OBJECTIVETo evaluate the safety and feasibility of radical surgery for giant desmoid in abdominal wall accompanied with the repair of giant myofascial defect using synthetic prosthesis.

METHODSWe analyzed the clinical and follow-up data of 31 patients with giant desmoid in abdominal wall undergoing radical resection and immediate abdominal wall reconstruction with synthetic prosthesis from January 2007 to January 2017 retrospectively. Patients were recruited at the Diagnostic and Therapeutic Center of Hernia and Abdominal Wall Diseases, the First Affiliated Hospital of Chinese PLA General Hospital and the Department of General Surgery, Chinese PLA General Hospital. Operative conditions, morbidity of complication, short- and long-term outcomes were summarized. All the patients underwent radical resection and infiltrated organs or tissues were simultaneously treated. Synthetic prosthesis was used to perform primary-intention reconstruction of giant myofascial defect in anterior or lateral abdominal wall. Bridging repair procedure for incisional hernia was used to perform double border fixation between prosthesis border and myofascial defect border. Placement and fixation of prosthesis followed the idea of "conformal repair", then prosthesis was finally repaired as arch in accordance with original abdominal wall.

RESULTSOf 31 patients, 28 cases were female with mean age of 35.2 (16-58) years and 3 were male with mean age of 42.6 (20-79) years. Six initial cases (19.4%) were diagnosed by preoperative biopsy, and 25 recurrent cases (60.6%) were diagnosed by medical history. The mean minimal diameter of tumors was 18.2 (14-25) cm, and the mean maximal diameter was 45.3 (32-53) cm. All 31 patients underwent radical resection and immediate abdominal wall reconstruction using synthetic prosthesis in bridging fashion successfully, and rapid pathological examination showed that all resection margins were negative. The average operative time was 335 (245-610) min, and the average intra-operative blood loss was 1260 (500-3500) ml. The size of abdominal wall defect after removal of desmoid ranged from 21 cm × 23 cm to 35 cm × 60 cm. The defects in 29 patients were repaired with compound synthetic prosthesis and the defects in 2 patients were repaired with compound prosthesis and polypropylene mesh. Four patients(12.9%) developed postoperative infection, in whom 3 patients had prosthesis infection during 1 month postoperatively, then 1 case recovered with conservative therapy, the other 2 cases were healed after the removal of infected prosthesis at 2 weeks and 3 months postoperatively, respectively; 1 patient had infection of artificial vessel prosthesis and received a second operation to remove the infected artificial vessel. The other 27 patients recovered smoothly and got primary intention wound healing. These 31 patients were followed up for a median of 60.5 (10-121) months with complete data. No marginal recurrence, incisional hernia, and abdominal wall bulge happened. One patient undergoing removal of all anterior and lateral abdominal wall had difficult defecation and urination during the first month after operation, and recovered through practising chest breathing. Ten patients developed fresh desmoids in other body positions postoperatively within 1-3 years, in whom 3 patients died of intestinal obstruction due to rapid neoplasm development and 7 patients survived with tumor receiving conservative therapy. All the 28 survival patients could restore normal life and workand have appropriate sports.

CONCLUSIONRadical resection and immediate reconstruction of giant myofascial defect using synthetic prosthesis for patients with giant desmoid in abdominal wall is safe and effective.

Abdominal Wall ; pathology ; surgery ; Adolescent ; Adult ; Female ; Fibromatosis, Aggressive ; surgery ; Hernia ; Hernia, Ventral ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Surgical Mesh ; Treatment Outcome ; Young Adult

PURPOSE: Laparoscopic approaches to the anterior abdominal wall are difficult because of the inherent limitations of laparoscopic surgery. METHODS: A 25-year-old young female visited to our hospital with an incidentally-found palpable abdominal mass. Computed tomography revealed a 7-cm round enhancing mass arising from the left posterior rectus sheath. The patient underwent laparoscopic excision of the inner mass arising from the anterior abdominal wall. RESULTS: The operation lasted for 45 minutes and there was no measurable bleeding during the procedure. Pathological assessment revealed that the tumor was a DTF 7.0 cm in size. The patient was discharged on the second postoperative day in good condition, and is currently being followed on a routine basis for surveillance without adjuvant therapy. CONCLUSION: Laparoscopic approach for the anterior abdominal wall tumor could be feasible in select patients. This minimally-invasive approach helps to ensure good cosmetic outcomes and quality of life.
Abdominal Wall* ; Adult ; Female ; Fibroma* ; Fibromatosis, Abdominal ; Hemorrhage ; Humans ; Laparoscopy ; Quality of Life

PURPOSE: To evaluate the imaging findings of desmoid tumors using various imaging modalities and to evaluate whether diffusion-weighted imaging (DWI) can help differentiate between desmoid and malignant tumors. MATERIALS AND METHODS: The study included 27 patients with pathologically confirmed desmoid tumors. Two radiologists reviewed 23 computed tomography (CT), 12 magnetic resonance imaging (MRI) and 8 positron emission tomography-computed tomography (PET-CT) scans of desmoid tumors and recorded data regarding the shape, multiplicity, size, location, degree of enhancement, and presence or absence of calcification or hemorrhage. The signal intensity of masses on T1- and T2-weighted imaging and the presence or absence of whirling or band-like low signal intensity on T2-weighted imaging were recorded. The apparent diffusion coefficient (ADC) values of the desmoid tumors in nine patients with DWIs were compared with the ADC values of 32 malignant tumors. The maximum standardized uptake value (SUV(max)) on PET-CT images was measured in 8 patients who underwent a PET-CT. RESULTS: The mean size of the 27 tumors was 6.77 cm (range, 2.5-26 cm) and four tumors exhibited multiplicity. The desmoid tumors were classified by shape as either mass forming (n = 18), infiltrative (n = 4), or combined (n = 5). The location of the tumors was either intra-abdominal (n = 15), within the abdominal wall (n = 8) or extra-abdominal (n = 4). Among the 27 tumors, 21 showed moderate to marked enhancement and 22 showed homogeneous enhancement. Two tumors showed calcifications and one displayed hemorrhage. Eleven of the 12 MR T2-weighted images showed whirling or band-like low signal intensity areas in the mass. The mean ADC value of the desmoid tumors (1493 × 10⁻⁶ mm²/s) was significantly higher than the mean of the malignant soft tissue tumors (873 × 10⁻⁶ mm²/s, P < 0.001). On the PET-CT images, all tumors exhibited an intermediate SUV(max) (mean, 3.7; range, 2.3–4.5). CONCLUSION: Desmoids tumors showed homogenous, moderate to marked enhancement on CT and MRI scans and a characteristic whirling or band-like pattern on T2-weighted images. DWI can be useful for the differentiation of desmoid tumors from malignant soft tissue tumors.
Abdominal Wall ; Diffusion* ; Electrons ; Fibroma ; Fibromatosis, Aggressive* ; Hemorrhage ; Humans ; Magnetic Resonance Imaging

Desmoid tumors are rare soft tissue tumors considered to have locally infiltrative features without distant metastasis until now. Although they are most commonly intraabdominal, very few cases have extra-abdominal locations. The origin of intrathoracic desmoid tumors is predominantly the chest wall with occasional involvement of pleura. True intrathoracic primary desmoid tumors with no involvement of the chest wall or pleura are extremely rare. We recently experienced a case of true intrathoracic desmoid tumor presenting as multiple lung nodules at 13 years after resection of a previous intraabdominal desmoid tumor.
Abdominal Wall* ; Fibromatosis, Aggressive* ; Lung* ; Multiple Pulmonary Nodules ; Neoplasm Metastasis ; Pleura ; Thoracic Wall ; Thorax

Familial adenomatous polyposis (FAP) is characterized by large numbers of adenomatous polyps in the colon and inherited as an autosomal dominant disease. Gardner's syndrome is a form of FAP accompanied by extra-colonic tumors and desmoid tumors. Desmoid tumors are rare, and benign tumors characterized by fibroblastic proliferation of fascial and musculoaponeurotic components. There is an approximate 1,000 times higher incidence of desmoid tumors in patients with FAP compared with the general population. Desmoid tumors in Gardner's syndrome occur in the small bowel mesentery in 80% of all cases, and the other 20% in the abdominal wall or the extremities. Almost all cases of desmoid tumors in Gardner's syndrome were incidentally found after prophylactic total proctocolectomy for colon cancer prevention in the patients with FAP. We report a case of Gardner's syndrome associated with codon 1099 mutation of the adenomatous polyposis coli gene, in which the patient was initially found to have desmoid tumors and subsequently diagnosed as FAP by screening colonoscopy.
Abdominal Wall ; Adenomatous Polyposis Coli ; Adenomatous Polyps ; Codon ; Colon ; Colonic Neoplasms ; Colonoscopy ; Extremities ; Fibroblasts ; Fibromatosis, Aggressive ; Gardner Syndrome ; Humans ; Incidence ; Mass Screening ; Mesentery

Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal fibromatosis is distinguishable from other forms of fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3x4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear beta-catenin staining.
Abdominal Wall ; beta Catenin ; Child* ; Collagen ; Female ; Fibroblasts ; Fibroma ; Fibromatosis, Abdominal* ; Humans ; Infant ; Necrosis ; Pregnancy ; Vimentin

OBJECTIVETo study the clinicopathologic features, immunophenotype and differential diagnosis of plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach.

METHODSThe clinical and pathologic findings of 3 cases of PAMT in the gastric antrum were retrospectively analyzed. Immunohistochemical study was carried out and the literature was reviewed.

RESULTSThe age of patients ranged from 31 to 47 years. The male-to-female ratio was 1:2. The clinical presentation included epigastric pain and distension. Endoscopically, the tumor mass protruded into the gastric cavity at the antrum and ranged from 4.5 cm to 8.0 cm in greatest dimension. One of the tumors studied was associated with surface ulceration. Histologically, the tumors were located in the gastric wall. They were composed of bland spindle cells and small vessels arranged in a plexiform or nodular pattern within a myxoid stroma. Immunohistochemical study showed that the spindle cells were consistently positive for smooth muscle actin and muscle-specific actin. There was focal staining for h-caldesmon, desmin in case 3 and focal positive for epithelial membrane antigen, CAM5.2 in case 1. Further, CD10 and progesterone receptor were positive in case 3.

CONCLUSIONSPAMT represents a rare novel mesenchymal tumor of the stomach, with a propensity of gastric antral involvement. The distinctive pathologic features help to differentiate this entity from other benign and malignant tumors.

Actins ; metabolism ; Adenomyoma ; metabolism ; pathology ; Adult ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Fibromatosis, Abdominal ; metabolism ; pathology ; Follow-Up Studies ; Gastrectomy ; methods ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Humans ; Male ; Middle Aged ; Myofibroma ; metabolism ; pathology ; surgery ; Myxoma ; metabolism ; pathology ; surgery ; Proto-Oncogene Proteins c-kit ; metabolism ; Stomach Neoplasms ; metabolism ; pathology ; surgery

Female ; Fibromatosis, Abdominal ; diagnostic imaging ; pathology ; surgery ; Humans ; Middle Aged ; Radiography ; Rupture, Spontaneous ; surgery

Mesenteric fibromatosis is a monoclonal, fibroblastic proliferation arising from musculoaponeurotic structure, and it is distinctive lesions defined as a group of non-metastasizing fibroblastic tumors which has local invasion and has a high recurrence rate after the surgical excision. The main treatment modality is the surgical excision. Radiation therapy, chemotherapy, and hormone therapy are also known as useful treatments. We report our experience of a recent case of Mesenteric fibromatosis. A 62-year old female patient had undergone gastrectomy due to gastric cancer. 18 months after gastrectomy, we detected an abdominal mass. The preoperative radiologic findings were suggestive of recurrence. Exploratory laparotomy was performed and post-operative pathologic diagnosis was confirmed as fibromatosis. We report a patient with mesenteric fibromatosis that mimic recurrence after gastrectomy for gastric cancer.
Female ; Fibroblasts ; Fibroma ; Fibromatosis, Abdominal ; Gastrectomy ; Humans ; Hydrazines ; Laparotomy ; Recurrence ; Stomach Neoplasms

Mesenteric fibromatosis is a rare benign disease characterized by proliferating fibrous tissue in the bowel mesentery. We report a case of aggressive mesenteric fibromatosis with ureteral stenosis arising in a 46-year-old woman who suffered from intermittent right abdominal pain. Computed tomography revealed a right retroperitoneal mass with right ureteral stenosis at the level of the right common iliac vessel. The mass was excised with resection of the affected segment of the ileum, ascending colon, and ureter, and end-to-end ureter anastomosis was performed. Pathological examination confirmed mesenteric fibromatosis.
Abdominal Pain ; Colon, Ascending ; Constriction, Pathologic* ; Female ; Fibroma* ; Fibromatosis, Aggressive ; Humans ; Ileum ; Mesentery ; Middle Aged ; Retroperitoneal Neoplasms ; Ureter*