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Aggressive fibromatosis

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
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
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
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...
September 16, 2016: Journal of Neurosurgery. Pediatrics
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
Danique L M van Broekhoven, Dirk J Grünhagenl, Thijs van Dalen, Frits van Coevorden, Han J Bonenkamp, Lukas B Been, Marc H A Bemelmans, Sander D S Dijkstra, Chiara Colombo, Alessandro Gronchi, Cornelis Verhoef
BACKGROUND: The efficacy of the classical treatment modalities surgery and radiotherapy in the treatment of aggressive fibromatosis is presently disputed and there is a shift towards a more conservative approach. The aim of the present study is to objectify tumor growth in patients with extra-abdominal or abdominal wall aggressive fibromatosis, while adhering to a "watchful waiting" policy. Other objectives are to investigate quality of life and to identify factors associated with tumor growth, in particular the relation with the presence of a CTNNB1-gene mutation in the tumor...
2016: BMC Cancer
Mohamed Fawzy, Mohamed Sedky, Hossam ElZomor, Magdy El Sherbiny, Emad Salama, Ahmed Mahdy
BACKGROUND: Nearly half of soft tissue sarcomas are nonrhabdomyosarcomas (NRSTSs). The low-grade (LG) form comprises a heterogenous group of diseases that rarely metastasize but are known for local recurrence. AIM OF THE STUDY: The aim of the study was to retrospectively evaluate pediatric LG-NRSTS with regard to demography, survival, and factors affecting outcome in Egyptian patients. PATIENTS AND METHODS: The study reviewed 66 NRSTS patients who presented to the Pediatric Oncology Department, National Cancer Institute, Cairo University, between January 2008 and December 2013...
August 19, 2016: Journal of Pediatric Hematology/oncology
Z Y Yue, B Ding, Y G Dong, H Wang, W X Li
No abstract text is available yet for this article.
August 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Shogo Shinohara, Atsushi Suehiro, Masahiro Kikuchi, Hiroyuki Harada, Ippei Kishimoto, Yukihiro Imai
Extra-abdominal desmoid tumor, also known as aggressive fibromatosis, has aggressive behavior with local infiltration and tendency for recurrence. Though head and neck is reported to be one of the most common sites, a desmoid tumor in the larynx is extremely rare. A 67-year-old male visited our hospital with prolonged hoarseness and received laryngo-microsurgery with the diagnosis of laryngeal polyp. After the operation, he eventually developed a laryngeal squamous cell carcinoma with papilloma, confirmed by second laryngo-microsurgery and received radiation therapy...
July 26, 2016: Auris, Nasus, Larynx
D Ma, S Li, R Fu, Z Zhang, Y Cui, H Liu, Y Meng, W Wang, Y Bi, Y Xiao
AIMS: The purpose of the study was to review a large series of chest aggressive fibromatosis (AF) cases with an emphasis on the outcomes of different degrees of resection and the value of postoperative radiotherapy. MATERIALS AND METHODS: The records of patients with chest AF treated at our hospital from 1982 to 2014 were retrospectively reviewed. Recurrence rates and non-disease survival (NDS) times were compared between the R0, R1, and R2 resection groups. RESULTS: Forty-seven cases of chest AF were treated during the study period (21 men, 26 women), with an average age at diagnosis of 40 years (range, 9-77 years)...
June 29, 2016: European Journal of Surgical Oncology
Kiran M Sargar, Elizabeth F Sheybani, Archana Shenoy, John Aranake-Chrisinger, Geetika Khanna
Pediatric fibroblastic and myofibroblastic tumors are a relatively common group of soft-tissue proliferations that are associated with a wide spectrum of clinical behavior. These tumors have been divided into the following categories on the basis of their biologic behavior: benign (eg, myositis ossificans, myofibroma, fibromatosis colli), intermediate-locally aggressive (eg, lipofibromatosis, desmoid fibroma), intermediate-rarely metastasizing (eg, inflammatory myofibroblastic tumors, infantile fibrosarcoma, low-grade myofibroblastic sarcoma), and malignant (eg, fibromyxoid sarcoma, adult fibrosarcoma)...
July 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Kyong-Hwa Park, Yoon Ji Choi, Kwan-Woo Kim, Kyung-Han Ro, Chang Ho Kang, Sang-Heon Song, Jong Hoon Park
OBJECTIVE: To elucidate the clinical benefit and safety of low-dose chemotherapy using methotrexate and vinblastine in patients (mostly adults) with progressive and/or symptomatic fibromatosis. METHODS: Patients were enrolled if they were treated with methotrexate and vinblastine chemotherapy for recurrences after surgical excision or newly diagnosed aggressive fibromatosis that was not amenable to surgical resection at the Korea University Medical Center from May 2008 to February 2016...
September 2016: Japanese Journal of Clinical Oncology
Jorge Tadeo Palacios-Zertuche, Servando Cardona-Huerta, María Luisa Juárez-García, Everardo Valdés-Flores, Gerardo Enrique Muñoz-Maldonado
BACKGROUND: Desmoid tumours are one of the rarest tumours worldwide, with an estimated yearly incidence of 2-4 new cases per million people. They are soft tissue monoclonal neoplasms that originate from mesenchymal stem cells. It seems that the hormonal and immunological changes occurring during pregnancy may play a role in the severity and course of the disease. CLINICAL CASE: The case is presented on 28-year-old female in her fifth week of gestation, in whom an abdominal wall tumour was found attached to left adnexa and uterus while performing a prenatal ultrasound...
June 15, 2016: Cirugia y Cirujanos
Gregory Nicolas, Tony Kfoury, Rasha Shimlati, Maroon Tohme, Raja Wakim
BACKGROUND: Mesenteric fibromatosis, also known as mesenteric desmoids, is part of the clinical-pathologic spectrum of deep fibromatosis, which encompasses a group of benign fibro-proliferative processes that are locally aggressive and have the capacity to infiltrate or recur without metastasis. CASE REPORT: Case of a 45-year-old man, with a history of hypertension and lung fibrosis, presenting for a left abdominal mass, which was found incidentally during his lung fibrosis imaging...
2016: American Journal of Case Reports
Narasimhan P Agaram, Lei Zhang, Yun-Shao Sung, Chun-Liang Chen, Catherine T Chung, Cristina R Antonescu, Christopher Dm Fletcher
The family of pediatric fibroblastic and myofibroblastic proliferations encompasses a wide spectrum of pathologic entities with overlapping morphologies and ill-defined genetic abnormalities. Among the superficial lesions, lipofibromatosis (LPF), composed of an admixture of adipose tissue and fibroblastic elements, in the past has been variously classified as infantile fibromatosis or fibrous hamartoma of infancy. In this regard, we have encountered a group of superficial soft tissue tumors occurring in children and young adults, with a notably infiltrative growth pattern reminiscent of LPF, variable cytologic atypia, and a distinct immunoprofile of S100 protein and CD34 reactivity, suggestive of neural differentiation...
October 2016: American Journal of Surgical Pathology
Deqin Ma, Natalya V Guseva, Laila Dahmoush, Robert A Robinson
Struma ovarii accounts for 5% of ovarian teratomas. Malignant transformation occurs in <0.3%, however, the underlying molecular mechanism is unknown. We report a patient with follicular variant and tall cell variant of papillary thyroid carcinoma (PTC) arising from struma ovarii and coexisting incidental PTC in the thyroid. Mutation analysis by next-generation sequencing identified a novel germline mutation, KIT p.V530I mutation in the tumors and normal ovarian and thyroid tissue. Immunohistochemical staining showed loss of KIT expression in the PTCs...
September 2016: International Journal of Gynecological Pathology
Marta Braschi-Amirfarzan, Abhishek R Keraliya, Katherine M Krajewski, Sree Harsha Tirumani, Atul B Shinagare, Jason L Hornick, Elizabeth H Baldini, Suzanne George, Nikhil H Ramaiya, Jyothi P Jagannathan
Desmoid-type fibromatosis (DF) is a locally aggressive fibroblastic neoplasm that has variable clinical and biologic behaviors ranging from indolent tumors that can undergo spontaneous regression to aggressive tumors with a tendency toward local invasion and recurrence. The management of DF has evolved considerably in the last decade from aggressive first-line surgery and radiation therapy to systemic treatment (chemotherapy, hormonal therapy, and targeted therapy) and symptomatic local control (surgery and radiation therapy)...
May 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Travis G O'Brien, Robert J Spinner, Andrea J Boon
INTRODUCTION: Neuromuscular choristomas (NMCs) are rare benign peripheral nerve lesions in which skeletal muscle tissue is admixed with nerve fascicles. METHODS: We describe a case of sciatic nerve NMC presenting with unilateral limb hypoplasia, monoparesis, and equinovarus contracture in a pediatric patient. We outline the unique clinical presentation and diagnostic work-up for our patient, including electromyographic and imaging studies. RESULTS: MRI revealed fusiform enlargement of the sciatic nerve, <50% intralesional fat, and signal characteristics similar to those of muscle tissue...
October 2016: Muscle & Nerve
Zoran Campara, Aleksandar Spasic, Predrag Aleksic, Bosko Milev
INTRODUCTION: Aggressive fibromatosis (AF) is a heterogeneous group of mesenchymal tumors that have locally infiltrative growth and a tendency to relapse. The clinical picture is often conditioned by the obstruction of the ureter or small intestine. Diagnosis is based on clinical, radiological and histological parameters. A CASE REPORT: We report a case of male patient, aged 35 years, with the retroperitoneal fibromatosis. He reported to the physician because of frequent urination with the feeling of pressure and pain...
April 2016: Medical Archives
Riyam T Zreik, Karen J Fritchie
OBJECTIVES: Desmoid-type fibromatosis is a locally aggressive neoplasm composed of long sweeping fascicles of bland fibroblasts/myofibroblasts. Tumors with classic features are easy to identify, but the morphologic spectrum of this entity spans beyond the conventional histologic pattern. Since sampling of an unrecognized histologic variant may lead to misdiagnosis, especially on small needle biopsy specimens, we sought to fully characterize the morphologic spectrum of this entity. METHODS: We examined 165 cases of desmoid-type fibromatosis and catalogued the morphologic patterns and percentage of those patterns identified in each case...
March 2016: American Journal of Clinical Pathology
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