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https://www.readbyqxmd.com/read/29606823/recurrent-dermatofibrosarcoma-protuberans-of-scalp-in-a-distant-location-10-years-after-primary-excision
#1
Chang Min Kim, Tae Jun Park, Bo Young Kim, Seung Hyun Chun, Il-Hwan Kim
Dermatofibrosarcoma protuberans (DFSP) is a slow growing low-grade cutaneous sarcoma. Local recurrence after excision is common due to the poorly defined periphery that renders histological control of surgical margin difficult, Mohs micrographic surgery is the optimal method for treatment. A 41 years old male patient, who had a previous history of DFSP, came to our dermatology clinic for evaluation of an asymptomatic firm flesh-colored nodule on the forehead. Total excision biopsy was done and the mass was histologically proved as DFSP...
April 2018: Annals of Dermatology
https://www.readbyqxmd.com/read/29531869/a-rare-case-of-dermatofibrosarcoma-protuberans-of-the-thumb-in-an-80-year-old-woman
#2
Georgi P Georgiev, Svetoslav A Slavchev, Julian Ananiev
Dermatofibrosarcoma protuberans (DFSP) has been described as a rare, locally invasive, malignant fibroblastic tumor with a high rate of recurrence that usually affects middle-aged patients. Herein, we describe a rare case of a pedunculated dermatofibrosarcoma protuberans of the right thumb in an 80-year-old woman treated with excision through the base of the pedicle. We also make a brief literature review concerning this tumor.
January 2, 2018: Curēus
https://www.readbyqxmd.com/read/29509956/relapse-in-dermatofibrosarcoma-protuberans-a-histological-and-molecular-analysis
#3
André S Molina, João P Duprat Neto, Eduardo Bertolli, Isabela W da Cunha, José H T G Fregnani, Pedro H M Figueiredo, Fernando A Soares, Mariana P Macedo, Clovis A Pinto Lopes, Ivan D de Abranches Oliveira Santos Filho
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare low grade tumor with a locally aggressive behavior and low metastatic potential. OBJECTIVES: To evaluate the factors that are associated with relapse in DFSP. Methods Retrospective analysis of medical records from 61 patients with dermatofibrosarcoma. Fluorescence in situ hybridization was used to detect translocations. RESULTS: Of 61 patients, 6 experienced a relapse. No patient with resection margins greater than 3 cm had a recurrence...
March 6, 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29492209/egfr-is-involved-in-dermatofibrosarcoma-protuberans-progression-to-high-grade-sarcoma
#4
Amélie Osio, Shuo Xu, Morad El Bouchtaoui, Christophe Leboeuf, Guillaume Gapihan, Christine Lemaignan, Guilhem Bousquet, Céleste Lebbé, Anne Janin, Maxime Battistella
Dermatofibrosarcoma protuberans (DFSP), amounting to 6% of all soft tissue sarcomas, has a slow growth rate, contrasting with a likelihood for local recurrence and a 10-20% evolution to higher-grade sarcoma, or "transformed DFSP" (DFSP-T). At molecular level, the characteristic COL1A1-PDGFB rearrangement, leading to sustained PDGFR signaling, is not linked to the evolutive potential. Here, we studied EGFR, another tyrosine kinase receptor, using laser-microdissection to select the different histologic components of DFSP (DFSP center, DFSP infiltrative periphery, DFSP-T higher-grade sarcoma), in 22 patients followed over 3 to 156 months...
February 2, 2018: Oncotarget
https://www.readbyqxmd.com/read/29483976/dermatofibrosarcoma-protuberans-retrospective-single-center-analysis-over-16-years
#5
Uwe Wollina, Dana Langner, Jacqueline Schönlebe, Katlein França, Torello Lotti, Georgi Tchernev
Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and Allergology between 08/2001 and 08/2017. The diagnosis had been confirmed by histology and immunohistology in all cases. We identified four adults and a pediatric patient with DFSP. All patients were treated by wide surgical excision and controlled by three-dimensional histologic margin control...
January 25, 2018: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29468850/an-unusual-clinical-presentation-of-myxoid-dermatofibrosarcoma-protuberans-with-a-prominent-vasculature-a-potential-pitfall-in-the-diagnosis-of-myxoid-soft-tissue-tumors
#6
Patrick Tran, Gregory P Henderson, Michael McLemore
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor that arises primarily on the trunk and extremities but seldom on the scalp. Several variants of DFSP have been described, including myxoid DFSP. Although typical DFSP may have focally myxoid areas, myxoid DFSP, in which most of the stroma is myxoid, is rare and can pose diagnostic challenges. Here, we report a case of myxoid DFSP with an unusual clinical presentation that could have been mistaken for a lipoma. Additionally, the myxoid DFSP displayed prominent vasculature in a myxoid stroma, which could have been mistaken for a myxoid liposarcoma...
February 22, 2018: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/29416224/a-rare-case-of-misdiagnosis-recurrence-of-dermatofibrosarcoma-protuberans-that-was-treated-surgicallyas-a-keloid
#7
Murat Ucak
Aim: In this report, we presented the patient with Dermatofibrosarcoma Protuberans (DFSP), removed by considering as the keloid scar in the general surgery clinic with misdiagnosis. Case report: The patient was a 19-year-old female student with no scar or previous trauma history in the lesion area. Pathology report of excisional biopsy revealed as a DFSP, reachedsubcutis and dermis. In staging by CT, there had been no distant metastases. There was a lesion with the size of 2x1...
February 2018: Medical Archives
https://www.readbyqxmd.com/read/29408302/the-role-of-small-molecule-platelet-derived-growth-factor-receptor-pdgfr-inhibitors-in-the-treatment-of-neoplastic-disorders
#8
REVIEW
Robert Roskoski
Platelet-derived growth factor (PDGF) was discovered as a serum-derived component necessary for the growth of smooth muscle cells, fibroblasts, and glial cells. The PDGF family is a product of four gene products and consists of five dimeric isoforms: PDGF-AA, PDGF-BB, PDGF-CC, PDGF-DD, and the PDGF-AB heterodimer. This growth factor family plays an essential role in embryonic development and in wound healing in the adult. These growth factors mediate their effects by binding to and activating their receptor protein-tyrosine kinases, which are encoded by two genes: PDGFRA and PDGFRB...
February 2, 2018: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/29309822/fluorescence-microscopy-for-the-evaluation-of-elastic-tissue-patterns-within-fibrous-proliferations-of-the-skin-on-hematoxylin-eosin-stained-slides
#9
Robert Borucki, David M Perry, Dan R Lopez-Garcia, Viktoryia Kazlouskaya, Dirk M Elston
BACKGROUND: Diagnosis of fibrous tumors can be challenging and expensive due to the use of special stains. OBJECTIVE: Determine the usefulness of fluorescence microscopy in the evaluation of elastic pattern on H&E stained slides. METHODS: A total of 228 slides were evaluated by fluorescence microscopy for elastic tissue patterns and sensitivity and specificity determined for relevant comparisons. RESULTS: Fluorescence microscopy was found to be useful especially in the case of distinguishing dermatofibroma (DF) vs dermatofibrosarcoma protuberans (DFSP) and also dermatomyofibroma (DMF) vs other fibrous tumors...
January 5, 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29240584/dermatofibrosarcoma-protuberans-of-distal-extremities-and-acral-sites-a-clinicopathologic-analysis-of-27-cases
#10
Kabeer K Shah, Jonathan B McHugh, Andrew L Folpe, Rajiv M Patel
Dermatofibrosarcoma protuberans (DFSP) of the distal extremities and acral sites are extremely rare and incompletely characterized. Twenty-seven DFSP occurring in these sites were retrieved from our collective archives and reevaluated. Tumors occurred in 16 males and 11 females. Median age at presentation was 42.5 years (range, 7 to 78 y). Lesions involved the foot (18 with 6 in the toes and 2 on the plantar foot), distal ankle (4), hand (4 with 2 in the thumbs), and wrist (1). All cases showed predominantly classic DFSP morphology and were diffusely CD34 positive...
March 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29175214/survival-in-patients-with-primary-dermatofibrosarcoma-protuberans-national-cancer-database-analysis
#11
Oleksandr Trofymenko, Jeremy S Bordeaux, Nathalie C Zeitouni
BACKGROUND: The predictors of mortality, second surgery, and postoperative radiation therapy for treating dermatofibrosarcoma protuberans (DFSP) are not well described. OBJECTIVE: We sought to determine the impact of patient demographics, tumor characteristics, and treatment site and modality on survival after primary DFSP. METHODS: A retrospective analysis of data from the National Cancer Database was performed for patients diagnosed with DFSP during 2003-2012...
November 23, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29140881/pdgfb-rearrangements-in-dermatofibrosarcoma-protuberans-of-the-vulva-a-study-of-11-cases-including-myxoid-and-fibrosarcomatous-variants
#12
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
#13
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
#14
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
#15
REVIEW
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 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, 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...
December 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
#16
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/28832078/the-role-of-cd34-and-d2-40-in-the-differentiation-of-dermatofibroma-and-dermatofibrosarcoma-protuberans
#17
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/28795284/dermatofibrosarcoma-protuberans
#18
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/28755830/-dermatofibrosarcoma-protuberans-surgical-margins-using-slow-mohs-micrographic-surgery-a-clinical-retrospective-study-about-20-cases
#19
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/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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