Nine cases of mesenteric desmoid-type fibromatosis were diagnosed and treated in Taizhou Hospital, Wenzhou Medical University between January 2010 and May 2022, including 2 females and 7 males, aged 16 to 59 years. The lesions were in the mesentery of small intestine with 7 cases, ileocecal junction with 1 cases and transverse colon with 1 case. The tumors had an unclear boundary and no envelope, the section was solid, gray and tough. The mean maximum diameter was (10.7±8.5) cm (range 3.5-33.0 cm). Microscopically, fusiform fibroblasts and myofibroblasts were parallel, bunched or staggered, buried in a large amount of extracellular collagen. The cell morphology was relatively consistent, without obvious atypia, and mitosis was rare. Immunohistochemistry showed that the tumor cells were positive for vimentin (9/9), β-catenin (9/9), while smooth muscle actin (5/9) stains were focally positive. Ki-67 proliferation index was 1%-10%. Cytokeratin Pan, S-100, STAT6, CD117, DOG1, CD34, desmin and anaplastic lymphoma kinase stains were negative. Genetic analysis showed that there were 7 cases of c.121G>A(p.Thr41Ala) mutation of CTNNB1 gene, 1 case of c.121G>A(p.Thr41Ala) and 1 case of c.134C>T(p.Ser45Phe) double mutation, and 1 case of wild type. Tumors were surgically resected in all 9 cases. Eight cases had no recurrence or metastasis, 1 case had recurrence 6 months later, and no recurrence or metastasis after additional surgical resection.
Male ; Female ; Humans ; Fibromatosis, Aggressive/diagnosis* ; Immunohistochemistry ; Fibroblasts/metabolism* ; Mesentery/pathology* ; beta Catenin/analysis*

Intra-abdominal desmoid tumor (IADT) and gastrointestinal stromal tumor (GIST) are both mesenchymal tumors mostly found in gastrointestinal tracts and easily misdiagnosed, which would directly damage the survival prognosis and quality of life of patients. With the advent of the era of precision medicine, the understanding of the above two diseases is more in-depth, and the requirements for accurate diagnosis and individualized precision treatment are more stringent. Moreover, there seems to be some internal relationship between IADT and GIST, and the lack of systematic research and discussion makes clinical decision-making and patient management easy to fall into traps and misunderstandings. Therefore, this paper reviews the clinical characteristics, pathogenesis and treatments of the two, and explore their differences and internal relations, so as to provide research and practical reference for promoting more precise and individualized diagnosis and treatment regimens.
Clinical Decision-Making ; Fibromatosis, Aggressive/diagnosis* ; Gastrointestinal Stromal Tumors/diagnosis* ; Humans ; Prognosis ; Quality of Life

PURPOSE: Desmoid tumors are locally aggressive tumors with no known potential for metastasis. They tend to recur even after complete excision. Sometimes it is not easy to differentiate between intra-abdominal desmoid and tumor recurrence, especially after gastrointestinal (GI) tumor resection. The current study aims to review the characteristics, management, and outcomes of patients with intra-abdominal desmoid tumor post GI resection.METHODS: During the period between 2007 and 2018, after a retrospective review of patients' clinical data, 10 patients were finally included. Medical records were screened for demographic, clinical, pathological data, management strategy, postoperative morbidity, mortality, recurrence rate and follow-up.RESULTS: The study comprised 10 patients (8 males). The median age was 53.5 years (range, 35–68 years). Two patients diagnosed as familial adenomatous polyposis (FAP). All the patients underwent previous GI resection: three (30%) for colon cancer, three (30%) gastrectomy, two (20%) total proctocolectomy with ileal pouch-anal anastomosis (TPC+IPAA) for FAP, one (10%) low anterior resection (three rectal cancers) and one (10%) distal pancreatectomy. The tumor was found to be in bowel mesentery in eight cases (80%). The median tumor size was 5.3 cm (range, 2.6–19.0 cm). Six patients (60%) underwent open resection, while four patients (40%) underwent laparoscopic surgery. Complications occurred in five cases (50%) and ranged from Clavien-Dindo (II-III). The median follow-up period was 16.5 months (1.5–136.0 months) with recurrence in one case (10%). Pathology came out to be desmoid tumor fibromatosis in all cases.CONCLUSION: When a mass develops after surgical resection for abdominal GI malignancy and tends to be large in size, located in the bowel mesentery and away from previous primary tumor site, most probably it is desmoid rather than tumor recurrence.
Adenomatous Polyposis Coli ; Colonic Neoplasms ; Fibroma ; Fibromatosis, Aggressive ; Follow-Up Studies ; Gastrectomy ; Humans ; Laparoscopy ; Medical Records ; Mesentery ; Mortality ; Neoplasm Metastasis ; Pancreatectomy ; Pathology ; Recurrence ; Retrospective Studies

PURPOSE: To identify prognostic factors influencing progression-free survival (PFS) of aggressive fibromatosis (AF) after postoperative radiotherapy (PORT) and assess correlations between immunohistochemistry (IHC) features of β-catenin/smooth muscle actin (SMA) and PFS. MATERIALS AND METHODS: Records of 37 patients with AF treated by PORT from 1984 to 2015 were retrospectively reviewed. Fifteen patients underwent wide excision for AF and 22 patients received debulking operation. The median total dose of PORT was 59.4 Gy. IHC staining results of β-catenin and SMA were available for 11 and 12 patients, respectively. RESULTS: The median follow-up duration was 105.9 months. Five-year PFS rate was 70.9%. Tumor size or margin status was not related to PFS in univariate analysis (p = 0.197 and p = 0.716, respectively). Multivariate analysis showed that increased interval from surgery to PORT (>5.7 weeks) was a marginal risk factor for PFS (p = 0.054). Administration of PORT at the initial diagnosis resulted in significantly improved PFS compared to deferring PORT after recurrence (p = 0.045). Patient with both risk factors of deferring PORT after recurrence and interval from surgery to PORT >5.7 weeks had significantly lower 5-year PFS than patients without risk factor (34.1% vs. 100.0%; p = 0.012). Nuclear β-catenin intensity tended to inversely correlate with 5-year PFS, although it did not reach statistical significance (62.5% at low vs. 100.0% at high; p = 0.260). SMA intensity was not related to PFS (p = 0.700). CONCLUSION: PORT should be performed immediately after surgery irrespective of margin status or tumor size especially in recurrent case. Nuclear β-catenin staining intensity of IHC might correlate with local recurrence.
Actins ; beta Catenin ; Diagnosis ; Disease-Free Survival ; Fibromatosis, Aggressive ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Multivariate Analysis ; Radiotherapy ; Radiotherapy, Adjuvant ; Recurrence ; Retrospective Studies ; Risk Factors

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

Fibromatosis is generally a benign tumor that arises from the musculoaponeurotic tissues of the body, rarely occurring in the head and neck region. This can be treated with a good prognosis, but sometimes recurs as a local invasion. Preoperative core needle biopsy and MR images are necessary to diagnose preoperatively and outline the tumor extent. The mainstay of treatment is complete surgical excision. Nonetheless, an excision is often difficult because of the complex anatomy or proximity of the tumor to vital structures in the head and neck region. We report a rare case of desmoid-type fibromatosis that occurred in the neck, closely attached to the transverse process of the cervical vertebra. The present article covers an extensive analysis of the case with a review of the related literature.
Biopsy, Large-Core Needle ; Cervical Vertebrae ; Female ; Fibroma* ; Fibromatosis, Aggressive ; Head ; Neck ; Prognosis ; Spine*

BACKGROUND: We retrospectively reviewed the outcomes of patients who had been treated with meloxicam for the extra-abdominal desmoid tumors and evaluated the correlation between clinical outcome and clinic pathological variables. METHODS: Twenty patients treated with meloxicam were followed up every 3 to 6 months. Meloxicam administration was planned at 15 mg/day orally for 6 months. RESULTS: Of the 20 patients evaluated, according to Response Evaluation Criteria in Solid Tumors criteria, there were five patients with partial response (25.0%), eight with stable disease (40.0%), and seven with tumor progression (35.0%). The cumulative probability of dropping out from our nonsurgical strategy using meloxicam was 35.0% at 1 year and 35.0% at 5 years. CONCLUSIONS: The present study suggests that conservative treatment would be a primary treatment option for this perplexing disease even though we were not able to determine that the use of a cyclooxygenase-2 inhibitor would have an additional influence on the natural course of a desmoid tumor.
Cyclooxygenase 2 ; Fibromatosis, Aggressive* ; Humans ; Response Evaluation Criteria in Solid Tumors ; Retrospective Studies

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

Gardner-associated fibroma (GAF) is a benign lesion of soft tissue which has recently been described and is exceedingly rare in children. GAF is associated with adenomatous polyposis coli gene mutation, familial adenomatous polyposis and desmoid. We report a case of patient with soft tissue tumor on her lower back which was turned out to be GAF. The patient was a 19-month-old female who visited out-patient clinic with palpable mass on her lower back and we performed surgical excision. The tumor was located at subcutaneous and we excised the tumor including surrounding soft tissue. She discharged without any complication on surgery day. The pathologic report showed dense collagenous tissue with spindle cell and adipose tissue, suggestive of GAF. We are planning to check gene study and to perform endoscopy and abdominal ultrasonography for at the age of 4.
Adenomatous Polyposis Coli ; Adipose Tissue ; Child ; Collagen ; Endoscopy ; Female* ; Fibroma* ; Fibromatosis, Aggressive ; Gardner Syndrome ; Humans ; Infant* ; Outpatients ; Ultrasonography

A mass excision surrounding the bronchial stump was performed to exclude malignancy in a 42-year-old man who had undergone a right lower lobectomy for lung cancer. The mass was identified as a cicatricial fibroma. Cicatricial fibromatosis, which is desmoid fibromatosis that arises in a surgical scar, is a well-known clinical condition. It consists of histologically benign neoplasms. Their occurrence after thoracic surgery is extremely rare. Biopsy or excision of suspicious lesions is very important for diagnosis. R0 resection remains the principal outcome for intra-thoracic desmoid fibromatosis. We report that a cicatricial fibromatosis in the subcarinal space was removed after suspicion of local recurrence at the bronchial stump follwing lobectomy for lung cancer.
Adult ; Biopsy ; Cicatrix ; Diagnosis* ; Fibroma* ; Fibromatosis, Aggressive ; Humans ; Lung Neoplasms* ; Lung* ; Recurrence* ; Thoracic Surgery