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https://www.readbyqxmd.com/read/27905688/liposomal-doxorubicin-effective-treatment-for-pediatric-desmoid-fibromatosis
#1
Prasanna Ananth, Annette Werger, Stephan Voss, Carlos Rodriguez-Galindo, Katherine A Janeway
Efficacy of liposomal doxorubicin (LD) in treating desmoid fibromatosis (DF) in children has not been well evaluated. This retrospective case series examines five children with progressive DF, treated with LD. We report progression-free intervals (PFIs) and radiographic as well as clinical responses for each medication received. LD was well tolerated, with an average 4.5% reduction in tumor size and median PFI of 29 months. Treatment with LD conferred the longest PFI of all medical therapies pursued. Thus, LD is an important treatment option for DF in pediatrics...
December 1, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27892778/first-report-of-palmar-fibromatosis-and-camptodactyly-in-the-2nd-century-ad
#2
Marios Papadakis, Andreas Manios, Constantinos Trompoukis
No abstract text is available yet for this article.
November 28, 2016: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/27891213/uk-guidelines-for-the-management-of-soft-tissue-sarcomas
#3
REVIEW
Adam Dangoor, Beatrice Seddon, Craig Gerrand, Robert Grimer, Jeremy Whelan, Ian Judson
Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues, and can occur almost anywhere in the body. Their rarity, and the heterogeneity of subtype and location means that developing evidence-based guidelines is complicated by the limitations of the data available. However, this makes it more important that STS are managed by teams, expert in such cases, to ensure consistent and optimal treatment, as well as recruitment to clinical trials, and the ongoing accumulation of further data and knowledge...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27875709/iodine-125-interstitial-brachytherapy-for-pediatric-desmoid-type-fibromatosis-of-the-head-and-neck-a-case-report
#4
Wen-Jie Wu, Hua-Qiu Guo, Guang-Yan Yu, Jian-Guo Zhang
Desmoid-type fibromatosis (DF) is a locally aggressive benign soft tissue tumor. It is rarely observed in the head and neck region and is particularly uncommon in the parotid gland. This report describes the case of a 32-month-old girl with DF of the head and neck. The tumor was resected with gross residual tumors. Recurrence occurred 3 months later and then the patient was treated with iodine-125 interstitial brachytherapy. The tumor was completely absent 6 months after brachytherapy. No recurrence was found 60 months after brachytherapy during follow-up...
October 29, 2016: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/27858546/massively-distended-abdomen-caused-by-fibromatosis-previously-misdiagnosed-as-myxofibrosarcoma
#5
Michaela Kuhlen, Prasad T Oommen, Arndt Borkhardt
No abstract text is available yet for this article.
November 2016: Journal of Oncology Practice
https://www.readbyqxmd.com/read/27855230/a-case-of-thyroid-fibromatosis-a-rare-lesion-of-this-gland
#6
Joana Simões-Pereira, Rafael Adame Cabrera, Valeriano Leite
: Thyroid fibromatosis is a very rare lesion; to our knowledge, there are only four cases reported in the medical literature. Herein, we report the clinical case of a woman with thyroid fibromatosis with a long follow-up (11 years). A 63-year-old female patient, with an increasing multinodular goitre without compressive symptoms, was admitted to total thyroidectomy. The histology revealed a spindle-cell proliferation with fibroblastic characteristics with no atypia and thin capillary vessels...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27817254/cytohistological-and-immunohistochemical-characteristics-of-spindle-shaped-mesenchymal-neoplasms-occurring-in-the-gastrointestinal-tract
#7
Antonio Ieni, Valeria Barresi, Luca Reggiani Bonetti, Giovanni Branca, Rosario Alberto Caruso, Giovanni Tuccari
The purpose of the present review is to analyze the cytohistological and immunohistochemical characteristics of spindle-shaped mesenchymal gastrointestinal neoplams (MGNs), a group of unusual neoplastic conditions with different biological behavior. These tumors exhibit clinical pictures strictly related to the site of origin and dimensions, even if they appear generally with an intramural localization. This latter point may suggest an useful application of endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), mainly followed by the cell-block procedure (CBP) in the differential diagnostic approach...
November 6, 2016: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27791424/giant-maxillary-gingival-fibromatosis
#8
K A Stephenson, G J Klopper, J Opperman, C Favara
We present a case of both unusual pathology and severity - giant maxillary gingival fibromatosis - and discuss the disease and its management, accompanied by clinical imaging. This represents an overlap between maxillofacial and oral surgery, and may present as demonstrated in this case.
October 28, 2016: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/27777327/wiedemann-steiner-syndrome-with-2-novel-kmt2a-mutations-variable-severity-in-psychomotor-development-and-musculoskeletal-manifestation
#9
Jung Min Ko, Jae So Cho, Yongjin Yoo, Jieun Seo, Murim Choi, Jong-Hee Chae, Hye-Ran Lee, Tae-Joon Cho
Wiedemann-Steiner syndrome is a rare genetic disorder characterized by short stature, hairy elbows, facial dysmorphism, and developmental delay. It can also be accompanied by musculoskeletal anomalies such as muscular hypotonia and small hands and feet. Mutations in the KMT2A gene have only recently been identified as the cause of Wiedemann-Steiner syndrome; therefore, only 16 patients from 15 families have been described, and new phenotypic features continue to be added. In this report, we describe 2 newly identified patients with Wiedemann-Steiner syndrome who presented with variable severity...
October 24, 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/27745791/sacrificing-the-internal-carotid-artery-in-infiltrating-neck-tumours-a-study-of-four-clinical-cases
#10
I M Zeitoun, G H Borhamy, M M Fata, A M Salloum
Tumour infiltration of the carotid arteries, especially the common carotid artery (CCA) and the internal carotid artery (ICA), is a great challenge in maxillofacial surgery. Cases in which the malignant tumour and/or lymph node is stuck to the carotid artery, especially the ICA, have previously been considered inoperable. Four such cases, two with recurrent metastatic nodal neck masses encasing the ICA, one with aggressive fibromatosis, and one with a carotid body tumour, are described herein. Successful resection of the mass along with the ICA was performed in all cases after a positive balloon occlusion test...
October 10, 2016: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/27713418/papillary-thyroid-carcinoma-with-nodular-fasciitis-like-stroma-and-%C3%AE-catenin-mutations-should-be-renamed-papillary-thyroid-carcinoma-with-desmoid-type-fibromatosis
#11
Caterina Rebecchini, Antoine Nobile, Simonetta Piana, Rossella Sarro, Bettina Bisig, Sykiotis P Gerasimos, Chiara Saglietti, Maurice Matter, Laura Marino, Massimo Bongiovanni
Various histological variants of papillary thyroid carcinoma have been reported, some with clinical implications, some with peculiar, sometimes misleading morphologies. One of these rare and poorly characterized variants is papillary thyroid carcinoma with nodular fasciitis-like stroma, of which fewer than 30 cases have been documented, mostly as isolated reports. It is a dual tumor comprising a malignant epithelial proliferation that harbors typical features of conventional papillary thyroid carcinoma, admixed with a prominent mesenchymal proliferation resembling nodular fasciitis or fibromatosis...
October 7, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27698644/proximal-interphalangeal-joint-fibromatosis-after-pyrocarbon-implant-insertion-a-case-report
#12
Jacob Rinkinen, Matthew D Chetta, Kevin C Chung
Background: Pyrocarbon implants represent an increasingly popular method to treat proximal interphalangeal joint dysfunction. To this point, no association has been shown between pyrocarbon biomaterials and fibromatosis. We present a potentially serious and destructive complication associated with pyrocarbon arthroplasty. Methods: We demonstrate a clinical case involving pyrocarbon arthroplasty and subsequent fibromatosis development in an otherwise healthy 23-year-old female. To present this association, we illustrate the diagnostic workup involved in a rapidly expanding soft tissue mass of the hand and explain the appropriate treatment...
September 2016: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/27688461/hyaline-fibromatosis-syndrome-a-rare-inherited-disorder
#13
Meeta Dipak Mantri, Mahajan M Pradeep, Patil O Kalpesh, Raj J Pranavsinh
Hyaline fibromatosis syndrome (HFS) is rare autosomal recessive disease characterized by the deposition of amorphous hyaline material in skin and visceral organs. It represents a disease spectrum with infantile systemic hyalinosis (ISH) being the severe form and juvenile hyaline fibromatosis (JHF) being the mild form. Dermatologic manifestations include thickened skin, perianal nodules, and facial papules, gingival hyperplasia, large subcutaneous tumors on the scalp, hyperpigmented plaques over the metacarpophalangeal joints and malleoli, and joint contractures...
September 2016: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/27684985/infantile-digital-fibroma-a-rare-fibromatosis
#14
Etan Marks, Michelle Ewart
Infantile digital fibroma is a rare benign lesion that usually occurs during the first 2 years of life. It can be multiple, but it is usually a single lesion. If it grows large enough it can cause joint deformities or interfere with everyday activities. Microscopically, the neoplastic cells usually have inclusion bodies that are best highlighted with a Masson trichrome stain but can often be seen on hematoxylin-eosin staining. Treatment for this entity is usually watchful waiting because of its ability to spontaneously regress, but excision is recommended if the lesion is symptomatic...
October 2016: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/27650749/image-gallery-complete-remission-of-aggressive-desmoid-type-fibromatosis-after-low-dose-methotrexate-for-2%C3%A2-weeks-in-a-child-with-psoriasis
#15
S Simonsen, A Vera Casaño, E Gómez Moyano, A Sanz Trelles, C Bravo Bravo, L Martínez Pilar
No abstract text is available yet for this article.
October 2016: British Journal of Dermatology
https://www.readbyqxmd.com/read/27635978/recurrent-giant-cranial-desmoid-tumor-in-a-3-year-old-boy-with-familial-adenomatous-polyposis-requiring-bifrontoparietal-cranioplasty-case-report
#16
Luyuan Li, John N Jensen, Sara Szabo, Peter VanTuinen, Sean M Lew
Desmoid tumors, also known as aggressive fibromatosis, are locally infiltrating musculoaponeurotic neoplasms arising in connective tissues. Desmoid tumors may be associated with familial adenomatous polyposis (FAP), a genetic disorder that presents with hundreds to thousands of precancerous colorectal polyps. The authors report the case of an 18-month-old boy who underwent resection of a right temporal desmoid tumor (initially diagnosed as cranial fasciitis) and developed a bilateral frontoparietal calvarial desmoid tumor 2 years later...
December 2016: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/27614106/genetic-analysis-of-hereditary-gingival-fibromatosis-using-whole-exome-sequencing-and-bioinformatics
#17
J Hwang, Y-L Kim, S Kang, S Kim, S-O Kim, J H Lee, D-H Han
OBJECTIVES: Our study aims to identify genetic variants associated with hereditary gingival fibromatosis (HGF) by applying whole-exome sequencing (WES) and bioinformatics analyses such as gene set enrichment analysis (GSEA) and protein functional network study. SUBJECTS AND METHODS: Two affected siblings whose grandparents and parents have normal gingiva were chosen for our investigation. Saliva collected from the patients and their parents were used for WES. GSEA and protein functional network study were performed to find gene groups in a biological coordination which are associated with HGF...
September 10, 2016: Oral Diseases
https://www.readbyqxmd.com/read/27612042/desmoid-type-fibromatosis-of-the-breast-a-case-report
#18
Deepa Bhat, Vanessa Wear, Elliot Weisenberg, Rosalinda Alvarado
Desmoid-type fibromatosis of the breast (also referred to as desmoid tumor or aggressive fibromatosis) is exceedingly rare. Although it does not metastasize, desmoid-type fibromatosisis is frequently locally aggressive. Recurrence is common (up to 35%), even after presumed total excision of the primary tumor [1]. The breast is an unusual location for the development of this tumor, with relatively few cases reported in the literature. We report a case of desmoid-type fibromatosis in the breast in a 31-year-old female who presented with a four-month history of a palpable left breast mass...
August 1, 2016: Breast Disease
https://www.readbyqxmd.com/read/27609299/gingival-fibromatosis-with-significant-de-novo-formation-of-fibrotic-tissue-and-a-high-rate-of-recurrence
#19
Katarzyna Gawron, Katarzyna Łazarz-Bartyzel, Andrzej Fertala, Paweł Plakwicz, Jan Potempa, Maria Chomyszyn-Gajewska
BACKGROUND Hereditary gingival fibromatosis is characterized by slowly progressive enlargement of the gingiva that can present as an isolated condition or present as part of various syndromes. CASE REPORT An 11-year-old female reported with a gingival lesion that caused masticatory problems and poor oral hygiene. Periodontal examination revealed a dense tissue covering 30% of her teeth crowns within both jaws. Panoramic x-ray showed a normal bone height and teeth positioning. The patient did not use any medications, but a similar condition was also present in other family members...
September 9, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27591500/desmoid-type-fibromatosis-evolving-treatment-standards
#20
REVIEW
Marco Fiore, Andrea MacNeill, Alessandro Gronchi, Chiara Colombo
Desmoid-type fibromatosis is a rare nonmetastasizing neoplasm with variable behavior. Recent discoveries into the biology of this disease hold promise for identifying prognostic and predictive features and novel therapeutic targets. Surgery has been the historical standard of care but carries considerable drawbacks in terms of high local recurrence rates and poor functional outcomes. Improved understanding of the natural history of desmoid-type fibromatosis has resulted in a paradigm shift toward nonoperative management...
October 2016: Surgical Oncology Clinics of North America
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