• Desmoids
• Familial adenomatous polyposis
• Intestinal transplantation

• Humans
• Adenomatous Polyposis Coli/surgery
• Adenomatous Polyposis Coli/mortality
• Adenomatous Polyposis Coli/pathology
• Intestines/transplantation
• Male
• Female
• Adult
• Middle Aged
• Desmoid Tumors/surgery

• Desmoid tumor
• fibromatosis
• giant
• sporadic

• Appendicitis
• Desmoid tumour
• Post appendicectomy

• Chemoemboliztion
• Desmoid fibromatosis (DFs)
• Doxorubicin-eluting beads embolization

• Ablation techniques
• Cryotherapy
• Desmoid-type fibromatosis
• Economic evaluation
• Surgery

• Boston Scientific Corporation

• aggressive-fibromatoses
• chest wall
• desmoid tumor
• recurrence

• Abdominal bloating
• Desmoid tumor
• Duodenal stricture
• Hematochezia

• 3D planning
• Desmoid Tumor
• Fiducial markers
• MRI-based navigation
• Percutaneous CRA

• Humans
• Cryosurgery
• Fibromatosis, Aggressive/diagnostic imaging
• Fibromatosis, Aggressive/surgery
• Fiducial Markers
• Magnetic Resonance Imaging
• Retrospective Studies
• Surgery, Computer-Assisted/methods
• Stereotaxic Techniques

• Desmoid tumor
• oncology
• plastic surgery
• reconstruction
• soft tissue tumor

• Aggressive fibromatosis
• Desmoid tumor
• Head and neck
• Treatment

• Humans
• Fibromatosis, Aggressive/surgery
• Fibromatosis, Aggressive/pathology
• Retrospective Studies
• Neoplasm Recurrence, Local/pathology
• Quality of Life
• Risk Factors

• Desmoid tumor
• Myoma
• Nullipara

• Adult
• Female
• Fibromatosis, Aggressive
• Humans
• Incisional Hernia
• Leiomyoma
• Myoma
• Nigeria
• Pregnancy

• Cryoablation
• Desmoid
• Ultrasound
• chemotherapy
• tumor

• Amyloid Precursor Protein Secretases/therapeutic use
• Anthracyclines/therapeutic use
• Anti-Inflammatory Agents/therapeutic use
• Fibromatosis, Aggressive/drug therapy
• Fibromatosis, Aggressive/pathology
• Humans
• Neoplasm Recurrence, Local/drug therapy
• Protein Kinase Inhibitors/therapeutic use

• Pediatric
• Sarcoma
• Soft tissue mass
• Tumor
• Vascular malformation

• Child
• Granuloma, Plasma Cell
• Humans
• Magnetic Resonance Imaging
• Neoplasms, Adipose Tissue
• Sarcoma
• Soft Tissue Neoplasms/diagnostic imaging

Tumors of the abdominal wall are rare, divided into benign and malignant lesions that are composed of primary tumors and by the parietal invasion of intra-abdominal tumors and metastatic parietal implants. In the case of metastases in the abdominal wall, the most frequent are metastases from neoplasms of colonic origin. The case described below is that of a 68-year-old patient with a history of stage IIA left lung adenocarcinoma who received partial response radiotherapy with an indication for chemotherapy, which she refused and lost follow-up.

• CTNNB1
• beta-catenin
• desmoid-type fibromatosis
• medical care guideline
• treatment modality

• Desmoid tumor
• Desmoid-type fibromatosis
• Outcome
• Surgery
• Treatment
• Wait-and-see

• Adult
• Aged
• Female
• Desmoid Tumors/mortality
• Desmoid Tumors/pathology
• Desmoid Tumors/surgery
• Humans
• Male
• Middle Aged
• Prospective Studies
• Survival Rate
• Watchful Waiting

Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate.

This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation.

This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF.

Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3–85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group.

Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.

• Percutaneous cryotherapy
• cryoprobe
• image-guided cryoablation
• metastases
• musculoskeletal tumors
• palliative care

• Desmoid fibromatosis
• Desmoid tumor
• Proton beam therapy
• Radiation

Desmoid tumours are benign neoplasms of myofibroblasts, often occurring after soft-tissue trauma. Rarely, desmoid tumours can occur following operative intervention, including spine surgery. In this case report, we describe the first reported case of desmoid tumour following scoliosis corrective surgery in an adolescent.

• β-catenin
• case report
• ctnnb1
• desmoid tumor
• lung cancer
• next-generation sequencing
• thoracotomy

• Abdominal Neoplasms/diagnosis
• Abdominal Neoplasms/diagnostic imaging
• Abdominal Neoplasms/genetics
• Adenocarcinoma/surgery
• Adenomatous Polyposis Coli/diagnosis
• Adenomatous Polyposis Coli/diagnostic imaging
• Adenomatous Polyposis Coli/genetics
• Adult
• Computed Tomography Angiography
• Diagnosis, Differential
• Fibromatosis, Aggressive/diagnosis
• Fibromatosis, Aggressive/diagnostic imaging
• Fibromatosis, Aggressive/genetics
• High-Throughput Nucleotide Sequencing
• Humans
• Lung Neoplasms/surgery
• Male
• Postoperative Complications/diagnosis
• Postoperative Complications/diagnostic imaging
• Postoperative Complications/genetics
• Prognosis
• Thoracotomy
• beta Catenin/genetics

• Aggressive fibromatosis
• Diagnostic accuracy
• Long-Tterm
• MRI
• Sarcoma center

• Adult
• Fibroma
• Fibromatosis, Aggressive
• Humans
• Magnetic Resonance Imaging
• Middle Aged
• Neoplasm Recurrence, Local/diagnostic imaging
• Sarcoma/diagnostic imaging
• Sensitivity and Specificity
• Young Adult

• Adult
• Fibromatosis, Aggressive/diagnosis
• Fibromatosis, Aggressive/surgery
• Foot
• Humans
• Neoplasm Recurrence, Local
• Radiotherapy, Adjuvant
• Retrospective Studies

• desmoid
• extra‐abdominal
• fibromatosis
• multifocal
• surgery

• Ablation techniques
• Cryoablation
• Desmoid tumors
• Pain

• Fibromatoses
• Recurrence
• Surgery
• Treatment options

• breast cancer
• breast desmoid tumor
• desmoid tumor

• CT, computed tomography
• MRI, magnetic resonance imaging

Desmoid-type fibromatosis is a benign mesenchymal neoplastic process. It exhibits an uncertain growth pattern and high recurrence rate. Previously radical surgical resection was the mainstay of treatment, but recently more surgeons are opting for conservative management with observation (“wait and see” policy). The authors intend to evaluate different therapeutic modalities and oncological outcomes for abdominal wall desmoid tumors.

We performed a retrospective study of patients who underwent surgical, hormonal or chemotherapy treatment for abdominal wall desmoid tumors between 1982 to 2014 at two institutions affiliated with the University of São Paulo, Brazil.

In the study period, 32 patients were included. Twenty-seven patients had surgery upfront. Of those, 89% were women with a median age of 33 years. Mean tumor size was 10 cm. Pathology confirmed free margins in 92% of resections. Tumor recurrence rate was 11%, with median relapse-free survival being 24 months. Multivariate analysis showed that positive final margins (p < 0.001) and positive frozen section (p = 0.001) were independent predictors of recurrence. For the 5 patients who underwent pharmacological therapy, median age was 33 years and median tumor diameter before treatment was 13 cm. Four patients exhibited partial response by Response Evaluation Criteria in Solid Tumors (RECIST). The single patient who did not respond to RECIST underwent radiotherapy.

Desmoid tumor treatment has been evolving over the past decade towards a more conservative approach. Pharmacological treatment may result in tumor size regression. When surgical excision is indicated, positive margins represent an important prognostic factor for local tumor recurrence.

• Abdominal wall desmoid
• Desmoid tumor
• Non aggressive management
• Watch and wait strategy

•

Relative good overall local control rate of all four different treatment groups of  >75%.

The treatment approach for aggressive fibromatosis is changing. Although surgery is the mainstay in common practice, recent literature is reporting a more conservative approach. We compared the local control rate for surgery, surgery with radiotherapy, radiotherapy alone and a wait and see policy in a systematic review.

A comprehensive search of the databases PubMed/Medline, Embase and Cochrane, of the medical literature published in 1999 till March 2017 was performed by two reviewers, including articles about extra abdominal aggressive fibromatosis without the genetical variants. A total of 671 studies were assessed for eligibility, and 37 studies were included for analysis, representing 2780 patients.

The local control rates for surgery alone, surgery and radiotherapy, radiotherapy alone and observation were 75%, 78%, 85% and 78%, respectively. For patients with recurrent disease observation had a better local control rate than surgery alone (p = 0.001). In the observation group, stabilization of the tumor was seen in median 14 (range 12–35) months. The time to local recurrence in the treatment group was median 17 (range, 11–52) months.

A watchful conservative first line approach with just observation and closely monitoring, by means of physical examination and MRI, appears to be justified in a subgroup of patients without clinical symptoms and no possible health hazards if the tumor would progress.

• Aggressive fibromatosis
• Different treatment strategies
• Systematic review

Desmoid-type fibromatosis (DF) is a aggressive (myo)fibroblastic neoplasm with an infiltrative growth and a tendency to local recurrence. Resection of the tumour and/or radiation were proposed as principal treatment. The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy.

From 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/− radiation therapy, NSAIDs or chemotherapy. In 4 cases a conservative approach was chosen. Thirty seven patients had primary, 17 recurrent disease. Endpoint was either local recurrence (LR), progression of residual disease or rare non-metastatic secondary lesions at the same extremity.

The mean age was 39,4 years. In 17 cases a R0, in 27 a R1 and in 6 cases a R2 resection was achieved. Four patients were treated conservatively. All together in 21 cases radiotherapy, in 5 NSAIDs, in 3 imatinib and in 2 cases each tamoxifen or chemotherapy had been applied. The median follow-up was 119 months. 5-year recurrence free survival after resection was 78%. 10 (20.4%) patients developed LR between 5 and 42 months after therapy. Recurrent disease was a negative factor on LR. Margins, radiotherapy, sex, or size of the tumour had no significant impact on LR. Patients younger than 40 years had a significant higher risk of LR.

Surgical margins are less important than keeping function. Radiotherapy might be an option in unresectable lesions, the role of adjuvant radiotherapy is controversially discussed.

• Desmoid
• Extra-abdominal
• Fibromatosis
• Prognosis
• Recurrence

• Abdominal wall reconstruction
• Case series
• Desmoid resection
• Desmoid type fibromatosis
• Surgical technique

A 49‐year‐old Pakistani male presented with “heaviness” in his chest. Chest radiograph and computed tomography (CT) confirmed a massive left‐sided pleural‐based opacity. Three years ago, he was investigated for a left‐sided lymphocytic, exudative pleural effusion following an episode of dengue fever. Tube thoracostomy removed 1.3 L of fluid. Pleural biopsy and bronchial washings were non‐contributory. He received empirical anti‐tuberculosis treatment and remained asymptomatic until this presentation. To investigate the new pleural mass, he underwent a video‐assisted thoracoscopic surgery, which revealed a 2.2 kg mass in the pleural cavity involving the anterior mediastinum and chest wall and adhered to the visceral pleura. Following conversion to an open thoracotomy, the mass was completely excised, which involved non‐anatomical lung resection. Histopathology and immunohistochemistry of the resected tumour were consistent for a desmoid tumour. He was followed up for 9 months with no evidence of tumour recurrence. Predominantly pleural‐based desmoid tumour is rare but should be included in the differential diagnosis of spindle cell tumours.

• Desmoid
• effusion
• mesothelial
• pleura

• aggressive fibromatosis
• cryoablation
• cryotherapy
• extraabdominal desmoid tumor

• autologous fibular graft
• desmoplastic fibroma
• reconstruction surgery
• repeated recurrences

• chest wall
• desmoid tumor
• extra-abdominal fibromatosis
• histology
• surgery

• Abdominal wall
• Desmoid tumor
• Oncological safety
• Tissue sparing
• Ultrasound

Desmoid tumors (DTs) are rare, benign soft-tissue tumors that have the potential for local invasion, but not for metastasis. The tumors are commonly characterized by a palpable mass, but present a variable and unpredictable clinical course. The current study presents the case of a giant mediastinal DT exhibiting lung involvement. A 50-year-old female was referred to the West China Hospital (Chengdu, Sichuan, China) due to a recurrent DT that was identified one year following radical surgery. The patient subsequently received radiation therapy. The DT arose from the mediastinum, unlike the usual presentation, and recurrence presented as extensive invasion into the lung tissue, almost being misdiagnosed as lung cancer with brain metastasis. Tumor recurrence was diagnosed through contrast-enhanced computed tomography and histological examination of the tumor. A routine follow-up revealed no further tumor progression at 9 months post-admission. Taking into account the unpredictable treatment complications, recurrent DTs can be managed simply and efficiently. A ‘wait-and-see’ policy could be a viable therapeutic option for this disease.

• computed tomography
• desmoid tumor
• mediastinum
• recurrent tumor

• desmoid tumor
• gastrointestinal stromal tumor

• Clinical practice guideline
• desmoid tumours
• follow-up
• treatment