Large desmoid tumour of the small bowel mesentery.

A 74-year-old man was being investigated for a pancreatic insulinoma when an incidental mesenteric mass measuring 2.6 cm x 2.5 cm was noticed on CT imaging. A wait-and-see approach was decided on. Thirty-nine months later, the patient presented with symptoms of abdominal obstruction. CT images revealed the mesenteric mass filled majority of the abdominal cavity and measured 29 cm x 26 cm x 16 cm. The patient underwent an open bypass gastrojejunostomy which stopped working a few weeks later due to further compression by the tumour. A debulking surgery was performed: a right hemicolectomy and small bowel resection with excision of the desmoid tumour and bypass gastrojejunostomy. The tumour measured 12.6 kg and was macroscopically visualised to have a white cut surface with a focal translucent area. Microscopic analysis revealed bland spindle cells with pale eosinophilic cytoplasm showing no cytological atypia, in keeping with a mesenteric desmoid tumour. Currently, two and a half years from the debulking surgery, the patient remains well and in remission with planned surveillance.

Familial adenomatous polyposis associated with desmoid tumors presenting with abdominal abscess: A case report and literature review.

RATIONALE: Familial adenomatous polyposis (FAP) associated with desmoids tumors (DTs) complicated by abscess formation is rare. The management is not easy and the choice of the best treatment may be controversial. PATIENT CONCERNS: A 33-year-old man was admitted to our hospital for abdominal pain, fever, chills, nausea, and vomiting. He had a family history of FAP, and history of abdominal surgery. DIAGNOSES: An abdominal enhanced chest computed tomography (CT) scan revealed a soft tissue mass in the abdominal wall and an irregular mesenteric soft tissue mass with internal fistula and intra-abdominal abscess. A CT-guided biopsy of the abdominal wall mass revealed DTs. INTERVENTIONS: The patient was given oral antibiotics for 6 months, and ultimately underwent surgery. OUTCOMES: The patient had no evidence of recurrence on follow-up at 10 months. LESSONS: This case indicates that for patients with FAP who have a history of abdominal surgery and a progressively enlarging mass and abscess in the abdomen, it is necessary to consider the possibility of DTs. FAP-related DTs are rarely complicated by abscess formation. Antibiotic therapy plus surgical resection of the tumor may be effective and make good prognosis.

Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports.

BACKGROUND: Intra-abdominal desmoid tumors are rare soft tissue tumors that arise mainly in the mesentery and pelvis. Their etiology may include genetic mutations, estrogen-associated changes after childbirth, and mechanical factors such as a history of abdominal surgery. However, there are cases of intra-abdominal desmoid tumors that develop in the absence of such causes. Since they are rare, diagnosis is often difficult based on clinical findings. We encountered two cases of patients with sporadic intra-abdominal desmoid tumors with a very unusual onset and contrasting features. CASE PRESENTATION: The first patient was a 51-year-old asian man who presented with sudden onset of abdominal pain. He was referred to our department because of a giant tumor detected on abdominal ultrasonography. Imaging revealed a 19-cm tumor with internal tumoral hemorrhage; however, no definitive diagnosis was made. Tumor resection was performed for diagnostic and therapeutic purposes. The second patient was a 41-year-old asian man, and right hydronephrosis was detected on abdominal ultrasonography during a periodic medical checkup. We diagnosed invasion of the primary mesenteric tumor into the right ureter using diagnostic imaging and performed ileocecal resection with partial right ureteral resection for a definitive diagnosis and therapeutic purposes. Although the tumors of both patients had developed from the ileal mesentery, the tumors were substantially different from each other based on their imaging findings, macroscopic morphology, and progression pattern. Meanwhile, they showed similar pathological characteristics. Both consisted of bundles of collagen fibrils of spindle-shaped fibroblasts with low cell atypia. Moreover, they were diagnosed as desmoid tumors using positive immunohistochemical staining for ß-catenin. CONCLUSIONS: Neither patient had susceptibility factors for desmoid tumors, and to our knowledge, there have been very few reports to date of intra-abdominal desmoid tumors that were diagnosed because of acute abdominal pain caused by tumoral hemorrhage or asymptomatic obstructive uropathy. Furthermore, it is clinically interesting that the two patients showed contrasting progression patterns and imaging findings. Intra-abdominal desmoid tumors are rare and may present with various symptoms and findings similar to those observed in our patients. Diagnosis therefore requires experience and knowledge that is not bound by preconceptions.

Surgical management of a giant abdominal wall desmoid tumour.

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.

A Giant Solid-Cystic Mesenteric Fibromatosis with Unusual Synchronous Skin Fibromatosis.

Mesenteric fibromatosis (MF) is a rare, locally aggressive tumor without distant metastasis, which has a high recurrence rate. Based on its location, it is classified as intra-abdominal, from abdominal wall, and extra-abdominal. The incidence of cystic-solid, retroperitoneal tumors is very low in comparison to other MF forms. Intra-abdominal MFs are asymptomatic in early stages, but their symptoms appear late in the tumor course. There is no specific imaging finding since radiological diagnosis is mostly impossible. Thus, diagnosis is made histopathologically. Nowadays, there is no consensus about its treatment although surgical resection is widely used. In the present study, a very rare case of cystic-solid retroperitoneal MF associated with separate synchronous skin tumors is reported.

Intra-abdominal small intestinal desmoid tumour mimicking GIST.

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.

Giant desmoid tumour of abdominal wall: resection and reconstruction by posterior component separation.

Management of large abdominal wall desmoid tumours is complicated due to the unpredictable behaviour of desmoids and the need for laborious reconstruction of the abdominal wall after wide local excision. A multidisciplinary team approach, including surgeons, oncologists and plastic surgeons, is necessary for proper management. This case highlights the diagnostic and surgical challenges related to the reconstruction of abdominal wall defect, after radical excision of a 30×30×25 cm desmoid tumour, originating from left rectus muscle. The defect was closed successfully by a perspicuous technique of posterior component separation. The awareness of this straightforward technique will allow the surgeons to do these radical procedures with confidence and without any consternation of complex reconstructive procedures.

Gardner syndrome with giant abdominal desmoid tumor during pregnancy: a case report.

BACKGROUND: Gardner syndrome is a subtype of familial adenomatous polyposis (FAP), characterized by a combination of adenomatous intestinal polyps and extracolonic lesions such as multiple osteomas, dental abnormalities, and soft tissue tumors. Although 12% of patients with intestinal polyposis of FAP may occur intra-abdominal desmoid tumors, pregnancy complicating with giant abdominal desmoid tumors is a relatively rare case. CASE PRESENTATION: A 28-year-old pregnant woman was diagnosed with Gardner syndrome in whom an intra-abdominal tumor was found a year after undergoing a laparoscopic total colectomy due to family adenomatous polyposis. At 32 weeks' gestation, she presented to our department for the third time complaining upper abdominal pain caused by the giant abdominal mass about 21 × 12 cm2 in size. After multidisciplinary consultation and discussion, the decision of fetal preservation treatment was made. After the delivery of a baby girl, abdominal mass resection was performed, and pathological examination revealed a fibrous adenoma. The patient was discharged after a week and was uneventful in the follow-up for half a year. CONCLUSIONS: Gardner syndrome is characterized by typical syndrome including family adenomatous polyposis and extra-intestinal tissue tumor. Were desmoid tumors rarely as large as fetus and local aggressively. In our case, we selected surgery to remove the intra-abdominal desmoid tumor after the natural delivery of the fetus and no abnormalities were observed during the 6 months follow-up. Women during pregnancy have an increased risk for the development of desmoid tumors, likely with the sex hormone to be one of the triggers. Therefore, we suggested that when a patient with Gardner syndrome desire to conceive again, they should go to the hospital for a regular review at least once every 3 months.

Differentiation of intra-abdominal desmoid tumor from peritoneal seeding based on CT and/or 18F-FDG PET-CT in patients with history of cancer surgery.

PURPOSE: To investigate differential imaging features of intra-abdominal desmoid tumors and peritoneal seeding in patients with history of cancer surgery. METHODS: Thirty-two patients who had a single pathologically proven intra-peritoneal lesion that developed after cancer surgery were enrolled between January 2000 and June 2019. There were 16 desmoid tumors and 16 peritoneal seeding lesions. Portal phase CT and/or 18F-FDG PET findings were analyzed by two radiologists in consensus for the following items: location, size, shape, margin, contour, homogeneity, necrosis, adjacent organ invasion, calcification, intra-lesional fat, peritoneal infiltration, mass effect, and degree of enhancement. Hounsfield units (HU) and maximum standardized uptake values (SUVmax) of the lesions were measured. Imaging findings were compared using the Chi square test, Fisher's exact test, and student t test. RESULTS: Desmoid tumors frequently showed well-defined margins (9/16) and smooth contours (12/16), whereas peritoneal seeding had ill-defined margins (13/16) and lobulated contours (11/16) (P = 0.028 and 0.013, respectively). Intra-lesional fat was found more frequently in desmoid tumors (7/16) than peritoneal seeding (1/16) (P = 0.014). Desmoid tumors showed iso-attenuation (13/16) compared to psoas muscle in portal phase, while peritoneal seeding depicted high attenuation (12/16) (P = 0.002). Mean HU was significantly lower in desmoid tumors (64.3) than peritoneal seeding lesions (95.1) (P = 0.001). However, the mean SUVmax of desmoid tumors (4.1) did not significantly differ from peritoneal seeding lesions (5.2) (P = 0.519). CONCLUSION: Several CT features including iso-attenuation in portal phase and presence of intra-lesional fat can be helpful in differentiating desmoid tumors from peritoneal seeding in patients with history of intra-abdominal cancer surgery.

Intra-abdominal desmoid tumor and chronic inflammatory bowel disease. An uncommon scenario.

We present the case of a patient with an inflammatory bowel disease to whom a pelvic desmoid tumor is discovered during an infertility study.

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.

A giant mesenteric fibromatosis involving the muscular layer of the colon wall: A case report.

RATIONALE: Mesenteric fibromatosis (MF) is a rare tumor whose biological behavior is intermediate between benign fibrous neoplasms and fibrosarcomas, and the characteristic of these tumors are local aggressive lesions which is prone to local recurrence but non-metastasizing. The common symptom is abdominal distention or painless mass. We report a case of giant MF in abdominal cavity with abdominal distention as the main symptom. PATIENT CONCERNS: A 26-year-old male presented with 2-month history of abdominal distention, lack of appetite, and symptoms grew progressively more debilitating with time. DIAGNOSES: This patient underwent a contrast-enhanced computed tomography scan which showed a giant (37 × 25 × 13 cm), inhomogeneous enhancing, well-defined, and soft tissue density mass in abdominal cavity, possibly arising in mesocolon, which suggested a high possibility of MF. The postoperative pathology showed that the tumor cells to be positive for ß-catenin, vimentin, negative for CD34, CD117, DOG-1, S-100, Desmin, which confirmed the diagnosis of MF. INTERVENTIONS: Exploratory laparotomy was performed, which revealed a large mass involving the transverse colon wall, the root of mesocolon, and encasing the middle colic vessels and the 1st branch of jejunal arteries. The complete surgical resection was performed and the mass weighted 10 kilograms (kg). OUTCOMES: The patient recovered uneventfully and was discharged 9 days after surgery. Three-month, 6-month, 12-month and 18-month on follow-up after surgery, showed no evidence of recurrence. LESSONS: The MF is a very rare tumor, especially a giant tumor (10 kg) involving the muscular layer of colon wall. In addition, treatment of giant MF still remains a challenge. We consider that surgical resection with negative margins is the goal but not at the expense of damaging the function of vital organs. Specific measures should be considered based on the individual patient in order to relieve symptoms and improve quality of life.

Desmoid-type fibromatosis mimicking cystic retroperitoneal mass: case report and literature review.

BACKGROUND: Retroperitoneal desmoid-type fibromatosis (DF) is an uncommon mesenchymal neoplasm presenting as a firm mass with locally aggressive features. It usually manifests as a well-circumscribed or ill-defined, solid mass on cross-sectional imaging. Cystic changes of DF have been described in the literature in association with prolonged medical treatment or abscess formation. However, spontaneous cystic change is rarely reported. CASE PRESENTATION: Here we report the case of a 46-year-old patient with a DF mimicked a large cystic tumor in the retroperitoneum. Ultrasonography and computed tomography were performed in order to search for localizations and characteristics of the cystic tumor. Radiological findings showed an oval cystic mass with a relatively thick wall, measuring 18.3 × 12.3 × 21.5 cm in the left upper abdomen. Laparoscopic spleen-preserving distal pancreatectomy was performed and histopathological examination by immunohistochemical study enabled us to diagnose a DF invading the pancreatic parenchyma. The patient remained asymptomatic during an 8-month follow up period. CONCLUSIONS: We report an extremely rare case of retroperitoneal DF with spontaneous cystic change. DF can manifest as a mainly cystic mass with a thick wall, as in our case, which makes the correct diagnosis difficult. DF should be included in the preoperative differential diagnosis of a cystic retroperitoneal mass, regardless of its rarity.

Desmoid tumor of the mesentery in a patient after restorative proctocolectomy as a result of familial adenomatous polyposis - case reports.

Desmoid (desmoid tumor) is a cytologically benign fibrous tumor that originates from musculoskeletal structures of the entire body [1]. The term "desmoid", first introduced by Muller in 1838, derives from the Greek word desmos, which means tendinous [2]. The etiopathogenesis of desmoid is not fully understood, most reports of publications regarding its treatment are based on individual case reports. The prognosis is good, and healing is achieved mainly through surgical excision of the lesion. The aim of the paper is to present a case of a 33-year-old woman with diagnosed familial polyposis in the colon, who has been identified with mesenteric desmoid.ic desmoid.