[A Case of Desmoid Fibromatosis of the Small Intestinal Mesentery Institutions].

The patient was a 27-year-old man. He was referred to our hospital because he was aware of a mass in his abdomen. An abdominal ultrasound showed a 70-mm mass lesion. Enhanced computed tomography showed a 70-mm mass with well- defined margins and heterogeneous internal enhancement near the proximal jejunum. The patient was diagnosed with a suspected primary submucosal tumor of the duodenum or small intestine, and surgery was planned to diagnose and treat the tumor. The tumor was located in the upper jejunal mesentery, and tumor resection and partial small bowel resection were performed. Histopathological examination revealed proliferation of spindle-shaped cells without karyomitosis, and mixed collagen fibers in the tissue. Immunohistochemistry showed ß-catenin(+), SMA(+), AE1/AE3(-), KIT(-), CD34(-), and S-100(-). Based on these findings, we diagnosed primary desmoid fibromatosis of the small intestinal mesentery. In this report, we describe a case of primary desmoid fibromatosis of the small intestinal mesentery with a review of the literature.

[A Case of Giant Abdominal Desmoid Tumor in a Young Man].

Desmoid tumor is a rare tumor of the soft tissue. The frequency of occurrence is 2.4 to 4.3 cases per year per million people, which is a very rare disease. We experienced a huge intra-abdominal desmoid tumor which is thought to be the primary mesentery. The case was a male in his 20s. He visited a nearby doctor with a complaint of abdominal bloating and abdominal pain. Abdominal contrast CT revealed a huge abdominal mass with a clear boundary of 35×25 cm in size extending from the upper right abdomen to the pelvis. Surgery was performed with a diagnosis of an intra-abdominal mass. Open abdominal tumor resection. Due to infiltration into the duodenum, transverse colon, and pancreas, right hemicolectomy and duodenal combined resection were performed. The pathological diagnosis was a diagnosis of desmoid tumor.

Unusual and rare cause of abdominal pain: abdominal desmoid fibromatosis masquerading as a postoperative haematoma.

Desmoid fibromatosis is a rare connective tissue malignancy. It can occur in a variety of locations, including the abdominal wall, extremities and abdominal cavity. There has been an association with development in a prior surgical scar. Common symptoms can vary depending on the location and can include being painless to having pain at the site, functional impairment and bowel obstruction from intra-abdominal masses. In the following report, we discuss a case in which a patient's abdominal pain was attributed to a postoperative haematoma based on CT radiographic features; however, further work-up and biopsy yielded desmoid fibromatosis, a rare locally aggressive malignancy.

[A Case of Intra-Abdominal Desmoid Tumor after Laparoscopic Resection of Colon Cancer].

An 84-year-old man underwent laparoscopic descending colon resection for colon cancer with stage Ⅰ. Follow-up computed tomography(CT), 18 months after surgery showed a soft tissue density nodular mass, 30 mm in size, in the small intestinal mesentery. The surgical resection of the tumor was performed after a thorough examination. Histopathological examination showed spindle-shaped fibroblasts and abundant collagen fibers. Immunohistochemical staining was negative for c-kit and CD34 and positive for α-SMA and ß-catenin. From the above, this tumor was diagnosed as intra-abdominal desmoid tumor.

Giant mesenteric fibromatosis associated with non-Hodgkin lymphoma. A case report and literature review.

BACKGROUND: Mesenteric fibromatosis is a benign locally-aggressive mesenchymal neoplasm that lacks the potential for metastasis. It is related to Gardner's Syndrome, previous trauma, abdominal surgery, and prolonged intake of oestrogen. Differentially diagnosing this from similar tumours is crucial in order for establishing the appropriate treatment and only immunohistochemical features can be used for a definitive diagnosis. Although medical therapies play a role in the treatment of mesenteric fibromatosis, surgical resection is the gold-standard procedure. METHODS: Our case study is a 40-year-old male with a concomitant diagnosis of non-Hodgkin lymphoma and mesenteric fibromatosis, not associated with any of the risk factors mentioned above. We performed CT and PET scans and observed a vascularised and well-defined mesenteric centre-abdominal hypermetabolic solid mass in contact with the gastric body, duodenum, body and tail of the pancreas, transverse colon, and spleen. An ultrasound-guided tru-cut biopsy revealed features suggestive of mesenteric fibromatosis. RESULTS: An elective laparotomy was carried out and a giant mass, arising from mesentery, was excised, including a partial gastrectomy and segmental resection of the transverse colon. Distal pancreatectomy, small bowel resection and successive splenectomy were performed due to a large hypertensive component. The postoperative period was uneventful. The histopathology of the surgical pieces was compatible with intra-abdominal desmoid fibromatosis. CONCLUSION: As far as we know from the literature, this is the largest mesenteric fibromatosis tumour ever to be excised. We also noticed that this is the first reported case of the concomitant presence of mesenteric fibromatosis and non-Hodgkin lymphoma that is not related to any of the described risk factors. Further research is needed to establish what type of association this presentation may indicate.

[A Case of Solitary Desmoid Tumor after Laparoscopic Sigmoid Colon Cancer Resection].

A 71-year-old man with pathological Stage Ⅰ(pT1bN0M0)underwent laparoscopic sigmoid colon cancer resection. After 18 months postoperatively, follow-up computed tomography(CT)showed a 30 mm enhanced soft tissue tumor near the anastomotic site. Considering the magnetic resonance imaging(MRI)and positron emission tomography(PET)results, we diagnosed sigmoid colon cancer with local recurrence. Laparoscopic radical resection of the colon and intestine, including the tumor, was performed. Pathologically, the tumor comprised spindle-shaped cells with collagen fibers and was diagnosed as a desmoid tumor by immunostaining(ß-catenin+, c-kit-, CD34-, α-SMA-, and DOG-1-). We report a case of intra-abdominal desmoid tumor near the anastomotic site after laparoscopic sigmoid colon cancer resection.

Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study.

BACKGROUND: Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. QUESTIONS/PURPOSE: This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. METHODS: This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF. RESULTS: Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3-85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. CONCLUSION: Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.

Desmoid tumours in the surveillance era: What are the remaining indications for surgery?

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 (FAP). 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.

Retroperitoneal fibromatosis presenting as a mesenteric mass: A case report.

RATIONALE: Fibromatoses or desmoid tumors are relatively rare tumors derived from the musculoaponeurotic system. This tumor has no specific clinical symptoms and it is sometimes misdiagnosed as other diseases such as gastrointestinal stromal tumors (GISTs). PATIENT CONCERNS: A 28-year-old man visited Peking Union Medical College for a tangible abdominal mass without abdominal pain or distention. DIAGNOSES: 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. INTERVENTIONS: 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. OUTCOMES: 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. LESSONS: To sum up, intra-abdominal fibromatosis is an extremely rare tumor that must be differentiated from other tumors of the digestive tract, and pathological and immunohistochemical examination is a critical part of the diagnosis. Early diagnosis of fibromatosis is essential for the outcome. Extensive resection of the mass minimizes the risk of relapse.

Primary mesenteric fibromatosis: A single Center experience.

AIM: Primary mesenteric fibromatosis is a rare, locally invasive, non-metastasizing type of intra-abdominal fibromatoses with a very high rate of recurrence. In this study, we aimed to present our surgical approach, tumor characteristics, clinical presentation and long-term follow-up results in cases of primary mesenteric fibromatosis. MATERIAL AND METODS: The data collected from 11 patients who underwent surgery due to primary mesenteric fibromatosis in our clinic between 2010 and 2019 were analyzed retrospectively. RESULTS: Abdominal ipain, abdominal distention, and two patients (18.2%) were operated on with a diagnosis of acute abdomen in the emergency setting due to mechanical bowel obstruction in one patient There were 11 patients in our study. Six patients were female and 5 were male. The mean age was 44.2±15.8 years. Abdominal mass was detected in 5 patients (45.5%) who had complaints of mechanical bowel obstruction such as nausea and vomitingand gastrointestinal perforation in other patient. Mesenteric mass was detected in 3 patients (27.3%) with vague abdominal pain. One patient (9.1%) presented with abdominal pain and swelling of the right leg. After a mean follow-up period of 43.4±28.4 months, only 1 patient (9.1%) had recurrence and required reoperation approximately 80 months after the first operation. One patient (9.1%) died of anastomotic leakage and sepsis in the first 30 days postoperatively, and other patient (9.1%) idied of other reasons 1 year later postoperatively. CONCLUSIONS: Although mesenteric fibromatosis is a benign tumor pathologically, the main principle in the treatment of this tumor which is clinically aggressive and has high recurrence rate is wide surgical resection. Mesenteric fibromatoses have a varied clinical presentation. Radiological imaging methods helps diagnosis and planning the surgical treatment. Immunohistochemical characteristics confirms the diagnosis and differentiates from other similar tumors. KEY WORDS: Desmoid tumor, Fibromatosis, Mesentery, Mesenteric tumor,Mesenteric fibromatosis.

Mesenteric Fibromatosis: A Case Report.

Fibromatosis of mesentery is a very rare disease and only a few cases have been reported. Here we report a case of 40 year old man admitted on 16 October, 2017 in the Department of Surgery (Unit-3) of Mymensingh Medical College Hospital with history of recurrent abdominal pain and progressively enlarging intra-abdominal lump for 2 years. Out of different diagnostic dilemmas, finally we came to the conclusion of its being a mass lesion primarily arising from the mesentery and involving the adjoining gut according to the report of CT abdomen. Laparotomy was performed and a lump measuring about 18 cm × 20 cm arising from mesentery was found, in which distal part of jejunum and proximal part of ileum were embedded. The mass was excised with incorporated gut loops and histopathology report revealed fibromatosis of mesentery.

Not all abdominal masses after colorectal cancer surgery are malignant: intra-abdominal fibromatosis masquerading as recurrence.

AIM: Intra-abdominal fibromatosis is an unusual mesenchymal tumour that can be locally aggressive without any metastatic potential. Fibromatosis may simulate cancer recurrence on imaging surveillance for colorectal cancer follow-up. The optimal treatment of recurrent peritoneal malignancy is cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Confirmatory biopsy of lesions suspicious for colorectal cancer recurrence may not be feasible, thereby rendering surgery the safest option. Our aim was to determine the incidence of fibromatosis in a cohort of patients undergoing CRS and HIPEC for suspected colorectal cancer recurrence. METHODS: One hundred and seventy-one CRS and HIPEC cases were performed at our Peritoneal Malignancy Institute between February 2007 and October 2018 for colorectal peritoneal metastases and were included in a prospectively maintained database. RESULTS: A total of 49 (29%) of 171 cases were performed for primary colorectal cancer with peritoneal metastases, whereas 122 (71%) of 171 cases were performed for suspected colorectal cancer recurrence detected on surveillance imaging after primary colorectal cancer resection. On histological analysis of the resected specimen, five (4.1%) of 122 cases undergoing CRS and HIPEC for colorectal recurrence had fibromatosis. CONCLUSION: Fibromatosis can masquerade as colorectal cancer recurrence. In this series it occurred with an incidence of 4.1% among a cohort of patients undergoing CRS and HIPEC for probable recurrence. Surgical resection may be the only option to confirm the diagnosis and rule out malignancy.

[Two Cases of Resectable Intra-Abdominal Desmoid Tumor after Laparoscopic Gastrectomy for Gastric Cancer].

Case 1: A 52-year-old man underwent laparoscopic total gastrectomy for gastric cancer. After 2 years, a follow-up computed tomography(CT)scan showed a large, solid 10 cm-sized mass in his left upper abdomen. Under the diagnosis of a suspected mesenchymal tumor, a tumor resection with a partial resection of the upper jejunum and transverse colon was performed. Case 2: A 61-year-old man underwent laparoscopic pylorus-preserving gastrectomy for gastric cancer. After 1.5 years, follow-up CT showed a tumor of 2 cm in diameter near the greater curvature side of the upper stomach. Under the diagnosis of a suspected gastrointestinal stromal tumor(GIST), a laparoscopic partial resection of the stomach was performed. Histologically, spindle-shaped cells without atypia and rich collagen fibers were observed, and the sample was positive for b-catenin by immunostaining in both cases; from this evidence, the patients were diagnosed with desmoid tumors. Desmoid tumors have invasive proliferation characteristics, and treatment requires consideration of the balance between securing a surgical margin and increasing surgical stress.