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: 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

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

PURPOSE: The objective of this study was to examine the clincopathologic characteristics and outcomes of familial adenomatous polyposis (FAP) patients with and without desmoid tumors (DTs), including the risk factors for progression of FAP-related DTs. MATERIALS AND METHODS: We reviewed the medical records and database of all patients with FAP who were treated between January 1993 and December 2011. RESULTS: Of 75 FAP patients, 18 (24%) were FAP with DTs. Seventeen of these had intra-abdominal DTs and one had intra- and extra-abdominal DTs. We divided the patients into two groups according to type of resection; the R0 or R1 resection group, referred to as the curative resection group (eight patients), and the R2 resection/palliative operation/medical treatment group, referred to as the palliative resection group (10 patients). Two patients in the curative resection group and two patients in the palliative group had progressed to tumor growth by the follow-up (p=0.800). In univariate analysis, DT diagnosis before or simultaneously with FAP diagnosis (DTs unrelated to surgical trauma) was a significant risk factor for tumor progression at final follow-up. CONCLUSION: A multidisciplinary approach to DT treatment is needed, including nonsteroidal antiinflammatory drugs, anti-estrogens, cytotoxic agents, and surgery. However, the role of surgery in resectable and complicated tumors may be limited. DT unrelated to surgical trauma has a relatively poor prognosis.
Adenomatous Polyposis Coli* ; Cytotoxins ; Diagnosis ; Fibromatosis, Aggressive* ; Follow-Up Studies ; Humans ; Medical Records ; Prognosis ; Risk Factors

Desmoid type fibromatosis is a benign fibroblastic tumor arising from the fascia or musculoaponeurosis. It may occur in various locations, but most commonly in the shoulder girdle and neck; to our knowledge, there has been no reported case originating from a facet joint of the spine. We report CT and MR imaging findings of a desmoid type fibromatosis, involving the facet joint of the L3-4 spine with bone involvement.
Adult ; Biopsy ; Fibromatosis, Aggressive/*diagnosis ; Humans ; *Lumbar Vertebrae ; Magnetic Resonance Imaging ; Male ; Spinal Neoplasms/*diagnosis ; *Zygapophyseal Joint

OBJECTIVE: To evaluate the clinical feature and therapy of rare aggressive fibromatosis in the head and neck. METHOD: Clinicopathologic data of 12 cases of aggressive fibromatosis in the head and neck were analysed. All cases underwent surgical excision, one case received radiotherapy in addition to surgery. RESULT: Six cases recurred after cutting off. One of them recurred twice no death was found following operation. CONCLUSION Aggressive fibromatosis in the head and neck is a rare benign, non-metastatic proliferation tumor of fibrous tissue with a capacity of infiltration to surrounding structures and a tendency of local recurrence after surgical resection. Complete surgical excision was considered to be the only effective method of cure. Chemotherapy and radiotherapy can be used in unsatisfactory surgical marginal recurrence.
Adult ; Female ; Fibromatosis, Aggressive ; diagnosis ; surgery ; Head and Neck Neoplasms ; diagnosis ; surgery ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Young Adult

OBJECTIVE: To evaluate the clinic manifestation, pathologic behavior, therapy and prognosis of rare aggressive fibromatosis in the head and neck. METHOD: Two cases of aggressive fibromatosis were analyzed and relevant literatures were reviewed. RESULT: Aggressive fibromatosis was characterized as infiltrative, locally aggressive and tended to recur after surgical resection. Pathology showed fibroblastic monoclonal proliferation. Fibromatosis was composed of well-differentiated fibroblasts and myofibroblasts, lacking cytological features of malignancy and scanty or absent mitotic activity. Complete surgical excision of aggressive fibromatosis was considered to be the only effective method of cure by most authorities. Chemotherapy and radiotherapy can be used together with surgery in recurrence or unsatisfactory surgical margin. In our study, one patient recurred after the first operation, and after another operation, the patient did not recur after 6 months follow up, and the other one did not recur after 6 months follow up. CONCLUSION The diagnosis of aggressive fibromatosis depended on pathological examination. Radical removal was an important way to reduce recurrence rate. Radiation therapy and chemotherapy can be used as adjuvant therapy in patients with recurrent or unresectable or inoperable disease.
Adolescent ; Female ; Fibromatosis, Aggressive ; diagnosis ; surgery ; Head and Neck Neoplasms ; diagnosis ; surgery ; Humans ; Middle Aged

Central odontogenic fibroma is a rare odontogenic neoplasm that originates from odontogenic ectomesenchyme. Here, a case of central odontogenic fibroma in a 17-year-old male is reported. Since the present case showed a multilocular radiolucency with partially ill-defined border between the right mandibular condyle and the distal root of the right mandibular third molar, differential diagnosis involved a wide range of pathosis from benign lesions like ameoloblastic fibroma and odontogenic myxoma to more aggressive lesions such as desmoplastic fibroma, juvenile aggressive fibromatosis, or fibrosarcoma.
Adolescent ; Diagnosis, Differential ; Fibroma ; Fibroma, Desmoplastic ; Fibromatosis, Aggressive ; Fibrosarcoma ; Humans ; Male ; Mandibular Condyle ; Molar, Third ; Myxoma ; Odontogenic Tumors

Fibromatosis can occur at various sites, but intracranial fibromatosis is exceptionally rare. Here, we report a case of intracranial fibromatosis arising in the suprasellar area of a 52-year-old woman who had undergone a surgery at that site. A computed tomography scan revealed a heavily calcified, highly enhancing, poorly demarcated mass in the left sellar area that extended into the left suprasellar, parasellar areas, and orbital apex and completely encased the left distal inferior cerebral artery. Histologic and immunohistochemical features were compatible with those of fibromatosis, although the cellularity was focally higher than usual. The etiology of extra-abdominal fibromatosis is unknown, but physical injuries such as trauma and irradiation have been reported to be associated with its occurrence. Although fibromatosis is rare in the intracranial area, it should be considered as a differential diagnosis when an intracranial mass occurs at a previously injured site.
Central Nervous System ; Cerebral Arteries ; Diagnosis, Differential ; Female ; Fibroma ; Fibromatosis, Aggressive ; Humans ; Middle Aged ; Orbit ; Sella Turcica