Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study

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.
Source: BMC Cancer - Category: Cancer & Oncology Authors: Tags: Research Source Type: research

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or Breast Screening Pathology European Working Group for Breast Screening Pathology Triple-negative breast cancers (TNBC), as a group of tumours, have a worse prognosis than stage-matched non-TNBC and lack the benefits of routinely available targeted therapy. However, TNBC is a heterogeneous group of neoplasms, which includes some special type carcinomas with a relatively indolent course. This review on behalf of the European Working Group for Breast Screening Pathology reviews the literature on the special histological types of BC that are reported to have a triple negative phenotype and indolent behaviour. These incl...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research
Gerasimos P. Sykiotis Desmoid-type fibromatosis (DTF) is a very rare variant of papillary thyroid carcinoma (PTC). It is essentially a dual tumor with a component of classical PTC with malignant epithelial proliferation (BRAF-mutated) and another component of mesenchymal proliferation (CTNNB1-mutated). We conducted a literature review on PTC-DTF. In total, 31 articles were identified, that together reported on 54 patients. The mean age was 47 years, with a 2.2:1 female predominance. No ultrasound features were found to be helpful in differentiating PTC-DTF from other PTC variants. Of the 43 cases that reported histo...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research
A retroperitoneal finding on a computerized tomography scan, in a symptomatic patient, can harbor the clinician to many differential diagnoses from infectious to malignancy. Desmoid fibromatosis (DF), a relatively innocuous mass that can spread locally, can be found in that anatomical region. Even for a rare tumor such as DF, our patient did not meet the usual benchmarks of this cancer, being an elderly female and having it appear as an abscess radiologically. Timely clinical response with radiotherapy and tamoxifen allowed our patient ’s DF to regress and resolved her symptoms.Case Rep Oncol 2021;14:1025 –1030
Source: Case Reports in Oncology - Category: Cancer & Oncology Source Type: research
Biochem Biophys Res Commun. 2021 Jun 24;568:37-42. doi: 10.1016/j.bbrc.2021.06.062. Online ahead of print.ABSTRACTCancer-associated fibroblasts (CAFs) are a major component of the tumor microenvironment and have been shown to promote cancer aggressiveness. In our previous study, analysis of expression profiles obtained from paired CAFs and normal fibroblasts from colorectal cancer (CRC) tissue revealed that gene sets related to the Wnt signaling pathway were highly enriched in colorectal CAFs. Furthermore, among the components of the β-catenin-independent Wnt pathway, Wnt5a was highly expressed in CAFs. Since Wnt5a is...
Source: Biochemical and Biophysical Research communications - Category: Biochemistry Authors: Source Type: research
This study (NCT04289077) is an international, multicentre, cross-sectional, observational cohort study. Patients ≥ 18 years with sporadic DTF from the Netherlands and the United Kingdom will be invited to complete a set of questionnaires specifically composed for this patient group. Questionnaires will be completed using PROFILES (Patient Reported Outcomes Following Initial treatment and Long-term Evaluation of Survivorship). Analyses will include testing the psychometric properties of the DTF-QoL and evaluating the prevalence of HRQoL issues using the DTF-QoL, EORTC QOL-C30 and EQ-5D-5L, among other questionnaires. Thi...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Study Protocol Source Type: research
In this series of patients with sporadic desmoid-type fibromatosis treated with low-dose chemotherapy and evaluated with MRI, T2 signal reduction was detected in more patients as compared to RECIST responses and volume reductions, and seemed to better reflect symptomatic improvement and worsening. RECIST may have underestimated treatment responses as compared to volumetric and T2 signal intensity changes. A high T2 signal intensity at baseline seemed to be related to a higher risk of RECIST progression after the end of chemotherapy.​ AbstractBackgroundWe aimed to investigate changes in volume and MRI T2-weighted intensit...
Source: Cancer Medicine - Category: Cancer & Oncology Authors: Tags: ORIGINAL RESEARCH Source Type: research
CONCLUSIONS: Our study showed that PIK3CA and TERT promoter mutations were common genetic features of FLSCCs. These findings contribute to our understanding of FLSCCs biology.PMID:33952589 | DOI:10.1136/jclinpath-2020-207071
Source: Clinical Genitourinary Cancer - Category: Cancer & Oncology Authors: Source Type: research
This report summarizes genotype-based recommendations for screening patients withPTCH1 andSUFU-related Gorlin syndrome, discussed during a workshop of the Host Genome Working Group of the European branch of the International Society of Pediatric Oncology (SIOPE HGWG) held in January 2020. In order to allow early detection of BCC, dermatologic examination should start at age 10 inPTCH1, and at age 20 inSUFU PV carriers. Odontogenic keratocyst screening, based on odontologic examination, should begin at age 2 with annual orthopantogram beginning around age 8 forPTCH1 PV carriers only. For medulloblastomas, repeated brain MRI...
Source: Familial Cancer - Category: Cancer & Oncology Source Type: research
Tumori. 2021 Apr 13:3008916211009316. doi: 10.1177/03008916211009316. Online ahead of print.ABSTRACTINTRODUCTION: Familial adenomatous polyposis (FAP) is a hereditary autosomal dominant disorder characterized by the development of multiple adenomas in the colon and rectum with a lifetime risk of 80%-100% to develop colorectal cancer if undetected or untreated. Gardner-associated fibroma (GAF) is a rare, benign soft tissue lesion with uncertain pathogenesis. GAF is generally associated with FAP in its clinical variant, called Gardner syndrome (GS).CASE DESCRIPTION: A 16-year-old boy with no comorbidities and no significant ...
Source: Tumori - Category: Cancer & Oncology Authors: Source Type: research
Clin Cancer Res. 2021 Apr 1;27(7):2120. doi: 10.1158/1078-0432.CCR-21-0041.NO ABSTRACTPMID:33795374 | DOI:10.1158/1078-0432.CCR-21-0041
Source: Clinical Cancer Research - Category: Cancer & Oncology Authors: Source Type: research
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