60–80 cm proximal to the ileocecal valve. Not specified Laparotomy with resection of tumour and a segment of small bowel. Alive
2007 Shah and Azam36 33 M Abdominal pain Tumour located to ileum (25 cm proximal to the ileo-caecal valve on the mesenteric border). 5 Laparotomy and resection of 30 cm small bowel with a right hemicolectomy and side-to-side anastomosis. Alive
2008 Tanaka et al37 73 M Swelling and pain of the right leg Mass located to the right pelvis. 9.5 No operation—medically treated. Alive
2007 Rakha et al38 38 F
  1. Abdominal swelling

  2. Chronically ill, anaemic, cachetic and toxic

  1. Tumour encroaching on the pelvic and abdominal organs and displacing the bowel cranially

  2. Intra-abdominal tumour, filling most of the pelvic and abdominal cavity with an extra-abdominal fungating mass protruding through an abdominal wall defect; adhesions to the surrounding tissues, pelvic and abdominal organs (liver, colon, stomach and parietal peritoneum).

  1. 28

  2. 36

  1. Resection of tumour and part of adjacent rectus muscle

  2. Recurrence: fungating extra-abdominal mass excised, intra-abdominal tumour radically excised en bloc, debridement and closure of the abdominal wall defect, reconstruction of the abdominal wall with plastic procedure.

Alive
1993 Disher et al39 18 M Weight loss, vomiting The mass had a pedicle from the lower greater omentum and displaced both the common and internal iliac arteries. 12.6 Laparotomy with wide surgical resection. Alive
1980 Logio et al40 60 F Abdominal pain The tumour originated from the mesentery of mid-jejunum and infiltrated the bowel wall to the submucosa, interrupting continuity of the muscle coat. 3 Laparotomy and tumour dissection from mesenteric vascular pedicle, a 37-cm segment of jejunum and its mesentery and mesenteric nodes resected with end-to-end jejunostomy. Alive
2019 Kim et al41 59 F Asymptomatic Peritoneum, coeliac area. 3.7 Open biopsy. Alive
63 F Not specified Peritoneum, pelvic area. 2 Small bowel resection and anastomosis, mass excision. Alive
58 M Not specified Peritoneum, LUQ area. 1.7 Laparoscopic wedge resection of stomach, mass excision. Alive
50 M Not specified Peritoneum, pelvic area. 6.7 Small bowel resection and anastomosis, mass excision. Alive
67 M Not specified Peritoneum, pelvic area. 2.2 Excision of small bowel mesentery. Alive
56 M Not specified Peritoneum, pelvic area. 3 Small bowel resection and anastomosis. Alive
40 F Asymptomatic Peritoneum, pelvic area. 3.3 Small bowel resection and anastomosis. Alive
72 M Abdominal discomfort Peritoneum, adrenal area. 1.3 Mass excision. Alive
2018 Cheng et al42 34 M Palpable mass The mass originated from the mesentery root, and adhered tightly to the descending duodenum, a portion of small intestine and the head of the pancreas. The SMA and SMV were engulfed by the tumour. 20 En bloc resection, including the whole small intestine, right and proximal transverse colon, while the SMA and SMV were resected at their root. Alive
2015 Kim et al43 78 M Palpable mass The tumour involved the external and internal oblique muscles above the transversus abdominis. 4 Diagnostic laparoscopy confirmed no peritoneal seeding lesions and no tumour invasion to the peritoneal surface. Metastatic lesion was resected. Alive
2015 Efthimiopoulos et al44 40 M Palpable mass A ‘tennis ball-shaped’ tumour of the mesentery close to the small intestine, 40 cm from the ileocecal valve. 8.5 A wide excision of the involved mesentery and adjacent small intestine with a side-to-side anastomosis between the proximal and the distal end of the small intestine. Alive
2014 Palladino et al45 69 M Palpable mass The tumour arose from the mesentery. It involved the duodenojejunal angle, and compressed the inferior vena cava. 20 Laparotomy and resection of large mesenteric mass with a part of the adherent jejunal and colic segment. Dead-MI on postop day 1
2006 Galeotti et al46 31 F Palpable mass Mass located to left rectus muscle. 10 Laparotomy and removal whole thickness of the abdominal wall including the tumour. Alive
1991 Umemoto et al47 37 M Palpable mass Mass in abdominal wall, mesentery and retroperitoneum. 12 Laparotomy and tumour excision. Alive
2020 Shayesteh et al48 64 M Incidental CT finding Hypodense lesion in the body of the pancreas with the abutment of the splenic artery and vein. The surgical pathology showed intrapancreatic fibromatosis. 2.9 Distal pancreatectomy and splenectomy. Alive
2020 Maemoto et al49 70 F Incidental CT finding Presacral region below the bifurcation of the common iliac artery and involving the mesentery of the ileum. 2.8 Resection of the tumour combined with part of the mesentery. Alive
2019 Muneer et al50 63 F Incidental finding during abdominoplasty for elective body-contouring surgery Mass arose from the midline of the rectus sheath, in the para-umbilical region, both superior and inferior to the umbilicus. 3 The umbilicus was sacrificed and excised with the tumour, and neoumbilical reconstruction was done. The abdominal wall defect was closed primarily, and rectus sheath plication was performed. Alive
2014 Kobayashi and Sugihara51 55 M Incidental CT finding Duodenum and mesentery. 1.2 Laparotomy with tumour resection and wedge resection of the duodenum. Alive
2013 Okamura et al52 57 M Incidental CT finding Mesentery of the transverse colon. 1.5 Tumour resection and part of transverse colon was also removed. Alive
2012 Shih et al53 56 M Incidental CT finding The tumour was found in the retroperitoneum adhering to the peritumour vessels, nerves and the pancreatic tail. 3.6 Tumour resection en bloc with sacrifice of adjacent vessels and nerves. Alive

(a): Findings of initial tumour; (b): findings of tumour recurrence.

F, female; LUQ, Left Upper Qudratn; M, male; SMA, superior mesenteric artery; SMV, superior mesenteric vein.