• Aggressive fibromatosis
• Case report
• Desmoid tumor
• Sigmoid mass
• Subepithelial tumor

• CTNNB1
• P53
• TP53
• beta-catenin
• deep aggressive fibromatosis
• desmoid fibromatosis
• desmoid tumor

• Humans
• Fibromatosis, Aggressive/diagnosis
• Fibromatosis, Aggressive/genetics
• Tumor Suppressor Protein p53/genetics
• Mutation
• Germ-Line Mutation
• Immunohistochemistry
• beta Catenin/genetics

Desmoid tumors are benign fibrous entities developed from connective tissue, and they result from muscle fascia or aponeurosis. Surgical excision is the main pillar of treatment.

A 29-year-old female patient presented with a left postero-lateral cervical swelling that had been evolving for 11 months. Cervical MRI showed a mass at the expense of the trapezium muscle measuring 41 × 68 × 81 mm. A biopsy of the mass was performed, concluding a desmoid tumor. The patient underwent a large resection of the tumor involving the left trapezius muscle. An immunohistochemistry staining was performed, demonstrating cytoplasmic labeling with anti AML antibody, and nuclear labeling of tumor cells with anti-beta-catenin antibody. The case was analyzed by a multidisciplinary committee, and it was decided to follow the patient for surveillance.

The localization of desmoid tumors in the trapezius muscle is extremely rare. The main risk of these benign lesions is infiltration of surrounding tissue leading to morbidity and mortality. Recurrence is a main feature of these tumors, even if complete excision has been performed. Unresectable desmoid tumors require medical and non-surgical treatment such as hormone therapy (tamoxifen), and chemotherapy with vinblastine and methotrexate.

Desmoid tumor of trapezius muscle is classified as benign without metastatic power, and the main risk is infiltration of surrounding tissue.

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Desmoid tumors are benign fibrous entities developed from connective tissue.

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.

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 β-catenin.

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.

• Aggressive fibromatosis
• Mesenteric fibromatosis
• Obstructive uropathy
• Surgery
• Tumoral hemorrhage

• rare diseases
• nose
• paranasal sinuses
• anterior skull base
• malformation

• Humans
• Nose
• Nose Neoplasms
• Papilloma, Inverted
• Paranasal Sinus Neoplasms/diagnosis
• Paranasal Sinus Neoplasms/epidemiology
• Paranasal Sinus Neoplasms/therapy
• Paranasal Sinuses/surgery
• Rare Diseases/epidemiology
• Rare Diseases/therapy
• Skull Base

• aggressive fibromatosis
• desmoid tumor
• regimen
• treatment

• Computer Tomography
• Desmoid
• Diagnostic radiology
• Mesentery
• Surgical pathology

• Ablation techniques
• Cryoablation
• Desmoid tumors
• Pain

Desmoplastic fibroblastoma (collagenous fibroma) is an uncommon benign soft-tissue tumor, rarely involving bone. It shares some overlapping features with other infiltrate tumors, such as desmoid-type fibromatosis, neurofibroma, and low-grade fibromyxoid sarcoma. The misdiagnosis may cause unnecessary surgical overtreatment, especially for those involving bone. In order to deepen the understanding of the diagnosis and differential diagnosis of desmoplastic fibroblastoma, we planned to analyze the clinical, radiological, and histopathological features and the outcome of desmoplastic fibroblastoma on the basis of case analysis and literature review.

Sixteen cases were retrieved from the surgical pathology records from May 2011 to April 2016 in the Department of Pathology in Beijing Jishuitan Hospital. Formalin-fixed, paraffin-embedded specimens of 16 cases of desmoplastic fibroblastoma were collected. Hematoxylin and eosin stain and immunohistochemistry were used to observe the histological features of desmoplastic fibroblastoma of soft tissue and bone. The images for diagnosis obtained from the ultrasonic examination, X-ray, magnetic resonance imaging, and computed tomography were used to observe the radiological features. Related literatures were retrieved from the PubMed and CNKI databases.

Sixteen cases of desmoplastic fibroblastoma of soft tissue were located in the hand (n = 7), foot (n = 4), upper arm (n = 1), shoulder (n = 1), forearm (n = 2), and one case occurred in the proximal femur. Age ranged from 32 to 82 years (median age: 58 years). There were six females and ten males. Histologically, the lesions of soft tissue appeared as well-circumscribed masses with abundant collagenous matrix and low vascularity. Tumor cells were stellate- or spindle-shaped and uniformly distributed within the extracellular matrix. In five cases, the desmoplastic fibroblastoma were found to have infiltrated into the skeletal muscle tissue. In one case of desmoplastic fibroblastoma of bone, radiographs revealed osteolytically well-defined lesion. Immunohistochemistry stain showed that vimentin and smooth muscle actin were positive in all cases of desmoplastic fibroblastoma.

Desmoplastic fibroblastoma (collagenous fibroma) has prominent clinical, histopathological, and radiological features. Before the differential diagnosis from other tumors is obtained by thorough analysis and comparison of the similar and different characteristics, the appropriate surgical management and accurate prognosis evaluation could not be delivered to the patient.

• Desmoid tumours
• Head and neck
• Surgery

Desmoid tumors (fibromatosis) are rare benign tumors, they arise from musculoaponeurotic structures throughout the body. They are locally infiltrative, resulting in a high rate of local recurrence following surgical resection. Due to the rarity of these tumors in the head and neck region, we report a case of a patient with a desmoid tumor in the upper neck that was diagnosed and treated in our institution, to increase the awareness of the Otolaryngology-Head and Neck surgeons, and report available treatment options of this condition.

• Adult
• Female
• Fibromatosis, Aggressive/diagnosis
• Fibromatosis, Aggressive/surgery
• Head and Neck Neoplasms/diagnosis
• Head and Neck Neoplasms/surgery
• Humans
• Neoplasm, Residual/radiotherapy
• Radiotherapy, Adjuvant

• Aggressive fibromatosis
• Chest wall defect
• Desmoid
• Reconstruction

• Aggressive
• Desmoid
• Extra-abdominal
• Fibromatosis
• Management
• Review

• desmoid-type fibromatosis
• desmoid tumor
• aggressive fibromatosis
• pancreatic head
• musculoaponeurotic tissues
• immunohistochemistry

• Adult
• Fibromatosis, Aggressive/pathology
• Fibromatosis, Aggressive/surgery
• Humans
• Male
• Pancreatic Neoplasms/pathology
• Pancreatic Neoplasms/surgery
• Pancreaticoduodenectomy
• Prognosis
• Tomography, X-Ray Computed

• Adolescent
• Adult
• Aged
• Cell Nucleus/chemistry
• Child
• Cyclooxygenase 2/analysis
• Cyclooxygenase 2 Inhibitors/therapeutic use
• Female
• Fibromatosis, Aggressive/drug therapy
• Fibromatosis, Aggressive/metabolism
• Humans
• Immunohistochemistry
• Ki-67 Antigen/analysis
• Male
• Meloxicam
• Middle Aged
• Thiazines/therapeutic use
• Thiazoles/therapeutic use
• beta Catenin/analysis

The local control of desmoid tumors constitutes a continuing treatment dilemma due to its high recurrence rates. The purpose of this systematic review was to critically examine the current treatment of these rare tumors and to specifically evaluate the local failure and response rates of surgery, radiation and systemic therapy. We comprehensively searched the literature for relevant studies across Cinahl, Embase, Medline and the Cochrane databases. Articles were categorized as surgery, radiation, surgery + radiation and systemic therapy (including cytotoxic and non cytotoxic). Methodological quality of included studies was assessed using the Newcastle-Ottawa Scale. Pooled odd ratios (OR) for comparative studies and weighted proportions with 95% confidence intervals (CI) are reported. Thirty-five articles were included in the final analysis. Weighted mean local failure rates were 22% [95% CI (16–28%)], 35% [95% CI (26–44%)] and 28% [95% CI (18–39%)] for radiation alone, surgery alone and surgery + radiation respectively. In the analysis of comparative studies, surgery and radiation in combination had lower local failure rates than radiation alone [OR 0.7 (0.4, 1.2)] and surgery alone [OR 0.7 (0.4, 1.0)]. Weighted mean stable disease rates were 91% [95% CI (85–96%)] and 52% [95% CI (38–65%)] for non cytotoxic and cytotoxic chemotherapy respectively.

The current evidence suggests that surgery alone has a consistently high rate of local recurrence in managing extra-abdominal desmoid tumors. Radiation therapy in combination with surgery improves local control rates. However, the limited data on systemic therapy for this rare tumor suggests the benefit of using both cytotoxic and non cytotoxic chemotherapy to achieve stable disease.

• chemotherapy
• desmoid tumors
• meta-analysis.
• radiation
• surgery

• Anastomosis, Surgical
• Colon, Transverse/pathology
• Colon, Transverse/surgery
• Female
• Fibroma/pathology
• Fibroma/surgery
• Humans
• Intestine, Small/blood supply
• Intestine, Small/surgery
• Mesenteric Artery, Superior/surgery
• Mesentery/blood supply
• Mesentery/surgery
• Microsurgery
• Peritoneal Neoplasms/pathology
• Peritoneal Neoplasms/surgery
• Veins/surgery
• Young Adult