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Dermatofibrosarcoma

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https://www.readbyqxmd.com/read/29140881/pdgfb-rearrangements-in-dermatofibrosarcoma-protuberans-of-the-vulva-a-study-of-11-cases-including-myxoid-and-fibrosarcomatous-variants
#1
Khadijeh Jahanseir, Deyin Xing, Patricia T Greipp, William R Sukov, Gary L Keeney, Brooke E Howitt, J Kenneth Schoolmeester
Dermatofibrosarcoma protuberans (DFSP) is a low-grade fibroblastic sarcoma that tends to arise in young to middle age adults and involve the trunk and proximal extremities. Rare examples of vulvar DFSP have been reported, including myxoid, myoid, and fibrosarcomatous variants, but detection of the characteristic t(17;22)(q22;q13) that produces COL1A1-PDGFB gene fusion has not been evaluated in a large series of primary vulvar tumors. The clinical, morphologic, immunohistochemical, and molecular cytogenetic features of 11 cases were examined...
November 14, 2017: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/29058523/orbitofacial-dermatofibrosarcoma-protuberans-with-intranasal-extension
#2
Siti Nurhuda Sharudin, Siow Wei Tan, Nor Fadhilah Mohamad, Suresh Kumar Vasudevan, Hanizah Khairan, Y C Mun, Jothi Shanmuganathan
A 25-year-old Chinese woman presented with recurrent painless swelling over the left medial canthus region for 3 months and intranasal mass for an indeterminate duration. Initial incision biopsy of the mass was reported as nodular fasciitis but the lesion recurred 3 weeks later. Intraoperative findings during repeat biopsy showed a mass extending from the deep dermal tissue into the anterior orbit and polyp-like nasal mass. Histopathology findings were that of dermatofibrosarcoma protuberans (DFSP). The mass recurred 4 months later without orbital or intranasal recurrence...
October 23, 2017: Orbit
https://www.readbyqxmd.com/read/29026517/dermatofibrosarcoma-protuberans-coexisting-in-a-patient-with-a-vascular-malformation-a-rare-coincidence
#3
Parthena Deskoulidi, Michael Sofopoulos, Pantelis Diamantopoulos, Thaleia Nikolaidou, Nikolaos Maltzaris, Maria Theodorakopoulou, Christos Klonaris, Niki Arnogiannaki, Maria Kotrotsiou, Spiros Stavrianos
Dermatofibrosarcoma protuberans with fibrosarcomatous differentiation (DFSP-FS) is a rare soft tissue tumor with more aggressive behavior and it is not clear what causes this type of skin cancer. We describe the case of a 48-year-old woman who was born with a vascular malformation in the sternal region and presented suddenly with a soft tissue sarcoma (DFSP-FS) in the same territory. She was initially treated by embolization as the sarcoma was misdiagnosed but the tumor within 6 months seemed to be growing rapidly and reached a giant dimension with ulceration and required surgical intervention...
October 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28988501/dermatofibrosarcoma-protuberans-and-gastrointestinal-stromal-tumor-as-models-for-targeted-therapy-in-soft-tissue-sarcomas
#4
Hanna Koseła-Paterczyk, Piotr Rutkowski
The development of novel targeted treatment in soft tissue sarcomas (STS) is important since many sarcoma subtypes are resistant to conventional chemotherapy and effective therapeutic options are limited. Areas covered: This review discusses the molecular background and treatment in two STS types which became a model for targeted therapy - gastrointestinal stromal tumor (GIST) and dermatofibrosarcoma protuberans (DFSP). DFSP is characterized, in the majority of cases, by chromosomal translocation which results in the formation of COL1A1-PDGFB fusion gene causing platelet-derived growth factor receptor beta(PDGFRB) signaling activation in tumor cells...
October 9, 2017: Expert Review of Anticancer Therapy
https://www.readbyqxmd.com/read/28977029/unforeseen-clonal-evolution-of-tumor-cell-population-in-recurrent-and-metastatic-dermatofibrosarcoma-protuberans
#5
Ensel Oh, Hae Min Jeong, Mi Jeong Kwon, Sang Yun Ha, Hyung Kyu Park, Ji-Young Song, Yu Jin Kim, Jong-Sun Choi, Eun Hee Lee, Jeeyun Lee, Yoon-La Choi, Young Kee Shin
Dermatofibrosarcoma protuberans (DFSP) is a very rare soft tissue sarcoma, generally of low-grade malignancy. DFSP is locally aggressive with a high recurrence rate, but metastasis occurs rarely. To investigate the mechanism of metastasis in DFSP, we analyzed the whole exome sequencing data of serial tumor samples obtained from a patient who had a 10-year history of recurrent and metastatic DFSP. Tracking various genomic alterations, namely somatic mutations, copy number variations, and chromosomal rearrangements, we observed a dramatic change in tumor cell population during the occurrence of metastasis in this DFSP case...
2017: PloS One
https://www.readbyqxmd.com/read/28971134/recurrent-dermatofibrosarcoma-protuberans-treated-with-neoadjuvant-imatinib-mesylate-followed-by-mohs-micrographic-surgery
#6
Natalia M Fontecilla, Nicole W Kittler, Larisa Geskin, Faramarz H Samie, George Niedt, Thomas Imahiyerobo, Gary Schwartz, Matt Ingham, Jesse M Lewin
No abstract text is available yet for this article.
November 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28940884/involvement-of-pdgf-ligands-and-receptors-in-tumorigenesis
#7
REVIEW
Carl-Henrik Heldin, Johan Lennartsson, Bengt Westermark
Platelet-derived growth factor (PDGF) isoforms and their receptors have important roles during embryogenesis, particularly in the development of various mesenchymal cell types in different organs. In the adult, PDGF stimulates wound healing and regulates tissue homeostasis. However, over-activity of PDGF signaling is associated with malignancies and other diseases characterized by excessive cell proliferation, such as fibrotic conditions and atherosclerosis. In certain tumors, genetic or epigenetic alterations of the genes for PDGF ligands and receptors drives tumor cell proliferation and survival...
September 22, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28932344/-a-pink-nodule-on-the-face
#8
Amina Kissou, Badredine Hassam
We report the case of a 32-year old female patient, with no notable medical history, requiring dermatology consultation for evaluation of a nodule on her right cheek which had evolved over the past 10 years. Clinical examination showed a reddish dermal-based nodule with a smooth surface. The lesion measured 1cm in diameter and was located at the level of the right cheek (A). There was no adenopathy and the remainder of the clinical examination was normal. The patient underwent skin biopsy which showed tumor proliferation composed of fusiform cells with poorly limited eosinophilic cytoplasm and lightly atypical elongated nuclei without mitosis and with mononuclear inflammatory cell infiltrate at the level of the dermis...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28929166/dermatofibrosarcoma-protuberans-presenting-as-a-large-atrophic-plaque-on-the-chest
#9
Ryosuke Saigusa, Takuya Miyagawa, Satoshi Toyama, Jun Omatsu, Tomomitsu Miyagaki, Yuri Masui, Daisuke Yamada, Shinichi Sato
No abstract text is available yet for this article.
September 20, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28905843/orbital-dermatofibrosarcoma-protuberans-with-frontal-and-ethmoid-sinus-involvement-a-case-report-and-brief-review-of-literature
#10
Deepti Sharma, Garima Singh, Neha Kakkar, Vidya Jha
Dermatofibrosarcoma protuberans is a soft tissue sarcoma that is dermal in origin. The incidence is <0.1% of all malignancies and 1% of soft tissue sarcoma. Most commonly, it involves trunk (62%) followed by extremities (25%) and head and neck (13%). It is a slow growing tumor with locally aggressive behavior. Here, a 50-year-old female diagnosed with orbital dermatofibrosarcoma developed extra-axial component in right frontal region even on chemotherapy. Hence, the bad prognostic factors are yet to be established in dermatofibrosarcoma protuberans...
September 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28883003/discovery-of-human-similar-gene-fusions-in-canine-cancers
#11
Ronan Ulvé, Mélanie Rault, Mathieu Bahin, Laetitia Lagoutte, Jérôme Abadie, Clotilde De Brito, Jean-Michel Coindre, Nadine Botherel, Audrey Rousseau, Valentin Wucher, Edouard Cadieu, Catherine Thieblemont, Christophe Hitte, Laurence Cornevin, Florian Cabillic, Laura Bachelot, David Gilot, Benoit Hennuy, Thierry Guillaudeux, Arnaud Le Goff, Thomas Derrien, Benoît Hédan, Catherine André
Canine cancers represent a tremendous natural resource due to their incidence and striking similarities to human cancers, sharing similar clinical and pathologic features as well as oncogenic events, including identical somatic mutations. Considering the importance of gene fusions as driver alterations, we explored their relevance in canine cancers. We focused on three distinct human-comparable canine cancers representing different tissues and embryonic origins. Through RNA-Seq, we discovered similar gene fusions as those found in their human counterparts: IGK-CCND3 in B-cell lymphoma, MPB-BRAF in glioma, and COL3A1-PDGFB in dermatofibrosarcoma protuberans-like...
September 7, 2017: Cancer Research
https://www.readbyqxmd.com/read/28873112/dermatofibrosarcoma-protuberans
#12
Jessica LeBlanc, Conner Chan, Ann Zedlitz
No abstract text is available yet for this article.
July 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28832078/the-role-of-cd34-and-d2-40-in-the-differentiation-of-dermatofibroma-and-dermatofibrosarcoma-protuberans
#13
Canan Sadullahoğlu, Yelda Dere, Türkan Rezanko Atasever, Mine Tunakan Öztop, Önder Karaaslan
OBJECTIVE: Dermatofibroma (DF) is a benign fibrohistiocytic tumor whereas dermatofibrosarcoma protuberans (DFSP) has intermediate malignant potential. CD34 is the most commonly used antibody in differentiating these tumors. Various studies have stated the rates of D2-40 expression as 0-50% in DFSPs and 86-100% in DFs. Our aim in this study was to determine the expression of CD34 and D2-40 in DFs and DFSPs and the possible use of D2-40 in the differential diagnosis of these lesions. MATERIAL AND METHOD: This is a retrospective study including 30 DF and 15 DFSP cases which were reevaluated for epidermal changes, the presence of a transmission zone (Grenz zone), infiltration of soft tissues, infiltration pattern and histologic subtypes in addition to cellular pleomorphism, nuclear atypia, and necrosis...
2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28802503/fibrohistiocytic-tumors
#14
REVIEW
Ryan C Romano, Karen J Fritchie
Fibrohistiocytic tumors are a diverse group of reactive and neoplastic lesions including xanthoma, fibrous histiocytoma and its variants, solitary xanthogranuloma, dermatofibrosarcoma protuberans, and atypical fibroxanthoma. This article reviews some of the more commonly encountered fibrohistiocytic tumors with an emphasis on clinical presentation, macroscopic and histologic characteristics, molecular/cytogenetic findings where applicable, and differential diagnoses.
September 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/28802488/characterization-of-surgical-procedures-in-the-spanish-mohs-surgery-registry-regesmohs-for-2013-2015
#15
E de Eusebio Murillo, A Martín Fuentes, V Ruiz-Salas, J R Garcés, R Miñano Medrano, J L López-Estebaranz, T Alonso-Alonso, M Á Rodríguez-Prieto, Y Delgado Jiménez, V Morales, P Redondo Bellón, E Manubens-Mercadé, O Sanmartín-Jiménez, C Guillén Barona, R Cabeza, N Cano, A Toll-Abelló, R Navarro Tejedor, J L Artola Igarza, I Allende Markixana, A Alfaro Rubio, C Ciudad Blanco, H Vázquez-Veiga, L Barchino Ortiz, M A Descalzo, I García-Doval
INTRODUCTION: The Spanish Mohs Surgery Registry is used to collect data on the use and outcomes of Mohs micrographic surgery (MMS) in Spain. The aim of this study was to describe perioperative and intraoperative data recorded for MMS procedures performed between July 2013 (when the registry started) and January 2016. MATERIAL AND METHODS: Prospective cohort study of data from 18 hospitals. The data collected included type of anesthesia, surgical technique, hospital admission, number of Mohs stages, management of preoperative risk factors, additional treatments, previous treatments, type of tumor, operating time, and complications...
November 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28795284/dermatofibrosarcoma-protuberans
#16
REVIEW
Alvaro E Acosta, Catalina Santa Vélez
Dermatofibrosarcoma protuberans (DFSP) is a slow growing tumor with a very low metastatic potential but with significant subclinical extension and great capacity for local destruction. Thus, the first surgeon approached with such challenging tumor must attempt to cure the patient with a method that spares healthy tissue and ensures an optimal oncological, functional, and esthetic result. The treatment of DFSP often requires a multidisciplinary approach. Depending on location, dermatologic surgeons, surgical oncologists, head and neck surgeons, neurosurgeons, plastic surgeons, and occasionally medical oncologists may be involved with the management...
August 10, 2017: Current Treatment Options in Oncology
https://www.readbyqxmd.com/read/28776579/significance-of-h3k27me3-loss-in-the-diagnosis-of-malignant-peripheral-nerve-sheath-tumors
#17
Melike Pekmezci, Areli K Cuevas-Ocampo, Arie Perry, Andrew E Horvai
The diagnosis of malignant peripheral nerve sheath tumors can be challenging and other spindle cell sarcomas commonly enter in the differential diagnosis. Loss of trimethylation at lysine 27 of histone-H3 (H3K27me3) by immunohistochemistry was recently described in malignant peripheral nerve sheath tumors. However, its specificity remains controversial. We therefore studied 82 synovial sarcomas, 39 malignant peripheral nerve sheath tumors, and 10 fibrosarcomatous dermatofibrosarcoma protuberans for H3K27me3 loss by immunohistochemistry...
August 4, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28755830/-dermatofibrosarcoma-protuberans-surgical-margins-using-slow-mohs-micrographic-surgery-a-clinical-retrospective-study-about-20-cases
#18
C Verbruggen, A S Ricard, O Cogrel, M Bondaz, S Carrier
OBJECTIVES: The main objective of this study is to determine the necessary surgical margins to obtain a complete R0 resection for head and neck dermatofibrosarcoma protuberans (DFSP) using Slow-Mohs micrographic surgery. The secondary objective is to study the recurrence rate of these tumors. PATIENTS AND METHODS: Slow-Mohs micrographic surgery was used for patients included between 2005 and 2015 at Bordeaux universitary hospital. For each patient the age, the sex and death occurrence, the initial surgical margins, the surgical margins for complete R0 resection, the occurrence of local or general recurrence during follow-up were reported...
July 26, 2017: Annales de Chirurgie Plastique et Esthétique
https://www.readbyqxmd.com/read/28751990/dermal-substitutes-use-in-reconstructive-surgery-for-skin-tumors-a-single-center-experience
#19
Mariane Campagnari, Andrea S Jafelicci, Helio A Carneiro, Eduard R Brechtbühl, Eduardo Bertolli, João P Duprat Neto
Reconstructive surgery following skin tumor resection can be challenging. Treatment options after removing the tumor are skin grafting, local pedicled and axial flaps, or microsurgery for complex and extensive wounds correction. Recently, the use of dermal substitutes has been extended to reconstructive surgery in cutaneous oncology. Objectives. To report both a single-center experience using dermal substitutes in reconstructive surgery for skin malignancies and reconstructive surgery's outcomes. Methods and Results...
2017: International Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28711648/alteration-of-pdgfr%C3%AE-akt-mtor-pathway-signaling-in-fibrosarcomatous-transformation-of-dermatofibrosarcoma-protuberans
#20
Yuka Hiraki-Hotokebuchi, Yuichi Yamada, Kenichi Kohashi, Hidetaka Yamamoto, Makoto Endo, Nokitaka Setsu, Kuma Yuki, Takamichi Ito, Yukihide Iwamoto, Masutaka Furue, Yoshinao Oda
Dermatofibrosarcoma protuberans (DFSP) is a cutaneous mesenchymal tumor of intermediate malignancy and fibroblastic/myofibroblastic differentiation. Fibrosarcomatous (FS) component is a high-grade component of DFSP. The detailed oncogenic difference between DFSP and FS components is not clear. We thus investigated the Akt-mTOR pathway in both components. We used 65 tumor samples obtained from 65 patients. The phosphorylation of Akt-mTOR pathway proteins (Akt, mTOR, 4EBP1, and S6RP) and PDGFRα/β was assessed by immunohistochemical staining, the results of which were confirmed by Western blotting...
September 2017: Human Pathology
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