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https://www.readbyqxmd.com/read/28421972/-a-tumour-mistaken-for-a-scar
#1
A C van den Brand, A Erceg
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare type of cutaneous soft tissue sarcoma. The tumour metastasises rarely, but the percentage of recurrences is high and the tumour can be locally very aggressive. CASE DESCRIPTION: A 50-year-old man was referred to our clinic for a long existing scar on his back, which had become painful in the last few weeks. In the hospital we decided to take a biopsy. Based on the histopathological investigation, the diagnosis DFSP was made...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28420485/subcutaneous-dermatofibrosarcoma-protuberans-a-rare-subtype-with-predilection-for-the-head-a-retrospective-series-of-18-cases
#2
Beatriz Llombart, Carlos Serra-Guillén, Luis Rubio, Eduardo Nagore, Celia Requena, Victor Traves, Laura Calomarde, Bernardo Bancalari, José Antonio López-Guerrero, Carlos Guillen-Barona, Onofre Sanmartín
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) typically affects the dermis and subcutaneous tissue. The subcutaneous variant is rare. OBJECTIVE: We sought to characterize the subcutaneous DFSP (SC-DFSP) variant and compare it with cutaneous DFSP (C-DFSP). METHODS: This work was a retrospective study of DFSP treated in our institution. RESULTS: Of 124 cases of DFSP, 18 were SC-DFSP (14.5%). Except for the deep location, the pathologic and genetic features were indistinguishable from the C-DFSP variant...
April 15, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28365129/long-term-results-of-treatment-of-advanced-dermatofibrosarcoma-protuberans-dfsp-with-imatinib-mesylate-the-impact-of-fibrosarcomatous-transformation
#3
P Rutkowski, A Klimczak, I Ługowska, B Jagielska, M Wągrodzki, M Dębiec-Rychter, B Pieńkowska-Grela, T Świtaj
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is rare, infiltrating dermal neoplasm, characterized by indolent growth and low probability of metastases. The first effective systemic therapy in DFSP introduced into clinical practice was imatinib, demonstrating high activity in advanced cases. The aim of the study was to perform an analysis of patients with advanced DFSP treated with imatinib, with or without surgery, in routine clinical practice with long-term follow-up. PATIENTS AND METHODS: We analyzed the data of 31 Caucasian patients (14 male, 17 female; median age 56 years) with locally advanced/initially inoperable and/or metastatic DFSP who started therapy with imatinib at initial dose 800 mg daily between 12/2004 and 07/2014...
March 22, 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28362700/dermatofibrosarcoma-protuberans-an-immunomarker-study-of-57-cases-that-included-putative-mesenchymal-stem-cell-markers
#4
Joon Seon Song, Eun-Ju Kim, Chan-Sik Park, Kyung-Ja Cho
Dermatofibrosarcoma protuberans (DFSP) is a low-grade fibroblastic sarcoma with a superficial location that has been suggested to potentially be a type of mesenchymal stem cell tumor. We studied the expression of various immunomarkers, including putative stem cell markers, in a series of 57 DFSPs including variants, and 12 dermatofibromas (DFs). CD105, a mesenchymal stem cell marker, was weakly expressed in 24 DFSPs, whereas other stem cell markers, including CD133, ALK-1, and Oct3/4, were completely negative in all samples...
March 30, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28329585/report-of-rare-case-of-dermatofibrosarcoma-protuberans-in-the-buccal-mucosa-review-of-diagnostic-histopathological-and-immunohistochemical-criteria
#5
Amanda Katarinny Goes Gonzaga, Angélica Lopes Cordeiro Mandú, Alexandre Oliveira Sales, Ana Miryam Costa Medeiros, Rodrigo Rodrigues Rodrigues, Patrícia Teixeira Oliveira, Adriano Rocha Germano, Éricka Janine Dantas Silveira
Dermatofibrosarcoma protuberans (DFSP) is an uncommon intermediate-grade soft tissue sarcoma. The tumor is found preferentially on the trunk, whereas the head and neck region are affected in only 10% to 15% of cases. We report the case of a 44-year-old woman with a 5-month history of an asymptomatic, firm, yellow nodule in the mucosa of the right cheek measuring 2.5 cm. The clinical diagnosis was lipoma and an excisional biopsy was obtained. Histopathological analysis revealed a proliferation of spindle-shaped mesenchymal cells arranged in interlacing fascicles amidst fibrous stroma...
October 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28323651/clinical-features-and-treatment-of-dermatofibrosarcoma-protuberans-affecting-the-vulva-a-literature-review
#6
Austin H Nguyen, Shannon Q Detty, Maria I Gonzaga, Christopher Huerter
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous neoplasm with a low propensity for metastasis and a high rate of local recurrence. It typically presents as a dermal plaque or nodule on the trunk, limbs, or head and neck region. Vulvar DFSP has also been described, although it is less common. OBJECTIVE: To review the available literature and discuss the clinical course of DFSP affecting the vulva. MATERIALS AND METHODS: We reviewed the existing English-language literature on DFSP of the vulva with respect to clinical presentation, diagnosis, treatment, and outcome...
March 17, 2017: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/28191686/dermoscopy-of-uncommon-variants-of-dermatofibrosarcoma-protuberans
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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...
April 2017: Revue de Pneumologie Clinique
https://www.readbyqxmd.com/read/27922973/incidence-and-outcomes-of-dermatofibrosarcoma-protuberans-in-the-us-pediatric-population
#13
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
#14
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
#15
REVIEW
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
#16
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
#17
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
#18
COMPARATIVE STUDY
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
#19
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
#20
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
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