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https://www.readbyqxmd.com/read/28191686/dermoscopy-of-uncommon-variants-of-dermatofibrosarcoma-protuberans
#1
Claudia Costa, Milena Cappello, Giuseppe Argenziano, Vincenzo Piccolo, Massimiliano Scalvenzi
Darier-Ferrand dermatofibrosarcoma protuberans (DFSP) is a locally aggressive fibrohistiocytic tumour with a low metastatic potential.(1) Because of its rarity, slow progression and lack of early clinical clues, the diagnosis of DFSP is often delayed. Classical DFSP clinically appeared like an indurated, irregularly-shaped plaques exhibiting flesh to reddish-brown colour. Some lesions also showed thin teleangectasia on the surface (Fig. 1, a). This article is protected by copyright. All rights reserved.
February 13, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28150334/application-of-col1a1-pdgfb-fusion-gene-detection-by-fluorescence-in%C3%A2-situ-hybridization-in-biopsy-tissue-of-dermatofibrosarcoma-protuberans
#2
Zhang Zhang, Huijiao Chen, Min Chen, Xin He, Yiying Wang, Hongying Zhang
Several uncommon variants of dermatofibrosarcoma protuberans (DFSP) and the limitations of small biopsies render pathological diagnosis difficult. The aim of this study was to analyze the utility of fluorescence in situ hybridization (FISH) in the detection of the collagen type I-α1/platelet derived growth factor-β (COL1A1-PDGFB) fusion gene in biopsies of DFSP. Twenty-three consecutive biopsy specimens of DFSP were reviewed for clinicopathological features and examined with the COL1A1-PDGFB fusion probe and PDGFB break-apart probe using FISH analysis...
February 2, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28134725/sclerosing-dermatofibrosarcoma-protuberans-shows-significant-overlap-with-sclerotic-fibroma-in-both-routine-and-immunohistochemical-analysis-a-potential-diagnostic-pitfall
#3
Maram Yousef Odeh Abdaljaleel, Jeffrey P North
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-to-intermediate grade sarcoma with several histologic variants, including pigmented (Bednar tumor), sclerosing, myxoid, atrophic, and DFSP with fibrosarcomatous changes. Two patterns of sclerosis in DFSP can be observed, a sclerotic fibroma-like pattern and a morphea/lichen sclerosus-like pattern. Partial biopsies of sclerosing DFSPs with the sclerotic fibroma pattern can be misdiagnosed as sclerotic fibroma or other benign sclerosing tumors (eg, perineurioma, dermatofibroma)...
February 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28056985/a-single-institutional-review-of-68-patients-with-dermatofibrosarcoma-protuberans-wide-re-excision-after-inadequate-previous-surgery-results-in-a-high-rate-of-local-control
#4
Kamran Harati, Kim Lange, Ole Goertz, Armin Lahmer, Nicolai Kapalschinski, Ingo Stricker, Marcus Lehnhardt, Adrien Daigeler
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous soft tissue sarcoma characterized by an indolent but aggressive local growth. Unplanned excisions with positive margins are common, and the prognostic impact of radical re-excisions is still unclear. The aim of the present study was to identify prognostic indicators of recurrence-free survival (RFS) in patients with DFSP through a long-term follow-up. We tried particularly to determine the prognostic impact of surgical margins and re-excisions in patients after earlier inadequate surgery...
January 5, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28018683/nodular-fasciitis-complicating-a-staged-surgical-excision-of-dermatofibrosarcoma-protuberans
#5
C Helen Malone, Brandon Goodwin, Richard F Wagner, Vicente Resto, Brent Kelly
Dermatofibrosarcoma protuberans (DFSP) is an unusual spindle cell tumor with a high rate of local recurrence with traditional excision. Fortunately, Mohs micrographic surgery yields excellent cure rates for this neoplasm due to contiguous tumor spread and meticulous tumor mapping and margin analysis. We present the unique case of a patient treated with a modified Mohs technique with an analysis of the final margin with permanent sections, who developed a spindle cell neoplasm in the margins of her second stage excision consistent with nodular fasciitis...
2016: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/27979330/-what-surgery-for-recurrent-darier-ferrand-sarcoma-of-the-chest-wall
#6
S Rabiou, Y Ouadnouni, B Efared, L Belliraj, I Issoufou, F Z Ammor, J Ghalimi, M Lakranbi, R Sani, A Oufkir, M Smahi
INTRODUCTION: Dermatofibrosarcoma protuberans (Darrier-Ferrand sarcoma, DFSP) is an uncommon tumor. This sarcoma has a tendency to local recurrence, requiring a wide surgical resection. OBSERVATION: We report herein two cases of patients presenting with recurrent dermatofibrosarcoma protuberans after several surgical resections. A wide surgical resection with guided tissue regenaration has been performed in one case, whereas the second case required a skin graft...
December 12, 2016: Revue de Pneumologie Clinique
https://www.readbyqxmd.com/read/27922973/incidence-and-outcomes-of-dermatofibrosarcoma-protuberans-in-the-us-pediatric-population
#7
Gustavo A Rubio, Andrea Alvarado, David J Gerth, Jun Tashiro, Seth R Thaller
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade soft tissue sarcoma. In the pediatric population, DFSP is exceedingly rare. Aim of this study was to describe the epidemiology and clinical outcomes in a large pediatric cohort. METHODS: Surveillance, Epidemiology, and End Results (SEER) database (1973-2010) was analyzed for all patients with dermatofibrosarcoma occurring in patients <20 years of age. Data were extracted based on age, gender, race, anatomic site, histology, stage, treatment modalities, and survival...
January 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27895450/dermatofibrosarcoma-protuberans-a-case-report-and-review-of-the-literature
#8
D Paramythiotis, G Stavrou, D Panagiotou, G Petrakis, A Michalopoulos
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) represents less than 0.1% of all tumors, but it is considered the most common skin sarcoma. Wide local excision (=5 cm) has been largely replaced by Mohs micrographic surgery; however, recurrence is not rare. Description of the case: A 35-year-old man presented with a large tumor on the upper side of his back and underwent local excision with the possible preoperative diagnosis of lipoma. Upon histological examination, the diagnosis of DFSP was made, and the patient underwent wide local excision with skin flap reconstruction and was referred for adjuvant radiotherapy...
January 2016: Hippokratia
https://www.readbyqxmd.com/read/27806849/dermatofibrosarcoma-protuberans-pathology-genetics-and-potential-therapeutic-strategies
#9
Khin Thway, Jonathan Noujaim, Robin L Jones, Cyril Fisher
Dermatofibrosarcoma protuberans (DFSP), the most common dermal sarcoma, is a malignant fibroblastic tumor most frequently arising in middle-aged adults. It is typically a low-grade sarcoma that grows slowly but has a high rate of local recurrence with low metastatic potential. Dermatofibrosarcoma protuberans is characterized by a specific translocation t(17;22)(q22;q13) leading to the formation of COL1A1-PDGFB fusion transcripts. Histologically, DFSP has characteristic morphology, of storiform islands of bland spindle cells, and immunohistochemically, it shows diffuse expression of CD34...
December 2016: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/27799980/a-tete-a-tete-of-benign-borderline-and-malignant-fibrohistiocytic-tumor
#10
Rashmi M V, Pavithra P, Shivakumarappa G M
Dermatofibrosarcoma protruberans is a relatively uncommon slow growing, locally aggressive fibrous tumor of the skin. It has a prospensity of progressing to fibrosarcomatous change in 5% of the cases. We present a case of a 56 yr old male with presented to the outpatient department of surgery, Sri Siddhartha Medical College, Tumkur with a chest swelling in 2013. FNAC was inconclusive and the mass was excised. On histopathology, areas of benign fibrohistiocytic tumor, dermatofibrosarcoma protruberans and fibrosarcomatous dermatofibrosarcoma were identified in the same tumor...
2016: Iranian Journal of Pathology
https://www.readbyqxmd.com/read/27753236/dermatofibrosarcoma-protuberans-a-comprehensive-review-on-the-spectrum-of-clinico-radiological-presentations
#11
REVIEW
Tony Ht Sung, Annie Cw Tam, Jennifer Ls Khoo
Dermatofibrosarcoma Protuberans (DFSP) is a rare malignant soft-tissue neoplasm which is often misdiagnosed due to its indolent clinical course and non-specific radiological appearances. An observation case series was conducted with retrospective review of clinical and radiological data of DFSP patients presenting to a major tertiary hospital in Hong Kong for radiological assessment between November 2006 and February 2016. Seven patients with confirmed histological diagnosis of DFSP were included. Tumour sizes at presentation ranged from 1 to 5 cm, most commonly (n = 6) occurred over chest wall and abdominal wall...
October 18, 2016: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/27749444/a-comparison-of-mohs-micrographic-surgery-and-wide-local-excision-for-treatment-of-dermatofibrosarcoma-protuberans-with-long-term-follow-up-the-mayo-clinic-experience
#12
Garrett C Lowe, Oluwakemi Onajin, Christian L Baum, Clark C Otley, Christopher J Arpey, Randall K Roenigk, Jerry D Brewer
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with slow infiltrative growth and local recurrence if inadequately excised. OBJECTIVE: To compare long-term outcomes after Mohs micrographic surgery (MMS) and wide local excision (WLE). MATERIALS AND METHODS: Records of patients with DFSP surgically treated with WLE or MMS from January 1955 through March 2012 were retrospectively reviewed. RESULTS: Mean follow-up for patients treated with MMS (n = 67) and WLE (n = 91) was 4...
January 2017: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/27746644/pedunculated-nodules-as-a-variant-of-dermatofibrosarcoma-protuberans
#13
Min Jung Kim, Min Seok Hur, Byung Gon Choi, Soo Young Kim, Yang Won Lee, Yong Beom Choe, Kyu Joong Ahn
Dermatofibrosarcoma protuberans (DFSP) is a rare disease of dermal fibroblastic origin that accounts for less than 5% of all soft tissue sarcomas in adults. DFSP grows slowly and is an asymptomatic lesion at the initial diagnosis. Herein, we report a case of multiple pedunculated nodules as a variant of DFSP. A 47-year-old man presented with a 7-month history of multiple well-circumscribed, firm, pedunculated nodules on the inguinal area. Histopathologic examination results showed densely packed uniform spindle cells with a storiform and cartwheel pattern, and positivity for CD34...
October 2016: Annals of Dermatology
https://www.readbyqxmd.com/read/27639035/trends-in-incidence-and-survival-analysis-in-non-melanoma-skin-cancer-from-1994-to-2012-in-girona-spain-a-population-based-study
#14
J Rubió-Casadevall, A M Hernandez-Pujol, M C Ferreira-Santos, G Morey-Esteve, L Vilardell, G Osca-Gelis, N Vilar-Coromina, R Marcos-Gragera
INTRODUCTION: We present an epidemiological study focused on Non-melanoma skin cancers (NMSC), including squamous cell carcinoma (SCC), basal cell carcinoma (BCC), Merkel cell carcinoma (MCC), dermatofibrosarcoma protuberans (DFS) and adnexal and skin appendages neoplasm (ASAN), a neoplasm understudied in cancer registries. MATERIAL AND METHODS: We analyze trends of incidence and survival of NMSC registered with the Cancer Registry of Girona, Spain. RESULTS: We found 14389 cases of NMSC, accounting 3,474 SCC, 10729 BCC, 33 MCC, 61 DFSP and 71 ASAN...
December 2016: Cancer Epidemiology
https://www.readbyqxmd.com/read/27608549/adaptive-immunity-in-fibrosarcomatous-dermatofibrosarcoma-protuberans-and-response-to-imatinib-treatment
#15
Marcella Tazzari, Valentina Indio, Barbara Vergani, Loris De Cecco, Francesca Rini, Tiziana Negri, Chiara Camisaschi, Marco Fiore, Silvia Stacchiotti, G Paolo Dagrada, Paolo G Casali, Alessandro Gronchi, Annalisa Astolfi, Maria A Pantaleo, Antonello Villa, Claudia Lombardo, Flavio Arienti, Silvana Pilotti, Licia Rivoltini, Chiara Castelli
Dermatofibrosarcoma protuberans (DFSP), although rare, is the most frequent skin sarcoma. Here, we focus on DFSP carrying the fibrosarcomatous transformation (FS-DFSP). FS-DFSP responds to imatinib (IM); however, tumor relapse often occurs. In a series of 21 pre- and post-treatment FS-DFSP samples, the present study explored the events that occur at the tumor site during IM therapy. Gene expression profile and immunohistochemistry analyses documented the occurrence of IM-induced senescence phenotype in the tumor cells and showed the accumulation of activated CD3(+) T cells and CD163(+)CD14(+) myeloid cells expressing the CD209 marker in post-therapy lesions...
September 5, 2016: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27591501/dermatofibrosarcoma-protuberans-wide-local-excision-versus-mohs-micrographic-surgery
#16
REVIEW
John T Mullen
Dermatofibrosarcoma protuberans (DFSP) is a rare dermal soft tissue sarcoma characterized by a typically indolent clinical course. The greatest clinical challenge in management of DFSP is achieving local control. There is vigorous debate in the literature as to the optimal surgical approach to these tumors. The choice between wide local excision and Mohs micrographic surgery for DFSP should be governed by the attainment of three goals: (1) to completely excise the tumor with negative margins, tantamount to cure; (2) to preserve function, optimize cosmesis, and minimize morbidity of resection; and (3) to minimize cost and inconvenience to the patient and the health care system at large...
October 2016: Surgical Oncology Clinics of North America
https://www.readbyqxmd.com/read/27580559/effectiveness-of-imatinib-mesylate-treatment-in-a-patient-with-dermatofibrosarcoma-protuberans-with-pulmonary-and-pancreatic-metastases
#17
Toshiharu Tatai, Daisuke Gomi, Toshirou Fukushima, Takashi Kobayashi, Nodoka Sekiguchi, Akiyuki Sakamoto, Shigeru Sasaki, Tomonobu Koizumi, Kenji Sano
We herein encountered a case of abdominal wall dermatofibrosarcoma protuberans (DFSP) that developed pulmonary and pancreatic metastases 5 years after complete resection. Because specific rearrangements of the platelet-derived growth factor beta (PDGFB) locus by a novel fluorescence in situ hybridization method was detected, the patient was treated with imatinib mesylate at 400 mg/day. A partial response was achieved by imatinib without any specific toxicity. Although metastatic DFSP is an extremely rare disease, an evaluation of PDGFB fusion is essential and imatinib mesylate should be considered as an optimal therapeutic choice in patients with metastatic or locally advanced DFSP...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27572421/improving-precision-of-resection-by-pre-surgery-inspections-with-contrast-enhanced-ultrasound-for-dermatofibrosarcoma-protuberans
#18
Chuan Ma, Yang Sun, Xin Yang, Qian Zhang, Chunlei Zhang, Ligang Cui
Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon malignant soft tissue tumor. Although metastasis is rare, it has significant topo-aggressive characteristics with a high recurrence rate. The common treatment of DFSP is surgical resection. This study reported a case of a 22 years old woman who presented DFSP with single irregular red plaque in the right occipital area. The plaque is about 2 cm × 2 cm in diameter with an irregular and unclear border. In order to minimize the range of resection area, the contrast-enhanced ultrasound (CEUS) was used to precisely identify the tumor affected area before the surgery treatment...
November 2016: Dermatologic Therapy
https://www.readbyqxmd.com/read/27542641/dermatofibrosarcoma-protuberans
#19
REVIEW
Jeffrey Reha, Steven C Katz
Dermatofibrosarcoma protuberans (DFSP) is a rare superficial soft tissue sarcoma. Its rarity precludes large prospective studies. Clinical diagnosis requires an high index of suspicion. Effective management requires an appreciation of tumor biology and the nature of the characteristic infiltrative growth pattern. DFSP tends to recur locally, with a low risk of dissemination. Aggressive surgical resection with widely negative margins is essential to management. Radiotherapy may be indicated in special circumstances...
October 2016: Surgical Clinics of North America
https://www.readbyqxmd.com/read/27542587/hormone-receptor-expression-in-patients-with-dermatofibrosarcoma-protuberans
#20
Kathryn L Kreicher, Kord S Honda, David E Kurlander, Jeremy S Bordeaux
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which the exact etiology is unknown. Case reports exist of DFSP appearing and growing rapidly during pregnancy, suggesting a hormonal role. OBJECTIVE: Our goal was to determine the expression of estrogen receptors (ERs) and progesterone receptors (PRs) in patients with DFSP. METHODS: Archived formalin-fixed, paraffin-embedded tissue from patients with DFSP in the past 20 years at a single institution were analyzed for ER and PR using immunohistochemistry...
December 2016: Journal of the American Academy of Dermatology
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