Extremely Large Intra-Abdominal Fibromatosis Presents as a Life-Threatening Surgical Emergency (ENTIRE ARTICLE)

A 65 years old, heathy female patient, presented to our Emergency Medicine Department with severe lower abdominal pain of acute onset, associated with nausea and recurrent vomiting. Abdominal examination upon her admission revealed diffuse peritonitis, with a tender palpable mass on the mid abdomen. Per rectum exam was normal. A contrast enhanced computed tomography (CT) scan showed large space occupying lesion of 10*12 cm at left mid-lower abdomen, penetrating into small bowel loop, with free intra-abdominal air and fluid. On exploratory laparotomy, large amount of purulent fluid was demonstrated, along with a large (about 12 cm diameter) soft tissue tumor attached to several bowel loop with small intestine perforation due to tumor penetration. Resection of the tumor with long segment of small bowel, along with primary anastomosis was completed. Her post-operative period (POD) was uneventful, and she was discharged home on POD 6. Histopathological exam of the specimen with hematoxylin and eosin stain showed Mesenterial tumor of Intraabdominal fibromatosis (desmoid), invading to the wall of small intestine with perforation and peritonitis. Surgical margins of mesenterium are free of tumor. Reactive lymph nodes seen in mesenterium.

We report the case of a 55-year-old man with a surgical history of distal gastrectomy with Roux-en-Y reconstruction performed 3 years prior to the present episode. During the follow-up, a newly developed, rapidly growing intraabdominal mass was detected in the mesentery of the small intestine. Although the patient had been asymptomatic, surgical resection was planned with the suspicion of malignancy, especially lymph node recurrence of the gastric cancer, owing to its rapid growth. Laparotomy showed that the tumor was located in the mesentery of the small intestine near the Roux-en-Y limb, and due to the involvement of the feeding vessels to the Roux-en-Y limb, the anastomotic site was resected en bloc with the tumor, and the whole Roux-en-Y limb was reconstructed. The histopathological finding was compatible with desmoid-type fibromatosis of the mesentery of the small intestine. Here we report our case and discuss the previously reported literature, especially related to gastric cancer.

We report the case of a 55-year-old man with a surgical history of distal gastrectomy with Roux-en-Y reconstruction performed 3 years prior to the present episode. During the follow-up, a newly developed, rapidly growing intraabdominal mass was detected in the mesentery of the small intestine. Although the patient had been asymptomatic, surgical resection was planned with the suspicion of malignancy, especially lymph node recurrence of the gastric cancer, owing to its rapid growth. Laparotomy showed that the tumor was located in the mesentery of the small intestine near the Roux-en-Y limb, and due to the involvement of the feeding vessels to the Roux-en-Y limb, the anastomotic site was resected en bloc with the tumor, and the whole Roux-en-Y limb was reconstructed. The histopathological finding was compatible with desmoid-type fibromatosis of the mesentery of the small intestine. Here we report our case and discuss the previously reported literature, especially related to gastric cancer.

This was a cross-sectional cohort study of 412 patients with nonmesenteric desmoid tumors (DTs) who received local therapy at the authors’ institution between 1965 and 2018. The median follow-up time was 95 months (range, 1-509 months). Local recurrence occurred in 127 patients (31%) at a median time of 21 months (interquartile range, 12-38 months). The 5-year local control (LC) rate was 67%. Patient or tumor factors that were significantly associated with poorer 5-year LC in a multivariable analysis included an age ? 30 years (57% vs 75% for an age > 30 years; hazard ratio [HR], 1.73; P = .004), an extremity location (57% vs 71% for a nonextremity location; HR, 1.77; P = .004), and large tumors (59% for >10 cm [HR, 2.17; P = .004] and 65% for 5.1-10 cm [HR, 1.71; P = .02] vs 76% for ?5 cm). Subset analyses of these high-risk patients revealed no local therapy strategy to be superior for young patients ? 30 years old (HR for surgery, 1.42; P = .33; HR for RT, 1.36; P = .38) or for large tumors > 10 cm (HR for surgery, 1.55; P = .46; HR for RT, 0.91; P = .91). However, for patients with extremity tumors, surgery alone was significantly associated with inferior LC (HR for surgery, 5.15; P < .001; HR for RT, 1.51; P = .38). Local therapy provides durable tumor control in the majority of patients with DTs. However, young patients, patients with an extremity location, and patients with large tumors are at increased risk of recurrence. When active treatment is indicated, systemic therapy should perhaps be considered as a first-line option in these high-risk subsets. Prospective multi-institutional studies evaluating this strategy are warranted.

The treatment of desmoid tumours (DTs) has greatly evolved in recent years, and surgery is no longer considered a first-line treatment. Percutaneous biopsy with molecular analysis for beta-catenin or APC gene mutation provides a certain diagnosis. After imaging, a specialized multidisciplinary tumour board (MDT) addresses the following therapeutic strategy. As more than half of patients stabilize or regress, despite initial progression, active surveillance is offered to most patients as the first option. Surgery is proposed for complications such as perforation and occlusion, which sometimes are the first manifestations of the disease. In these cases, limiting surgery to the treatment of complications and leaving the tumour in place is possible if significant bowel sacrifice is needed, especially in patients with previous colectomy for polyposis. Medical therapy is discussed by the MDT in cases of functional or life-threatening masses and is preferred to local treatments that could be mutilating and often incomplete. First-line surgery is now contraindicated in cases of incomplete unplanned surgery, recurrence, pregnancy or DTs occurring in familial adenomatous polyposis(F.A.P.). The best indications of second-line surgery are significantly progressing disease when morbidity is acceptable, such as parietal locations. Medical and other locoregional treatments (radiotherapy, isolated limb perfusion and cryotherapy) should be considered by the MTB when surgery might cause sequelae.

A 28-year-old man visited Peking Union Medical College for a tangible abdominal mass without abdominal pain or distention. Considering the imaging characteristics and clinical manifestation, this mass was primarily diagnosed as GIST before surgery. During the surgery, the occupancy was found under the ileocecal mesentery, with grayish white appearance, tough texture, and poor mobility, which was not consistent with the character of the GIST. After the surgery, pathological examination and individual immunohistochemistry results demonstrated that the lesion was compatible with the diagnosis of retroperitoneal fibromatosis with purulent inflammation of chronic lymphadenitis. Therefore, we decided to perform tumor mass resection, right colon resection, partial duodenum resection, and intestinal anastomosis on laparotomy, but the right ureter was retained. After excision of the tumor, the ends of the intestine segment were continuously sutured. The patient experienced no intraoperative or postoperative complications, and was discharged 3 days after surgery. Periodic follow-up physical examinations such as the abdominal ultrasound and computed tomography were performed each 3 months, and no evidence of recurrence was observed during the whole 12 months.