3,112 results match your criteria Desmoid Tumor

Cutaneous desmoid-type fibromatosis: a rare case with molecular profiling.

J Cutan Pathol 2021 May 12. Epub 2021 May 12.

Department of Pathology, Stanford University, Stanford, California.

Fibromatoses encompass a broad group of histopathologically similar fibroblastic/myofibroblastic proliferations with divergent clinical manifestations and behavior. Deep (desmoid-type) fibromatoses are typically large, rapidly growing, and locally aggressive tumors that occur in the abdominal wall, mesentery, and extraabdominal soft tissue, principally the musculature of the trunk and extremities. Most sporadic cases of desmoid fibromatosis harbor inactivating mutations in CTNNB1, the gene encoding beta-catenin. Read More

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Desmoid-type fibromatosis: toward a holistic management.

Curr Opin Oncol 2021 May 10. Epub 2021 May 10.

Medical Oncology Department Clinical Research Department, Centre Oscar Lambret Lille University Medical School, Lille, France Medical Oncology Department, Sarcoma Unit, Mannheim University Medical Center, University of Heidelberg, Mannheim, Germany Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam Department of Medical Oncology, Erasmus MC Cancer Institute, Erasmus University Medical Center, Rotterdam, The Netherlands.

Purpose Of Review: Desmoid-type fibromatosis, a rare locally aggressive fibroblastic proliferation, is a treatment challenge. This review aimed to explore recent data about the management of desmoid-type fibromatosis.

Recent Findings: New data underline the role of kinases and [Latin Small Letter Gamma]-secretase in stimulating cell proliferation and invasiveness in desmoid-type fibromatosis. Read More

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Corrigendum: Development, Validation, and Visualization of A Web-Based Nomogram for Predicting the Recurrence-Free Survival Rate of Patients With Desmoid Tumors.

Front Oncol 2021 20;11:688620. Epub 2021 Apr 20.

Department of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, China.

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Erythema multiforme after treatment with sorafenib.

Proc (Bayl Univ Med Cent) 2021 Mar 8;34(3):380-381. Epub 2021 Mar 8.

Department of Dermatology, Baylor Scott & White Medical Center - Temple, Temple, Texas.

Sorafenib, an oral chemotherapeutic agent used in the treatment of solid tumors, is associated with a variety of adverse cutaneous drug reactions in up to 90% of patients. Infrequently, delayed-type hypersensitivity reactions such as erythema multiforme occur. This case describes a child treated with sorafenib for a retrosternal desmoid tumor who developed widespread erythema multiforme across his extremities, trunk, face, and mucosal membranes. Read More

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Infantile Rhabdomyosarcomas With VGLL2 Rearrangement Are Not Always an Indolent Disease: A Study of 4 Aggressive Cases With Clinical, Pathologic, Molecular, and Radiologic Findings.

Am J Surg Pathol 2021 Jun;45(6):854-867

Genetics Unit, Department of Tumor Biology.

VGLL2-rearranged rhabdomyosarcomas (RMS) are rare low-grade tumors with only favorable outcomes reported to date. We describe 4 patients with VGLL2-rearranged RMS confirmed by molecular studies, who experienced local progression and distant metastases, including 2 with fatal outcomes. Tumors were diagnosed at birth (n=3) or at 12 months of age (n=1), and were all localized at initial diagnosis, but unresectable and therefore managed with chemotherapy and surveillance. Read More

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Percutaneous Microwave Ablation of Desmoid Fibromatosis.

Korean J Radiol 2021 Apr 23. Epub 2021 Apr 23.

Department of Diagnostic Imaging, Virgen de las Nieves University Hospital, Granada, Spain.

Objective: To demonstrate the feasibility of percutaneous microwave ablation in desmoid fibromatosis with respect to tumor volume control and improvement in the quality of life.

Materials And Methods: Twelve microwave ablations were performed in 9 patients with a histological diagnosis of desmoid fibromatosis between January 2010 and January 2019. The study population included 6 female and 3 male, with an age range of 21-76 years (mean = 46. Read More

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Clinicopathological features of 70 desmoid-type fibromatoses confirmed by β-catenin immunohistochemical staining and CTNNB1 mutation analysis.

PLoS One 2021 29;16(4):e0250619. Epub 2021 Apr 29.

Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.

Desmoid-type fibromatosis (DF) is a locally aggressive neoplasm characterized by mutations in the CTNNB1 gene, which encodes the β-catenin protein. We reviewed 85 cases of DF and performed Sanger sequencing for detecting mutations in CTNNB1 and immunostaining for detecting β-catenin localization. We included 70 DF samples, of which 56 cases demonstrated nuclear β-catenin localization and 43 cases harboured CTNNB1 mutations. Read More

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Undervaluation of Radiotherapy for Gross Desmoid Tumors: The Need for Absolute Volume Assessment.

In Vivo 2021 May-Jun;35(3):1777-1784

Department of Radiation Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.

Background/aim: To compare absolute volume (AV) assessment according to Response Evaluation Criteria in Solid Tumors (RECIST) for the response evaluation of desmoid tumors (DTs) treated with radiotherapy.

Patients And Methods: Eighteen patients with DTs ≥3 cm in size were included.

Results: The median follow-up duration was 78. Read More

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January 2021

DNA methylation profiling discriminates between malignant pleural mesothelioma and neoplastic or reactive histological mimics.

J Mol Diagn 2021 Apr 19. Epub 2021 Apr 19.

Department of Neuropathology, Institute of Pathology, University Hospital of Heidelberg, Im Neuenheimer Feld 224, 69120 Heidelberg, Germany; Clinical Cooperation Unit Neuropathology, German Consortium for Translational Cancer Research (DKTK), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 224, 69120 Heidelberg, Germany.

Diagnosis of malignant pleural mesothelioma (MPM) is a challenging task because of its potential overlap with other neoplasms or even with reactive conditions. Recently, DNA methylation analysis proved to be an effective tool for tumor diagnosis. In the present study, this approach was tested for MPM diagnosis. Read More

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Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study.

BMC Cancer 2021 Apr 20;21(1):437. Epub 2021 Apr 20.

Orthopaedic Oncology Department, Azienda Ospedaliero Universitaria Careggi, Florence, Italy.

Background: 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.

Questions/purpose: 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. Read More

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Desmoid Type Fibromatosis of the Breast Masquerading as Breast Carcinoma: Value of Dynamic Magnetic Resonance Imaging and Its Correlation.

Eur J Breast Health 2021 Apr 31;17(2):197-199. Epub 2021 Mar 31.

Department of Biomedical Imaging, University of Malaya, Kuala Lumpur, Malaysia.

Desmoid type fibromatosis of the breast is a rare stromal tumor that accounts for <0.2% of all breast tumors. Bilateral and multicentric lesions are extremely rare, with only less than ten cases reported in the literature. Read More

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Large Intra-Abdominal Desmoid Tumor in a Deployed Soldier Initially Presented With Chronic Diarrhea.

Mil Med 2021 Apr 16. Epub 2021 Apr 16.

Carl R. Darnall Army Medical Center, Fort Hood, TX 76544, USA.

Diarrhea is a common condition seen among soldiers in both garrison and deployed environments. Although the vast majority of soldiers with diarrhea will recover uneventfully with supportive care, clinicians should also maintain suspicion for less common causes and perform a thorough physical exam. We report the case of a young, healthy soldier with chronic diarrhea and progressively worsening abdominal distention that began during his deployment to Honduras who was subsequently found to have a large intra-abdominal desmoid tumor. Read More

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MRI T1 Contrast-Enhanced Signal Intensity Is a Prognostic Indicator of Imatinib Therapy in Desmoid-Type Fibromatosis.

Front Oncol 2021 15;11:615986. Epub 2021 Mar 15.

Department Radiology, Peking University Cancer Hospital, Beijing, China.

Objective: To investigate the efficiency of pre-therapy magnetic resonance imaging (MRI) features in predicting the prognosis of desmoid-type fibromatosis patients treated with imatinib.

Materials And Methods: A total of 38 desmoid-type fibromatosis patients treated with imatinib were collected in this retrospective study. The high signal intensity on pre-therapy MRI was evaluated on axial T2 and T1 contrast-enhanced sequences with fat suppression. Read More

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Fibromatosis with aggressive demeanor: Benign impersonator of malignancy.

World J Nucl Med 2021 Jan-Mar;20(1):121-124. Epub 2020 Dec 12.

Department of Nuclear Medicine and PET CT, Apollo Hospitals, Chennai, Tamil Nadu, India.

Fibromatosis or desmoid fibromatosis is a rare benign neoplasm and develops commonly in the abdominal wall, abdominal cavity, or extra-abdominal sites. The mainstay of treatment is surgery. Chemotherapy and radiotherapy are preferred in cases of inoperable/relapse or a multifocal disease. Read More

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December 2020

An extremely rare case of desmoplastic fibroblastoma exhibiting rapid growth in the chest wall: a case report.

Surg Case Rep 2021 Apr 7;7(1):86. Epub 2021 Apr 7.

Department of Surgery, Yamagata Prefecture Shinjo Hospital, 12-55 Wakaba Town, Shinjo, Yamagata, 996-0025, Japan.

Background: Desmoplastic fibroblastoma is an uncommon, benign, fibrous tumor exhibiting infiltrative growth. Most of these tumors are small, slow-growing, and develop as subcutaneous lesions in the extremities. Cases of desmoplastic fibroblastoma in the chest wall are quite rare, and the preoperative diagnosis of such cases remains challenging as these tumors can mimic the characteristics of desmoid-type fibromatosis, which often occurs in the chest wall. Read More

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Solid Pseudopapillary Neoplasm of the Pancreas and Abdominal Desmoid Tumor in a Patient Carrying Two Different Germline Mutations: New Horizons from Tumor Molecular Profiling.

Genes (Basel) 2021 Mar 26;12(4). Epub 2021 Mar 26.

Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy.

This case report describes the history of a 41 year-old woman with a solid pseudopapillary neoplasm (SPN) of the pancreas and a metachronous abdominal desmoid tumor (DT) that occurred two years after the SPN surgical resection. At next-generation sequencing of 174 cancer-related genes, both neoplasms harbored a somatic mutation which was different in each tumor. Moreover, two pathogenic mutations were found in both tumors, confirmed as germline by the sequencing of normal tissue. Read More

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[A Case of Intra-Abdominal Desmoid Tumor with Acute Abdominal Pain].

Gan To Kagaku Ryoho 2021 Mar;48(3):434-436

Dept. of Surgery, Fukuoka Shin-Mizumaki Hospital.

A 70-year-old man was admitted to our hospital with acute abdominal pain. Abdominal CT showed a 6-cm-sized tumor near the third portion of the duodenum, and a duodenal GIST was suspected. Although the abdominal pain was intense and the tumor was suspected to be ruptured, the vital signs were stable; therefore, we initially planned to perform an elective surgery. Read More

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[Ⅰ. Evidence-Based Drug Treatment for Desmoids-Anti-Cancer Drugs, Molecular-Targeted Drugs].

Gan To Kagaku Ryoho 2021 Mar;48(3):342-345

Dept. of Rehabilitation, Nagoya University Hospital.

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A comparison of the usefulness of nuclear beta-catenin in the diagnosis of desmoid-type fibromatosis among commonly used anti-beta-catenin antibodies.

Pathol Int 2021 Mar 31. Epub 2021 Mar 31.

Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan.

Desmoid-type fibromatosis (DF) is a locally aggressive but non-metastatic (myo)fibroblastic neoplasm. A hallmark of the tumor is nuclear positivity for beta-catenin in immunohistochemistry due mostly to CTNNB1 mutations. However, a recent study has reported that even beta-catenin 'nuclear-negative' DFs can harbor CTNNB1 mutations and that the positive ratio of nuclear beta-catenin in DF is different among antibodies. Read More

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Accumulated genetic mutations leading to accelerated initiation and progression of colorectal cancer in a patient with Gardner syndrome: A case report.

Medicine (Baltimore) 2021 Apr;100(13):e25247

Department of Oncology, Longhua Hospital Affiliated to Shanghai University of Traditional Chinese Medicine (TCM), Shanghai, China.

Rationale: Gardner syndrome is a rare autosomal dominant disorder with a high degree of penetrance, which is characterized by intestinal polyposis, osteomas, and dental abnormalities. Majority of patients with Gardner syndrome will develop colorectal cancer by the age of 40 to 50 years. Mutations in the adenomatous polyposis coli gene are supposed to be responsible for the initiation of Gardner syndrome. Read More

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High cumulative risk of colorectal cancers and desmoid tumours and fibromatosis in South Asian APC mutation carriers.

J Med Genet 2021 Mar 25. Epub 2021 Mar 25.

Cancer Genetics Unit, Advanced Centre for Treatment Research and Education in Cancer, Navi Mumbai, Maharashtra, India

Management of familial adenomatous polyposis (FAP) is guided by the cumulative risk of colorectal cancer (CRC) and aggressive fibromatosis/desmoid (AF/D). The first non-Caucasian FAP cohort with cumulative risk estimates for CRC and AF/D shows distinct differences with the Caucasian and other Asian cohorts. The strong correlation between the adenomatous polyposis coli (APC) mutation location with the FAP phenotype and the geoethnic differences in APC mutation spectrum, genetic constitution, lifestyle and sporadic CRC rates, mandates the use of population-specific cumulative risk estimates for CRC and desmoid for counselling and risk management. Read More

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Pancreas sparing duodenectomy in the treatment of primary duodenal neoplasms and other situations with duodenal involvement.

Hepatobiliary Pancreat Dis Int 2021 Mar 9. Epub 2021 Mar 9.

Department of Hepatobiliary and Pancreatic Surgery, Hospital Universitari de Bellvitge, Research Group of Hepato-biliary and Pancreatic Diseases, Institut d'Investigació Biomèdica de Bellvitge - IDIBELL, Barcelona University, L'Hospitalet de Llobregat, Barcelona 08907, Spain.

Background: There are no clearly defined indications for pancreas-preserving duodenectomy. The present study aimed to analyze postoperative morbidity and the outcomes of patients undergoing pancreas-preserving duodenectomy.

Methods: Patients undergoing pancreas-preserving duodenectomy from April 2008 to May 2020 were included. Read More

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Aggressive fibromatosis of the right colon mimicking a gastrointestinal stromal tumour: a case report.

J Int Med Res 2021 Mar;49(3):300060521994927

Centre for Radiology and MRI, University Clinical Centre of Serbia, Belgrade, Serbia.

Aggressive fibromatosis is a rare type of intra-abdominal desmoid tumour that usually involves the small bowel mesentery. It is a locally-invasive lesion, with a high rate of recurrence, but without metastatic potential. Aggressive fibromatosis is seen more often in young female patients. Read More

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An intra-abdominal desmoid tumor.

Acta Radiol Open 2021 Feb 22;10(2):2058460121992455. Epub 2021 Feb 22.

Department of Medical Imaging, Faculty of Medicine, University of Debrecen, Hungary.

Desmoid tumor is a very rare neoplasm which develops from fibroblasts. These tumors do not have the ability to metastasize, but they can cause significant morbidity and mortality by local invasion and they are prone to local recurrence. We present a case of an aggressive fibromatosis in a 28-year-old male patient with no previous medical history. Read More

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February 2021

Pseudoaneurysm within a desmoid tumor in an extremity: report of 2 cases.

Skeletal Radiol 2021 Mar 16. Epub 2021 Mar 16.

Radiology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY, 10065, USA.

Desmoid tumor is considered a benign neoplasm, yet substantial morbidity can result from local invasion of structures adjacent to the tumor or from complications related to its treatment. We report two patients with extremity desmoid tumor who were each found at MRI to have an unsuspected pseudoaneurysm within their tumor after prior treatments (surgery and systemic therapy in one, surgery alone in the other). Such a pseudoaneurysm probably results from weakening of an arterial wall by adjacent desmoid tumor, as well as from local trauma. Read More

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Desmoid Fibromatosis: Management in an Era of Increasing Options.

Curr Oncol Rep 2021 Mar 14;23(4):41. Epub 2021 Mar 14.

Radiation Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 0097, Houston, TX, 77030, USA.

Purpose Of Review: Desmoid fibromatosis (DF) is a locally aggressive clonal neoplasm with locally aggressive behavior and no metastatic potential. Historical treatment of DF has consisted primarily of up-front surgery when feasible. In recent years, recognition that DF can spontaneously stabilize or involute has allowed for many patients to be managed with watchful waiting rather than intervention. Read More

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Development, Validation, and Visualization of A Web-Based Nomogram for Predicting the Recurrence-Free Survival Rate of Patients With Desmoid Tumors.

Front Oncol 2021 25;11:634648. Epub 2021 Feb 25.

Department of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, China.

Background: Surgery is an important treatment option for desmoid tumor (DT) patients, but how to decrease and predict the high recurrence rate remains a major challenge.

Methods: Desmoid tumor patients diagnosed and treated at Tianjin Cancer Institute & Hospital were included, and a web-based nomogram was constructed by screening the recurrence-related risk factors using Cox regression analysis. External validation was conducted with data from the Fudan University Shanghai Cancer Center. Read More

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February 2021

Percutaneous cryoablation for advanced and refractory extra-abdominal desmoid tumors.

Int J Clin Oncol 2021 Mar 11. Epub 2021 Mar 11.

Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France.

Purpose: To assess efficacy and safety of percutaneous cryoablation (CA) for advanced and refractory extra-abdominal desmoid tumors.

Materials And Methods: This retrospective study reviewed 30 consecutive patients with symptomatic desmoid tumors evolving after "wait and watch" periods, and despite medical treatment, treated by CA between 2007 and 2019. Progression free survival (PFS), objective response rate, pain reduction (decreased of visual analogic scale pain (VAS)[Formula: see text] 3 or disappearance of pain), total volume lesion (TVL) and complications were documented. Read More

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Desmoid Type Fibromatosis of the Breast: Ten-Year Institutional Results of Imaging, Histopathology, and Surgery.

Breast Care (Basel) 2021 Feb 28;16(1):77-84. Epub 2020 May 28.

MVZ Pathologie Hamburg-West, Hamburg, Germany.

Background: Desmoid type fibromatoses has proven to be a diagnostic and therapeutic challenge, as they often appear primarily as a carcinoma of the breast with a high recurrence risk.

Patients: A digital archive search was performed for the period from 2009 to the end of 2018. Inclusion criteria consisted of histological examination of at least the surgical specimen in the reference pathology department and at least a second opinion diagnosis in the reference radiology department. Read More

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February 2021

Treatment Concepts and Challenges in Nonrhabdomyosarcoma Soft Tissue Sarcomas.

Surg Oncol Clin N Am 2021 Apr 27;30(2):355-371. Epub 2021 Jan 27.

Department of Surgery, Brody School of Medicine, East Carolina University, 600 Moye Boulevard, Greenville, NC 27834, USA.

Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) encompass a heterogeneous group of mesenchymal tumors with more than 50 histologic variants. The incidence of NRSTS is greater than rhabdomyosarcoma; however, each histologic type is rare. The treatment schema for all NRSTSs is largely surgical. Read More

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