3,331 results match your criteria Desmoid Tumor


Clinical, histological and therapeutic modern approach of Ledderhose disease.

Rom J Morphol Embryol 2018 ;59(3):691-697

Department of Anesthesiology and Intensive Care, Emergency Clinical Hospital of Bucharest, Romania;

Ledderhose disease or plantar fibromatosis is a rare hyperproliferative disorder of the plantar aponeurosis, clinically characterized by nodules situated especially on the medial border of the foot. It is histopathologically associated with Dupuytren's disease. This disease has some risk factors, like old age, alcohol or nicotine abuse, liver dysfunction, trauma or exposure to vibrations and autoimmune disorders, but the exact etiology is still unknown. Read More

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January 2018

Wnt targets genes are not differentially expressed in desmoid tumors bearing different activating β-catenin mutations.

Eur J Surg Oncol 2018 Oct 18. Epub 2018 Oct 18.

Department of Medical Oncology, Erasmus MC Cancer Institute, Erasmus Medical Center Rotterdam, the Netherlands. Electronic address:

Introduction: Sporadic desmoid-type fibromatosis (DTF) is a rare soft tissue tumor of mesenchymal origin. It is characterized by local invasive growth and unpredictable growth behavior. Three distinct mutations involving the CTNNB1 (β-catenin) gene have been identified in the vast majority of DTF tumors, which cause activation of the Wnt signaling pathway and impact prognosis. Read More

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October 2018

Successful treatment of a recurrent chest wall desmoid tumor with cyclooxygenase-2 inhibitors.

J Thorac Cardiovasc Surg 2018 Nov 2. Epub 2018 Nov 2.

Department of General Thoracic Surgery, Graduate School of Medicine, Chiba University, Chiba, Japan.

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November 2018

Risk of desmoid formation after laparoscopic open colectomy and ileorectal anastomosis for familial adenomatous polyposis.

BJS Open 2018 Dec 8;2(6):452-455. Epub 2018 Aug 8.

The Polyposis Registry, Level 5 St Mark's Hospital Northwick Park, Watford Road, Harrow HA1 3UJ UK.

Background: Laparoscopy is used increasingly in prophylactic surgery for patients with familial adenomatous polyposis (FAP) undergoing colectomy with ileorectal anastomosis (IRA). Little is known about the impact of laparoscopy on subsequent desmoid risk. This study documented the risk of desmoid in patients undergoing laparoscopic and open IRA. Read More

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December 2018

Coexistence of duodenum derived aggressive fibromatosis and paraduodenal hydatid cyst: A case report and review of literature.

World J Gastrointest Surg 2018 Nov;10(8):90-94

Department of Pathology, Inonu University Faculty of Medicine, Malatya 244280, Turkey.

Intra-abdominal aggressive fibromatosis is a locally aggressive tumor mostly originating from the mesentery or retroperitoneal space, infiltrating adjacent tissues, and very rarely metastasizing to distant organs. There are only two case reports in the English language literature where intra-abdominal aggressive fibromatosis originated from the intestinal wall. In this study, we aimed to report a case of aggressive fibromatosis originating from the muscularis propria layer of the duodenum and invading pancreas. Read More

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November 2018

Desmoid tumors of the head and neck: Two decades in a single tertiary care unit and review of the literature.

Ear Nose Throat J 2018 Oct-Nov;97(10-11):362-367

Department of Otorhinolaryngology-Head and Neck Surgery, Turku University Hospital, FIN-20521, Turku, Finland.

Desmoid tumors (DTs) of the head and neck have typically been classified as extra-abdominal, although the anatomic challenges of the head and neck warrant consideration of these DTs as a special entity. We present a review of DTs and describe our series of five patients with DTs of the head and neck treated within 2 decades. Altogether, 53 patients with DTs treated surgically at a tertiary care center over a 20-year period were retrospectively reviewed. Read More

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November 2018
1 Read

MRI diagnosis and follow-up of chest wall and breast desmoid tumours in patients with a history of oncologic breast surgery and silicone implants: A pictorial report.

J Med Imaging Radiat Oncol 2018 Nov 16. Epub 2018 Nov 16.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Introduction: Breast and chest wall desmoid tumours can cause debilitating symptoms and deformity. The mutilating effects of surgical treatment have prompted a shift to medical treatments and even to a wait-and-see approach. This study sought to highlight specific characteristics of breast and chest wall desmoid tumours on long-term follow-up by sequential MRI scans. Read More

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November 2018
2 Reads

Large intra-abdominal desmoid tumour posing diagnostic and therapeutic challenges, a case report.

J Surg Case Rep 2018 Nov 13;2018(11):rjy304. Epub 2018 Nov 13.

Department of Surgery, Our Lady Of Lourdes Hospital, Drogheda, Ireland.

We present the case of a 46-year-old gentleman originally from China who presented to the acute surgical assessment unit complaining of upper abdominal discomfort, dyspepsia and early satiety ongoing for the previous 6 months. On exam he had a palpable mass in the left upper quadrant. He underwent an esophagogastroduodenoscopy which was normal and later received a CT abdomen which identified a well-circumscribed soft tissue mass in the mesenteric fat and lying adjacent to the transverse colon with no obvious cleavage plane between them. Read More

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November 2018
13 Reads

Surgical excision versus observation as initial management of desmoid tumors: A population based study.

Eur J Surg Oncol 2018 Oct 25. Epub 2018 Oct 25.

Department of Surgery, University of Calgary, Calgary, AB, Canada.

Synopsis: Desmoid tumors can be safely managed with watchful waiting, including either observation alone or tamoxifen/NSAIDs. Surgery at first presentation can be associated with significant treatment burden.

Background: Immediate surgery was historically recommended for desmoid tumors. Read More

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October 2018
2 Reads

Recurrent desmoid tumor arising from latissimus dorsi flap: A case report.

Clin Imaging 2019 Jan - Feb;53:191-194. Epub 2018 Nov 2.

Department of Radiology, University of Kentucky, 800 Rose Street, HX-302, Lexington, KY 40536-0293, United States of America. Electronic address:

Fibromatosis or desmoid tumor in the breast is a very rare benign soft tissue tumor. We report a case of recurrent desmoid tumor arising from latissimus dorsi flap after lumpectomy for breast carcinoma. To our knowledge, this is the first case of desmoid tumor arising from the latissimus dorsi flap. Read More

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November 2018
6 Reads

Association of MRI T2 Signal Intensity With Desmoid Tumor Progression During Active Observation: A Retrospective Cohort Study.

Ann Surg 2018 Nov 9. Epub 2018 Nov 9.

Gastric and Mixed Tumor Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY.

Objective: The aim of this study was to identify predictors of desmoid progression during observation.

Summary Of Background Data: Untreated desmoids can grow, remain stable, or regress, but reliable predictors of behavior have not been identified.

Methods: Primary or recurrent desmoid patients were identified retrospectively from an institutional database. Read More

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November 2018
3 Reads

Gardner syndrome with maxillofacial manifestation: A case report.

Spec Care Dentist 2018 Nov 12. Epub 2018 Nov 12.

Postgraduate Program, Dental College, Pontifical Catholic University of Rio Grande do Sul, Porto Alegre, RS, Brazil.

Gardner syndrome is a hereditary disease in which patients develop gastrointestinal polyps, osteomas, desmoid tumors, epidermoid cysts, fibromas, lipomas, and retinal lesions. Dental abnormalities such as supernumerary or impacted teeth, odontomas and dentigerous cysts are also reported. The most serious concern in this syndrome is the extremely high risk of gastrointestinal polyps undergoing malignant transformation. Read More

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November 2018
2 Reads

HIFU for Bone Metastases and other Musculoskeletal Applications.

Semin Intervent Radiol 2018 Oct 5;35(4):261-267. Epub 2018 Nov 5.

Department of Radiological, Oncological and Pathological Sciences, Policlinico Umberto I, Sapienza University of Rome, Rome, Italy.

High-intensity focused ultrasound (HIFU) is a totally noninvasive procedure that has shown promising results in the management of numerous malignant and nonmalignant conditions. Under magnetic resonance or ultrasound guidance, high-intensity ultrasound waves are focused on a small, well-defined target region, inducing biologic tissue heating and coagulative necrosis, thus resulting in a precise and localized ablation. This treatment has shown both great safety and efficacy profiles, and may offer a multimodal approach to different diseases, providing pain palliation, potential local tumor control, and, in some cases, remineralization of trabecular bone. Read More

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October 2018
1 Read

Desmoid fibromatosis in the foot: A case report and literature review.

Medicine (Baltimore) 2018 Nov;97(44):e13109

Department of Burn and Plastic Surgery, West China Hospital, Sichuan University, Chengdu, China.

Rationale: Desmoid fibromatosis is a rare benign neoplasm arising from musculoaponeurotic structures with the potential to infiltrate local tissues and recur locally. While the commonly affected regions are the shoulder, chest wall and back, thigh, head and neck, localization in the foot is rare and only a few cases have been reported. Deep-seated lesions of foot tend to spread along the fascia and nerve or tendon sheaths. Read More

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November 2018
4 Reads

Immunohistochemical Correlates of Recurrent Genetic Alterations in Sarcomas.

Genes Chromosomes Cancer 2018 Nov 1. Epub 2018 Nov 1.

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, United States.

Sarcomas encompass a broad group of malignant mesenchymal neoplasms, whose accurate diagnosis and classification relies on the integration of clinical, histopathological and molecular features. Our understanding of the latter has increased dramatically in recent years with the widespread application of high-throughput sequencing. Concomitantly, the role of immunohistochemistry has continued to expand, as many genomic alterations have been exploited by the development of novel diagnostic markers that act as surrogates for their detection. Read More

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November 2018
2 Reads

Oral and Extraoral Intermediate Tumors: Are MMP-9 and Ki-67 Biomarkers Correlated to Their High Recurrence Rates?

Appl Immunohistochem Mol Morphol 2018 Oct 24. Epub 2018 Oct 24.

Department of Oncology and Diagnostic Sciences, School of Dentistry, University of Maryland, Baltimore, MA.

Intermediate tumors of the head and neck fall in the borderline category. They are clinically aggressive tumors with no malignant phenotyping. They are locally infiltrative and have high recurrence rate with less chances to metastasize. Read More

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October 2018
5 Reads

Extra-abdominal Desmoid Tumor Mimicking Cervical Spine Schwannoma.

Cureus 2018 Aug 14;10(8):e3145. Epub 2018 Aug 14.

Neurosurgery, University of Missouri, Columbia, USA.

Extra-abdominal desmoid tumors (DTs) are rare tumors of apparent fibroblastic origin with unpredictable clinical behavior. Though histologically benign and slow growing, DTs can be proliferative, aggressive tumors, invading the surrounding areas. DTs located extra-abdominally are most commonly found in the extremities or proximal structures like the shoulders, chest wall, and neck. Read More

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August 2018
8 Reads

Challenges in the treatment of fibrodysplasia ossificans progressiva.

Rheumatol Int 2018 Oct 20. Epub 2018 Oct 20.

Department of Physical Medicine and Rehabilitation, Marmara University School of Medicine, 34899 Pendik Ust Kaynarca, Istanbul, Turkey.

Fibrodysplasia ossificans progressiva (FOP), is a rare autosomal dominant connective tissue disease with a prevalence of 1 in 2 million. It is characterized by congenital foot deformities and multiple heterotopic ossifications in fibrous tissue. It usually starts with painful soft tissue swellings occurring with attacks at the ages of three or four. Read More

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October 2018
7 Reads

Aggressive fibromatosis in pediatric population-A case series.

Int J Surg Case Rep 2018 4;52:45-48. Epub 2018 Oct 4.

Department of Surgery, Aga Khan University Hospital, Stadium Road, P. O. Box 3500 Karachi 74800, Pakistan. Electronic address:

Introduction: Aggressive fibromatosis or Desmoid fibromatosis is a benign tumor which originated form mesenchymal tissues of the body. This tumor has strong potential of recurrence and infiltration but it does not metastasize to other organs of the body. This case series is focused to determine the treatment outcomes for pediatric patients of aggressive fibromatosis. Read More

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October 2018
1 Read

Staghorn sign: Extra-abdominal desmoid fibromatosis.

J Med Imaging Radiat Oncol 2018 Oct;62 Suppl 1:157-158

Department of Radiology, Concord Repatriation General Hospital, Concord, New South Wales, Australia.

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October 2018
2 Reads

Is immunohistochemical staining for β-catenin the definitive pathological diagnostic tool for desmoid-type fibromatosis?: a multi-institutional study.

Hum Pathol 2018 Oct 4. Epub 2018 Oct 4.

Department of Orthopedic Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan.

Immunohistochemical staining with anti-β-catenin antibody has been applied as a diagnostic tool for desmoid-type fibromatoses (DF). In recent years, specific gene mutation (CTNNB1) analysis has also been reported to be useful for diagnosis of DF, however the association between CTNNB1 mutation status and immunohistochemical staining pattern of β-catenin is rarely reported. The purpose of this study is to clarify the relationship of the staining pattern of β-catenin with the CTNNB1 mutation status and various clinical variables, and investigate the significance of immunohistochemical staining of β-catenin in cases diagnosed with DF. Read More

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October 2018
2 Reads

Unusual complication in patient with Gardner's syndrome: Coexistence of triple gastrointestinal perforation and lower gastrointestinal bleeding: A case report and review of literature.

World J Clin Cases 2018 Sep;6(10):393-397

Department of Surgery and Liver Transplant Institute, Inonu University Faculty of Medicine, Malatya 44280, Turkey.

Gardner's syndrome (GS) is a rare syndrome with autosomal dominant inheritance, which is characterized by multiple intestinal polyps, dental anomalies, desmoid tumors, and soft tissue tumors. All gastrointestinal symptoms seen in GS are associated with the underlying familial adenomatosis polyposis and abdominal desmoid tumors, with the most common symptoms being anemia, lower gastrointestinal bleeding, abdominal pain, diarrhea, obstruction, and mucous defecation. To our best knowledge, no case of GS that has presented with gastrointestinal perforation and bleeding has ever been reported in the English language medical literature. Read More

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September 2018
3 Reads

Dedifferentiated Liposarcoma With Myofibroblastic Differentiation.

Arch Pathol Lab Med 2018 Oct;142(10):1159-1163

From the Department of Pathology, University of Michigan, Ann Arbor.

Context.—: Liposarcoma is divided into myxoid, pleomorphic, well-differentiated, and dedifferentiated subtypes. Dedifferentiated liposarcoma displays the greatest histomorphologic diversity, including a subset with myofibroblastic differentiation that shares similarities with a spectrum of reactive, benign, and malignant soft tissue lesions. Read More

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October 2018
1 Read

Silicone breast implant associated fibromatosis.

J Surg Case Rep 2018 Sep 27;2018(9):rjy249. Epub 2018 Sep 27.

Division of Breast Surgical Oncology, Department of Surgery, University of Arkansas for Medical Sciences and the Winthrop P. Rockefeller Cancer Institute, Little Rock, AR, USA.

Extra-abdominal desmoid tumors, also known as aggressive or deep fibromatosis, are uncommon soft tissue tumors that rarely involve the breast. Although the exact etiology is unknown, the development of these tumors has been correlated with sites of previous trauma, surgery or in association with familial adenomatous polyposis. Clinically, breast fibromatosis is often mistaken for carcinoma but lacks metastatic potential. Read More

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September 2018
10 Reads

Evidence of hydroxyurea activity in children with pretreated desmoid-type fibromatosis: A new option in the armamentarium of systemic therapies.

Pediatr Blood Cancer 2019 Jan 30;66(1):e27472. Epub 2018 Sep 30.

Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.

Introduction: The treatment paradigm in desmoid-type fibromatosis (DF) has changed in recent years from a surgery-based strategy to a multidisciplinary approach that includes systemic therapies. Among various medical therapies, hydroxyurea has been considered of potential interest. This case series summarizes the experience gained at four centers using hydroxyurea in relapsing DF. Read More

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January 2019
2 Reads
2.560 Impact Factor

Hump-like giant desmoid tumor of the chest: a postresectional reconstruction challenge.

AME Case Rep 2017 17;1. Epub 2017 Nov 17.

Department of Thoracic Surgery, Policlinico Tor Vergata University, Rome, Italy.

Desmoid tumors (DT) are rare neoplasms with unknown etiology arising from musculoaponeurotic structures. Chest wall localization is uncommon and has been associated with high recurrence rate unless radical resection with negative margins is carried out. Postresectional reconstruction can be challenging in presence of giant lesions and might require adoption of complex reconstruction methods including use of well vascularized muscle flaps. Read More

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November 2017
7 Reads

Giant Intra-Abdominal Desmoid Tumor in a Young Male without History of Surgery, Trauma, or Familial Adenomatous Polyposis.

Case Rep Surg 2018 4;2018:9825670. Epub 2018 Sep 4.

Department of Surgery, Akashi Medical Center, Hyogo, Japan.

Desmoid tumors are rare, monoclonal myofibroblastic neoplasms that occur in the extremities, the trunk, and the abdominal cavity. We present a case that is significant for its rarity and for consideration of its treatment plan. A 17-year-old male reported swelling of his abdomen and abdominal pain. Read More

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September 2018
10 Reads

Can Ultrasound Alone Predict Papillary Thyroid Carcinoma with Desmoid-Type Fibromatosis? A Retrospective Analysis of 13 Cases, Focusing on the Stromal Area.

Ultrasound Int Open 2018 Apr 5;4(2):E39-E44. Epub 2018 Jul 5.

Kuma Hospital, Department of Surgery, Kobe, Japan.

Purpose: Papillary thyroid carcinoma with desmoid-type fibromatosis (PTC-DTF) is extremely rare. So far, only 4 cases describing the ultrasound findings of this variant have been reported. Here, we describe the ultrasound findings of 13 cases of PTC-DTF, focusing especially on the DTF area. Read More

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April 2018
3 Reads

Dramatic progression of a chest wall desmoid-like fibromatosis.

ANZ J Surg 2018 Sep 21. Epub 2018 Sep 21.

Unit of Thoracic Surgery, IRCCS Arcispedale Santa Maria Nuova, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy.

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September 2018

Intra-abdominal desmoid tumour presenting as an acute abdomen.

ANZ J Surg 2018 Sep 21. Epub 2018 Sep 21.

Department of General Surgery, Capital and Coast District Health Board, Wellington, New Zealand.

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September 2018
1 Read

Pancreatic desmoid tumor: A rare case with radiologic-pathologic correlation.

Radiol Case Rep 2018 Oct 18;13(5):1079-1083. Epub 2018 May 18.

Chief of the anatomic pathology department, Hotel-Dieu hospital, CHU de Quebec, Quebec, Canada.

A 39-year-old female patient was referred to our tertiary oncologic center for additional investigations relating to a suspect pancreatic tail lesion. An abdominal computed tomography scan and magnetic resonance imaging scan showed a solid lesion demonstrating progressive enhancement. Complete resection was obtained and the final diagnosis was that of a desmoid tumor of the pancreas, an exceptionally rare tumor demonstrating overlap with other solid and cystic lesions of the pancreas [1]. Read More

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October 2018
1 Read

Desmoid-type fibromatosis mimicking cystic retroperitoneal mass: case report and literature review.

BMC Med Imaging 2018 09 17;18(1):29. Epub 2018 Sep 17.

Department of Radiology, Korea University Guro Hospital, Korea University College of Medicine, 80 Guro-dong, Guro-gu, Seoul, 152-703, South Korea.

Background: Retroperitoneal desmoid-type fibromatosis (DF) is an uncommon mesenchymal neoplasm presenting as a firm mass with locally aggressive features. It usually manifests as a well-circumscribed or ill-defined, solid mass on cross-sectional imaging. Cystic changes of DF have been described in the literature in association with prolonged medical treatment or abscess formation. Read More

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September 2018
1 Read

Familial adenomatous polyposis: complex patient management.

Vnitr Lek 2018 ;64(6):635-641

Familial adenomatous polyposis (FAP) is a hereditary disease characterized by presence of numerous colorectal adenomas. It often exposes its carrier to absolute risk of colorectal cancer, but also to other extracolonic tumours (especially to duodenal cancer and desmoid). Screening and surveillance of FAP patients leads to reduction of colorectal cancer incidence and mortality. Read More

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January 2018
1 Read

Immunohistochemical and Molecular Analyses Focusing on Mesenchymal Cells in Papillary Thyroid Carcinoma with Desmoid-Type Fibromatosis.

Pathobiology 2018 17;85(5-6):300-303. Epub 2018 Sep 17.

Department of Surgery, Kuma Hospital, Kobe, Japan.

Objective: This study was designed to evaluate the prevalence of CTNNB1 (β-catenin) mutations in cases of papillary thyroid carcinoma with desmoid-type fibromatosis (PTC-DTF) expressing aberrant nuclear and cytoplasmic immunoreactivity for β-catenin.

Methods: Eight cases of PTC-DTF were available for this study. Immunohistochemistry for β-catenin and BRAFV600E was performed. Read More

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September 2018
6 Reads

Current Update on Desmoid Fibromatosis.

J Comput Assist Tomogr 2018 Sep 10. Epub 2018 Sep 10.

Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX.

Objective: The purpose of this article is to review the etiopathogenesis, molecular cytogenetics, histopathology, clinical features, and multimodality imaging features of desmoid fibromatosis. Recent advances in the management of desmoid fibromatosis will also be discussed.

Conclusions: Desmoid fibromatosis is a rare soft tissue neoplasm with a high incidence of local recurrence. Read More

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September 2018
8 Reads

EXTRA-ABDOMINAL DESMOID TUMOR: ANALYSIS OF 23 CONSECUTIVE CASES IN A SINGLE INSTITUTION.

Acta Ortop Bras 2018 ;26(4):244-247

Orthopedic Oncology Group, Instituto de Ortopedia e Traumatología, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

Objective: Extra-abdominal desmoid tumor (EDT) is a rare condition, caused by proliferation of fibroblasts. Despite being a benign tumor, it is locally aggressive and has unpredictable clinical behavior. The objective of this study is to present the clinical outcomes of patients with EDT treated surgically between 1995 and 2016. Read More

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January 2018

Mesenteric fibromatosis in a patient with a history of neuroblastoma: a case report.

J Surg Case Rep 2018 Sep 1;2018(9):rjy209. Epub 2018 Sep 1.

Department of Surgery, University of California, Irvine, CA, USA.

Mesenteric fibromatosis (MF) is a locally aggressive proliferative spindle cell lesion of the mesentery. A 34-year-old male presented with increasing abdominal pain and constipation. On workup, patient was found to have a large pelvic mass on CT A/P concerning for cancer. Read More

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September 2018
1 Read

An immunohistochemical approach to detect oncogenic CTNNB1 mutations in primary neoplastic tissues.

Lab Invest 2018 Sep 3. Epub 2018 Sep 3.

Departments of Internal Medicine, Human Genetics, and Pathology, University of Michigan Medical School, Ann Arbor, MI, 48109, USA.

The Wnt/β-catenin signaling pathway is dysregulated in different types of neoplasms including colorectal cancer (CRC). Aberrant activation of this signaling pathway is a key early event in the development of colorectal neoplasms, and is mainly caused by loss of function mutations in Adenomatous Polyposis Coli (APC), and less frequently by β-catenin stabilization mutations via missense or interstitial genomic deletions in CTNNB1. In this study, we have defined an immunohistochemical algorithm to dissect Wnt pathway alterations in formalin-fixed and paraffin-embedded neoplastic tissues. Read More

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September 2018
11 Reads

Successful Pregnancy and Delivery after Autologous Abdominal Wall Reconstruction using Anterolateral-Thigh and Iliotibial-Tract Flap.

Plast Reconstr Surg Glob Open 2018 Jul 13;6(7):e1819. Epub 2018 Jul 13.

Division of Plastic and Reconstructive Surgery, National Cancer Center Hospital, Tokyo, Japan.

The surgical management of abdominal wall tumor in women with childbearing potential is a thorny issue. A synthetic mesh is widely used for abdominal wall reconstruction but not necessarily applicable in case of women of childbearing potential because it has been reported to cause severe pain during pregnancy. Autologous reconstruction is usually considered a feasible option for such cases; however, there is no consensus on this approach and almost no evidence to support it. Read More

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Surgical management of truncal soft tissue sarcoma and other selected soft tissue neoplasms.

Authors:
Shane Y Morita

Chin Clin Oncol 2018 Aug;7(4):38

Oncology Service Line, The Queen's Medical Center, Honolulu, HI, USA; Department of Surgery, John A. Burns School of Medicine, Honolulu, HI, USA; Clinical and Translational Sciences Program, University of Hawaii Cancer Center, Honolulu, HI, USA.

Soft tissue sarcoma (STS) is a heterogeneous entity comprising only 1% of all adult cancers that has received considerable attention since it was initially described after the 1st century as "fleshy" by Claudius Galenus. Nick-named the forgotten cancer, more than 100 histologic subtypes have been identified making treatment paradigms extremely complex. A key principle in the management of truncal STS is a defined multi-disciplinary team consisting of several providers. Read More

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Growth rate and myofibroblast differentiation of desmoid fibroblast-like cells are modulated by TGF-β signaling.

Histochem Cell Biol 2018 Sep 1. Epub 2018 Sep 1.

Genetics Unit, "Mauro Baschirotto" Institute for Rare Diseases (B.I.R.D.), Via B. Bizio, 1, Costozza di Longare, 36023, Costozza di Longare, VI, Italy.

Desmoid-like fibromatosis (DF) is a rare myofibroblastic benign tumor, often associated with local and repeated injuries, spontaneous regression and stabilization of disease progression suggesting the involvement of altered Wnt/β-catenin signaling activation and/or aberrant response of the DF cells to external environmental stimuli. The aim of this study was to investigate the response of DF cells to microenvironmental factors such as inflammatory and growth factors or hormones. We observed that the inflammatory cytokine, transforming growth factor-β (TGF-β1) stimulated cell growth and myofibroblast differentiation of DF cells regardless of the presence of a β-catenin mutation. Read More

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September 2018
7 Reads
3.050 Impact Factor

The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children: an international prospective case series.

Lancet Child Adolesc Health 2017 Dec 12;1(4):284-292. Epub 2017 Sep 12.

Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.

Background: In 2005, the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) proposed a conservative treatment algorithm-consisting of an initial wait-and-see strategy, non-mutilating surgery, and minimal-morbidity chemotherapy (in the case of tumour progression)-for paediatric patients with desmoid-type fibromatosis. We aimed to investigate the outcomes of this algorithm.

Methods: In this case series, patients (<25 years) with desmoid-type fibromatosis from 57 centres in eight countries were prospectively registered through a web-based system. Read More

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December 2017
5 Reads

Desmoid tumours in children: when wait-and-see becomes wait-and-treat.

Lancet Child Adolesc Health 2017 Dec 12;1(4):253-254. Epub 2017 Sep 12.

Seattle Children's Hospital, University of Washington, Seattle, WA, USA; Fred Hutchinson Cancer Research Center, Seattle, WA, USA.

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December 2017

Recurrent desmoid-type fibromatosis associated with underlying neuromuscular choristoma.

J Neurosurg 2018 Aug 31:1-9. Epub 2018 Aug 31.

Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota.

OBJECTIVE Desmoid-type fibromatosis (DTF) presents a therapeutic dilemma. While lacking metastatic potential, it is a locally aggressive tumor with a strong propensity for occurrence near nerve(s) and recurrence following resection. In this study, the authors introduce the association of an occult neuromuscular choristoma (NMC) identified in patients with DTF. Read More

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August 2018
1 Read

Desmoid fibromatosis of the esophagogastric junction.

Rev Esp Enferm Dig 2018 Oct;110(10):677-678

Aparato Digestivo, Hospital General Universitario Gregorio Marañón, España.

Desmoid fibromatosis is a mesenchymal clonal proliferation, which lacks metastatic potential. Nevertheless, it has an infiltrative growth and thus implies a high morbidity1. Although the etiology remains unclear, mutations in the B-catenin or APC genes are involved. Read More

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October 2018
3 Reads

Laparoscopic Resection of Desmoid Type Fibromatosis of the Splenic Hilum Mimicking a Greater Curvature Gastric GIST.

J Coll Physicians Surg Pak 2018 Sep;28(9):714-716

Fourth Surgical Department, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Desmoid tumor, recently renamed as desmoid type fibromatosis, is an uncommon neoplasm. The mesentery is the usual origin of intra-abdominal desmoid-type fibromatosis and despite the fact there is no metastatic potential, it can infiltrate adjacent organs. There etiology remains unknown, preoperative diagnosis is difficult and differential diagnosis includes among other gastrointestinal stromal tumor (GIST). Read More

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September 2018
5 Reads

Desmoid fibromatosis through the patients' eyes: time to change the focus and organisation of care?

Support Care Cancer 2018 Aug 28. Epub 2018 Aug 28.

Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, SW3 6JJ, UK.

Purpose: Desmoid fibromatosis (DF) is a rare, unpredictable disease with no established, evidence-based treatments. Individual management is based on consensus algorithms. This study aimed to examine the specific health-related quality of life challenges faced by DF patients, current experiences and expectations of care. Read More

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August 2018
5 Reads

Risk Factors Associated With Pouch Adenomas in Patients With Familial Adenomatous Polyposis.

Dis Colon Rectum 2018 09;61(9):1096-1101

School of Medicine, University of Augsburg, Augsburg, Germany.

Background: Restorative proctocolectomy with ileal pouch-anal reconstruction is the standard prophylactic surgical procedure for patients with familial adenomatous polyposis. However, several groups have reported the development of adenomas and even carcinomas within the ileal pouch. The predisposing factor was the time interval after pouch surgery in some studies, but it was the severity of the initial colonic disease and duodenal adenomatosis in others. Read More

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September 2018
6 Reads

Multiple mesenteric desmoid tumors after gastrectomy for gastric cancer: A case report and literature review.

Int J Surg Case Rep 2018 31;50:50-55. Epub 2018 Jul 31.

Department of Surgery, Yachiyo Hospital, 2-2-7, Sumiyoshi-cho, Anjo-shi, Aichi, 446-8510, Japan. Electronic address:

Introduction: Many patients with desmoids have an antecedent trauma, particularly surgical intervention for familial adenomatous polyposis. However, cases of mesenteric desmoid after gastrectomy are extremely rare. We present a case of multiple mesenteric desmoids after total gastrectomy for gastric cancer. Read More

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July 2018
8 Reads