Complex Case of Aggressive Intra-abdominal Desmoid-type Fibromatosis Status Post Cholecystectomy

Intra-abdominal desmoid-type fibromatosis (DF) can present sporadically, in sites of previous trauma, surgical scars and irradiation, or in association with familial adenomatous polyposis and Gardner syndrome. Intra-abdominal DF is uncommon and especially rare after a common surgery like cholecystectomy. We report a rare case of a 67-year-old male who presented with a locally aggressive intra-abdominal DF in the gallbladder fossa, status post cholecystectomy. This progressively enlarging infiltrative enhancing solid mass in the gallbladder fossa on serial computed tomography and magnetic resonance imaging demonstrated gastric outlet obstruction, biliary obstruction, portal vein narrowing and encasement of hepatic artery. Diagnosis of DF in this postoperative setting was delayed and challenging due to uncharacteristic clinical presentation.

This was a 44-year-old, para 2 (2 alive) who was referred to our gynaecology clinic from a secondary health facility on account of slowly increasing abdominal swelling of 2 years duration. There was no nausea, vomiting or diarrhea. There was associated mild to moderate dull lower abdominal pain that did not radiate to any other part of the body. There was no change in her monthly menstrual flow. Physical examination revealed a pelvic mass about 32 weeks pregnancy size and firm. Computed Topography scan showed a huge pedunculated sub-serous uterine fibroid. A huge mass adherent to the anterior surface of the body of the uterus was completely surgically excised at exploratory laparotomy. Histology of the excised tumour revealed a definitive histological diagnosis of pelvic desmoid tumour. The patient was clinically stable and discharged home 10 days post operation and was followed-up on out-patient gynaecology clinic basis. Pelvic desmoid tumour should be considered as a differential diagnosis in premenopausal women who present with abdominal swelling.

Intra-abdominal desmoid tumor is rare with no particular imaging features, so preoperative diagnosis is quite difficult. R0 resection is essential for treatment but often requires extensive surgical trauma, which can be a risk for recurrence. A laparoscopic approach for this tumor was effective, with the resulting diagnosis and resection being less traumatic. The first report of successful laparoscopic complete resection and reconstructive procedures were demonstrated.

We report four cases of desmoid-type fibromatosis (“desmoid tumors”) mimicking local recurrence after extremity sarcoma resection. We retrospectively reviewed the records of patients treated for extremity sarcoma by our orthopedic oncology service from 2014 to 2019 and identified four patients with biopsy-proven desmoid tumors. We extracted clinical, pathologic, radiographic, and operative data for the primary neoplasms and desmoid tumors. Four patients with post resection surveillance magnetic resonance imaging suspicious for local recurrence underwent further analysis showing desmoid tumors. Patients underwent image-guided needle biopsy, with specimens demonstrating fibromatosis-type histologic characteristics. Two cases were beta-catenin positive. Desmoid tumors were managed with observation. No patient had experienced local or distant recurrence of the primary tumor at a mean follow-up of 30 months after resection (range, 23-34 months); none underwent surgery for symptoms of desmoid tumors.

The purpose of this systematic review is to assess and compare the efficacy of surgical treatment for patients with asymptomatic extra-peritoneal desmoid-type fibromatosis to the wait-and-see policy by evaluating (1) the exacerbation rate (exacerbation; recurrence after surgery or progressive disease following non-surgical treatment) and (2) treatment-associated complications in extra-peritoneal desmoid-type fibromatosis. We evaluated documents published between 1 January 1990 and 31 August 2017. One prospective cohort study, four case-control studies and five case series studies were identified. Meta-analysis was performed to evaluate the exacerbation rate after treatment on one prospective cohort study and four case-control studies. In comparing surgical and non-surgical treatments, the exacerbation rate was significantly higher in the surgical treatment group (odds ratio: 1.32, 95% confidence interval 1.01-1.73, P = 0.05). However, in the case series study, the recurrence rate was 23.4% for the surgical treatment group, while the progressive disease rate was 28.1% for the non-surgical treatment group. The postoperative complication rates associated with surgical treatment in the two studies were 20.8 and 17.2%, respectively. When considering the exacerbation rate, non-surgical treatment might be appropriate for asymptomatic patients with extra-peritoneal desmoid-type fibromatosis. However, if patients with tumor-related symptoms opt for surgery, including those who face difficulties due to the presence of tumors, it is important to fully explain to them the possibility that the recurrence rate and treatment-associated functional failures may increase depending on the site of occurrence.