desmoid tumours
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2022 ◽  
Author(s):  
Arthur S. Aelvoet ◽  
Daphne Struik ◽  
Barbara A. J. Bastiaansen ◽  
Willem A. Bemelman ◽  
Roel Hompes ◽  
...  

Abstract Desmoid tumours (DT) are one of the main causes of death in patients with familial adenomatous polyposis (FAP). Surgical trauma is a risk factor for DT, yet a colectomy is inevitable in FAP to prevent colorectal cancer. This systematic review and meta-analysis aimed to synthesize the available evidence on DT risk related to type, approach and timing of colectomy. A search was performed in MEDLINE, EMBASE and the Cochrane Library. Studies were considered eligible when DT incidence was reported after different types, approaches and timing of colectomy. Twenty studies including 6452 FAP patients were selected, all observational. No significant difference in DT incidence was observed after IRA versus IPAA (OR 0.99, 95% CI 0.69–1.42) and after open versus laparoscopic colectomy (OR 0.88, 95% CI 0.42–1.86). Conflicting DT incidences were seen after early versus late colectomy and when analysing open versus laparoscopic colectomy according to colectomy type. Three studies reported a (non-significantly) higher DT incidence after laparoscopic IPAA compared to laparoscopic IRA, with OR varying between 1.77 and 4.09. A significantly higher DT incidence was observed in patients with a history of abdominal surgery (OR 3.40, 95% CI 1.64–7.03, p = 0.001). Current literature does not allow to state firmly whether type, approach, or timing of colectomy affects DT risk in FAP patients. Fewer DT were observed after laparoscopic IRA compared to laparoscopic IPAA, suggesting laparoscopic IRA as the preferred choice if appropriate considering rectal polyp burden. PROSPERO registration number CRD42020161424.


2021 ◽  
Vol 14 (9) ◽  
pp. e244361
Author(s):  
Nandesh Patel ◽  
Ralitsa Slivkova ◽  
Sunil James ◽  
Max Almond

Desmoid tumours are clonal fibroblastic proliferations in soft tissues, characterised by infiltrative growth and local recurrence, but not metastasis. Various treatment strategies for desmoid tumours exist, varying from observation, medical and systemic therapy to radiotherapy and surgery. A 25-year-old woman with a background of familial adenomatous polyposis was referred with an enlarging abdominal desmoid tumour measuring 40×40×40 cm despite repeated radiofrequency ablation, surgical debulking and hormone therapy. The patient had a two-stage operation. The first stage involved excision of the desmoid tumour with full-thickness abdominal wall. The abdominal wall was not closed, and a topical negative pressure seal was applied. After 2 days, she underwent the second stage: reconstruction of the abdominal wall defect with a large porcine mesh which was covered with anterolateral thigh flaps. Postoperative complications included ileus and a fall which required further surgery. The patient was discharged 1 month after the first operation. Abdominal MRI scans were performed at 3 and 7 months postdischarge and showed no recurrence of diseaseBackground


2021 ◽  
Vol 2021 (8) ◽  
Author(s):  
Boyodi Katanga Tchangai ◽  
Mazamaesso Tchaou ◽  
Fousseni Alassani ◽  
Joel Ekoué Amétitovi ◽  
Kwamé Doh ◽  
...  

Abstract Desmoid tumours are deep aggressive fibromatoses that usually arise in the soft tissues of the limbs or the abdominal wall. Intra-abdominal localisation, rarely occurs and their treatment may be challenging. When necessary, surgery must be personalized to what is achievable in terms of margins while preserving functional outcomes. This condition is illustrated herein with the case of a 40-year-old female presenting an unusually large sporadic desmoid tumour with abdominal, pelvic and perineal involvement. Resection was performed without organ involvement through a combined perineal approach. Tumour resection was macroscopically completed except in the perineum, where the tumour was left (R2 resection) to preserve anal sphincter. Adjuvant treatment with tamoxifen was given to achieve local control. The hormonal treatment was well tolerated, and no recurrence was observed after 36 months of follow-up.


2021 ◽  
Vol 15 (7) ◽  
pp. 1672-1675
Author(s):  
Muhammad Awais ◽  
Sundas Javeed ◽  
Mahnoor Mohydin ◽  
M. Kamil Zulfiquar ◽  
Usama Rafi ◽  
...  

Fibroid is a benign lump of growth, occurring within or outside the uterus. It is a rarity for such growths to occur intra-abdominally. Such cases are of aggressive fibromatosis which are marked by the presence of desmoid tumours. It is most commonly seen in patients with a history of familial adenomatous polyposis (FAP) or past surgical procedures. As FAP has a wide fibroepithelial growth spectrum, the symptomatology and therefore the prognosis varies. Unpredictable clinical behaviour, varied location and non-specific presentation are the factors accounting to diagnostic difficulty. Therefore, misdiagnosing the disease is not uncommon. Here we report a case ofa26-year-old pregnant female presenting with lower abdominal pain and mass. Preoperative diagnosis was of uterine fibroids as per radiological evidence, but on exploration it turned out to be a large right colonic mass. The patient underwent right hemicolectomy. Histopathological reports confirmed morphological and immuno-histochemical features indicating fibromatosis. This fibromatous mass though intra-abdominal was not growing from the mesentery/mesocolon or the pelvis. It appeared to grow from the wall of the colon. This is a confirmatory presentation of true colonic wall fibromatosis which is deep and isolated form of intra-abdominal desmoid tumours. As this lesion was isolated in nature, the treatment of choice was surgical resection. It is essential to have a multi-disciplinary team approach in the management of such a patient. This improves the treatment and the prognostic outcomes. Keywords: Fibroid, Colon, Fibromatosis, Hemicolectomy, Desmoid Tumour


Author(s):  
Christos Kakos ◽  
Savvas Lampridis ◽  
Georgios Geropoulos ◽  
Reena Khiroya ◽  
Achilleas Antonopoulos ◽  
...  

Desmoid tumours are rare, locally aggressive neoplasms exhibiting high tendency for recurrence, even after complete resection. Only 1 in 5 of them originates from the chest wall, usually measuring less than 10 cm at diagnosis. Herein, we report the case of a woman presenting with symptoms of gradual lung compression by a giant desmoid tumour occupying the entire hemithorax. She underwent complete surgical resection of the tumour and chest wall reconstruction. She had disease recurrence 15 months later and currently remains under regular follow-up. The management of intrathoracic desmoid tumours is challenging because they are usually not diagnosed until they become large enough to cause compression symptoms. While medical management is the primary modality of treatment, surgery could be considered in selected cases where significant symptoms arise, and the functional status is impaired secondary to the tumour. Adjuvant radiotherapy to minimise the risk of local recurrence should also be considered.


2021 ◽  
Vol 145 ◽  
pp. 109-120
Author(s):  
Shinji Kohsaka ◽  
Makoto Hirata ◽  
Masachika Ikegami ◽  
Toshihide Ueno ◽  
Shinya Kojima ◽  
...  
Keyword(s):  

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Fergus J. McCabe ◽  
Alan J. Hussey ◽  
John P. McCabe

Desmoid tumours are benign neoplasms of myofibroblasts, often occurring after soft-tissue trauma. Rarely, desmoid tumours can occur following operative intervention, including spine surgery. In this case report, we describe the first reported case of desmoid tumour following scoliosis corrective surgery in an adolescent.


2021 ◽  
Vol 14 (2) ◽  
pp. e237032
Author(s):  
Sai Krishna Eswaravaka ◽  
Swanit Hemant Deshpande ◽  
Roshan Chiranjeev ◽  
Jayashri Sanjay Pandya

Desmoid tumours, also known as aggressive fibromatosis, are fibromuscular neoplasms that arise from mesenchymal cell lines. Desmoid tumours are usually benign and are locally aggressive tumours. We report a case of a 31-year-old man presenting with abdominal mass associated with dyspepsia and early satiety. CT scan demonstrated a large heterogeneous mass adherent to or arising from the jejunum. The patient underwent a successful elective exploratory laparotomy with resection of the tumour arising from the wall of the ileum with a 10 cm margin. The patient had an uneventful recovery and no recurrence at 6-month follow-up. Pathology report and immunohistochemistry analysis revealed the mass to be a primary desmoid tumour of the small bowel, as the tumour was negative for c-kit and Discovered on GIST 1 (DOG-1) and positive for beta-catenin and smooth muscle actin.


2020 ◽  
Vol 75 ◽  
pp. e22
Author(s):  
Sean Michael Booth ◽  
Vivian Tang
Keyword(s):  

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