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Dermatofibrosarcoma protuberans

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https://www.readbyqxmd.com/read/28442848/dermatofibrosarcoma-protuberance-of-the-breast-a-diagnostic-challenge
#1
Ismail A S Burud, Ng Sze How, Gan CheeWei, Suboh Roslina
Dermatofibrosarcoma protuberans (DFSP) is an uncommon slow growing neoplasm of the dermis with tendency to invade the subcutaneous tissues. It presents during the third to fourth decade of life and is commonly seen over the trunk, extremities and head and neck. DFSP presenting as a breast lump is rare but few cases have been reported in the literature. Pre-operative diagnosis with mammography, ultrasonography and FNAC is challenging. We report a case of a DFSP of the right breast in a middle aged lady with history of recurrent breast lumps excised and diagnosed in the past as benign...
April 2017: Indian Journal of Surgery
https://www.readbyqxmd.com/read/28421972/-a-tumour-mistaken-for-a-scar
#2
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
#3
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/28414587/non-melanoma-skin-cancer-new-and-future-synthetic-drug-treatments
#4
Teresa Amaral, Claus Garbe
Non-melanoma skin cancers (NMSC) mainly comprise two different entities: basal cell carcinoma (BCC) and squamous cell carcinoma (SCC); beneath these two entities, Merkel cell carcinoma, adnexal tumors, dermatofibrosarcoma protuberans, angiosarcoma, and cutaneous lymphoma belong to NMSC. These rare skin tumors are not the topic of this review. BCC and SCC are the most common cancers diagnosed in humans. The preferred treatment is surgery, which in most cases is curative. Although a high recurrence rate is seen, these cancers rarely metastasize...
May 2017: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/28388964/breast-and-pleuropulmonary-metastasis-of-multirecurrent-scalp-dermatofibrosarcoma-protuberans-a-case-report
#5
N-A Ouédraogo Nde, N A Ouédraogo, H A Bambara, B M A Tiemtoré-Kambou, F Traoré, N Zongo, M S Ouédraogo, G P Tapsoba, Achach Thouraya Chtioui, A Lamien/Sanou
BACKGROUND: Dermatofibrosarcoma protuberans is a rare tumor, representing about 0.1% of skin malignant tumors. It is characterized by local aggressiveness with significant potential for recurrence. Although metastasis is rare, it does occur. We report a case of a Burkinabe woman with dermatofibrosarcoma protuberans. CASE PRESENTATION: A 27-year-old Burkinabe woman consulted our institution for a recurrent scalp nodule that had been evolving for 13 years. At clinical examination, she was in good condition with a dry cough...
April 8, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28365129/long-term-results-of-treatment-of-advanced-dermatofibrosarcoma-protuberans-dfsp-with-imatinib-mesylate-the-impact-of-fibrosarcomatous-transformation
#6
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
#7
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/28346254/toluidine-blue-stain-of-dermatofibrosarcoma-protuberans-highlighting-its-use-in-mohs
#8
Omeed Ahadiat, Shauna Higgins, Alexandre Ly, Ashley Wysong
No abstract text is available yet for this article.
March 23, 2017: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/28329585/report-of-rare-case-of-dermatofibrosarcoma-protuberans-in-the-buccal-mucosa-review-of-diagnostic-histopathological-and-immunohistochemical-criteria
#9
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
#10
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/28291056/perspectives-on-low-grade-sarcomas-the-extraordinary-contributions-of-sharon-w-weiss-md
#11
Hope Hastings, John R Goldblum
Soft-tissue pathology encompasses a wide spectrum of neoplasms that represent some of the most challenging and problematic tumors in surgical pathology. Owing to the intensive work of dedicated pathologists, this once esoteric field has become increasingly well defined. In this review, Dr Sharon Weiss' monumental contributions to low-grade sarcomas, including low-grade fibromyxoid sarcoma/so-called hyalinizing spindle cell tumor, atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma, epithelioid hemangioendothelioma, and dermatofibrosarcoma protuberans with fibrosarcomatous transformation will be discussed...
March 13, 2017: Advances in Anatomic Pathology
https://www.readbyqxmd.com/read/28270311/when-immunohistochemistry-deceives-us-the-pitfalls-of-cd34-and-factor-xiiia-stains-in-dermatofibroma-and-dermatofibrosarcoma-protuberans
#12
Ann M John, Heather H Holahan, Parmvir Singh, Marc Z Handler, W Clark Lambert
No abstract text is available yet for this article.
2017: Skinmed
https://www.readbyqxmd.com/read/28191686/dermoscopy-of-uncommon-variants-of-dermatofibrosarcoma-protuberans
#13
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/28154469/operative-management-of-dermatofibrosarcoma-protuberans-of-the-breast
#14
(no author information available yet)
No abstract text is available yet for this article.
January 2017: Proceedings of the Baylor University Medical Center
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
#15
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
#16
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/28110710/imatinib-in-dermatofibrosarcoma-targeted-therapy-or-immunotherapy
#17
Selma Ugurel, Jürgen C Becker
There is increasing evidence that certain kinase inhibitors are able to foster two strategies, i.e. inhibition of oncogenic activated molecular pathways and modulation of immunological processes. In this respect, the study of Tazzari et al. is of great interest because it shows both effects for the kinase inhibitor imatinib in dermatofibrosarcoma protuberans.
February 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28108880/unusual-signal-patterns-of-break-apart-fish-probes-used-in-the-diagnosis-of-soft-tissue-sarcomas
#18
Gergő Papp, Dóra Mihály, Zoltán Sápi
Break-apart FISH probes are the most popular and reliable type of FISH probes used to confirm certain pathological diagnoses. The interpretation is usually easy, however, in some instances it is not so unequivocal. Our aim was to reveal and elucidate the problems occurring in the process of evaluation of the break-apart probe results. Altogether 301 soft tissue sarcomas with confirmed molecular tests using break-apart probes were assessed to reveal the frequency and type of unusual signal pattern. Among 89 synovial sarcoma (SS18) 11%, 12 alveolar rhabdomyosarcoma (FOXO1) 50%, 53 myxoid liposarcoma (DDIT3) 7...
January 20, 2017: Pathology Oncology Research: POR
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
#19
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/28045748/superficial-acral-fibromyxoma-report-of-13-cases-with-new-immunohistochemical-findings
#20
MULTICENTER STUDY
Daniella Cullen, José Luis Díaz Recuero, Roberto Cullen, José Luis Rodríguez Peralto, Heinz Kutzner, Luis Requena
BACKGROUND: Superficial acral fibromyxoma (SAF) is a benign, soft tissue neoplasm preferably located on the digits. METHODS: We collected 13 cases of SAF and evaluated their clinical, histopathologic, and immunohistochemical features. RESULTS: This study included 9 males and 4 females, median age 54 years. The patients presented with a solitary asymptomatic or tender mass, most of them arising on fingers or toes. Histopathologically all lesions consisted of nonencapsulated dermal nodules, composed of spindled cells with variable myxoid and/or fibrotic stroma...
January 2017: American Journal of Dermatopathology
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