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https://www.readbyqxmd.com/read/28632950/fibroblastic-connective-tissue-nevus-clinicopathological-and-immunohistochemical-study-of-14-cases
#1
Ilaria Pennacchia, Heinz Kutzner, Dmitry V Kazakov, Thomas Mentzel
BACKGROUND: We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labelled as fibroblastic connective tissue nevus (FCTN). METHODS AND RESULTS: Eight patients were male and five were female, ranging in age from 1 to 56 years. Lesions appeared as isolated nodules or plaques on the trunk (7 cases), the limbs (4 cases) and the neck (2 cases). Histologically, all cases were composed of bundles of bland spindle cells of fibroblastic/myofibroblastic lineage irregularly branching within the reticular dermis and along fibrous septa in the subcutis...
June 20, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28627712/acral-dermatofibrosarcoma-protuberans-with-myoid-differentiation-a-report-of-two-cases
#2
Tariq Al-Zaid, Hatim Khoja
Dermatofibrosarcoma protuberans (DFSP) is a low-grade mesenchymal neoplasm with a tendency for local recurrence; it is rarely metastatic. Dermatofibrosarcoma protuberans typically arises in the trunk and proximal extremities. However, it can arise in unusual sites such as the hands and feet. Several variants have been described, including DFSP with myoid differentiation, which is very uncommon. This variant tends to be observed more with the fibrosarcomatous variant, and it can be either in the form of myoid bundles and nodules with no vascular relationship or represent hyperplastic blood vessels within the tumor...
June 19, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28609344/phosphohistone-h3-and-ki67-useful-markers-in-differentiating-dermatofibroma-from-dermatofibrosarcoma-protuberans-and-atypical-fibrohistiocytic-lesions
#3
Atin Agarwal, Arun Gopinath, Michael T Tetzlaff, Victor G Prieto
Dermatofibromas (DF) are common, benign, skin tumors, usually easily differentiated from dermatofibrosarcoma protuberans (DFSP) by the presence of a relative low cellularity, lesser degree of infiltration of subcutaneous tissue, and immunohistochemical pattern (eg, FXIIIa in DF and CD34 in DFSP). Atypical fibrohistiocytic lesions (AFL) have features intermediate to DF and DFSP (trunk location, storiform pattern, infiltration of the subcutaneous tissue, and focal expression of both CD34 and Factor XIIIa). It is unclear if mitotic counts/degree of proliferation is helpful to distinguish DF from DFSP...
July 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28607816/recurrence-case-of-rare-scalp-dermatofibrosarcoma-protuberans-two-case-reports-of-a-wide-radical-excision-craniectomy-bone-involvement-followed-by-cranioplasty-and-reconstruction
#4
Ahmad Faried, Wargian Hadisaputra, Muhammad Z Arifin
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade sarcoma of the fibroblast originating from the dermal layer of the skin, characterized by a locally aggressive growth and high rate of local recurrence. CASE DESCRIPTION: Two patients underwent a wide radical excision of recurrent scalp DFSP which was reconstructed with translational skin flap and split-thickness skin graft. We described above cases several years ago with a local excision of the tumor; recently, they developed local recurrence of DFSP with calvarial involvement...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28607608/clinical-features-pathological-findings-and-treatment-of-recurrent-dermatofibrosarcoma-protuberans
#5
Yanan Li, Chuan Wang, Bo Xiang, Siyuan Chen, Li Li, Yi Ji
Dermatofibrosarcoma protuberans (DESP) is a relatively rare and low-grade tumor of the skin and soft tissue. We review the clinical features, pathological findings and management of recurrent DESP with the aim to improve our understanding of this rare tumor. Fifty-seven patients were diagnosed with recurrent DFSP. The mean age at the presentation of DFSP was 30.9 years. The mean age at diagnosis of DFSP was 40.2 years. Chest wall was the dominant location. The histopathological diagnosis was ordinary DFSP (O-DFSP) in 46 cases and fibrosarcomatous DFSP (FS-DFSP) in 11 cases...
2017: Journal of Cancer
https://www.readbyqxmd.com/read/28584757/dermatofibrosarcoma-protuberans-in-a-9-year-old-child
#6
Priyanka Patil, Swagata Tambe, Chitra Nayak, C Ramya
Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm of low-to-intermediate grade malignant potential. Childhood onset of DFSP is rare. It is most commonly seen on the trunk and proximal extremities. In children, a high index of suspicion is necessary to avoid delays in diagnosis that can lead to further morbidity. Here, we report a case of DFSP in a 9-year-old female child. Excision biopsy of lesion was performed with 1 cm margin. After confirmation of the diagnosis by histopathology, the patient was observed for recurrence, but there was no recurrence after 1 and half years of follow up...
May 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/28551623/the-immunological-roles-of-periostin-tumor-associated-macrophage-axis-in-development-of-dermatofibrosarcoma-protuberans
#7
Taku Fujimura, Aya Kakizaki, Yota Sato, Kayo Tanita, Sadanori Furudate, Setsuya Aiba
BACKGROUND/AIM: Dermatofibrosarcoma protuberance (DFSP) is a fibrohistiocytic tumor of intermediate malignancy characterized by slow infiltrative growth and a high tendency to recur locally. Periostin is involved in modulating cell function and inducing the production of proinflammatory cytokines, chemokines, and matrix metalloproteinases (MMPs) from tumor-associated macrophages (TAMs) to promote fibrosis and tumor growth. This study aimed to examine the cancer stroma of DFSP, focusing on TAMs-related proteins and MMPs...
June 2017: Anticancer Research
https://www.readbyqxmd.com/read/28549894/diagnosis-and-management-of-dermatofibrosarcoma-protuberans-in-a%C3%A2-3-year-old-patient
#8
Rajiv Iyengar, Elizabeth Kiwanuka, Shamlal Mangray, Antonio P Cruz, Julia Katarincic, Reena Bhatt
In this case report, we present a 3-year-old boy with a diagnosis of dermatofibrosarcoma protuberans (DFSP) on the dorsum of his right hand. Although rarely metastatic, DFSP is highly locally invasive and can cause considerable local morbidity. In the hand, DFSP is uncommon in the young pediatric patient. In our patient, the tumor extended down to the second and third metacarpal heads and was treated with a multistaged excision of his tumor to achieve negative margins under slow Mohs micrographic surgery.
May 24, 2017: Journal of Hand Surgery
https://www.readbyqxmd.com/read/28546720/dermatofibrosarcoma-protruberans-of-the-breast-skin-simulating-mammary-carcinoma
#9
Prakriti Shukla, Hanni Vasudev Gulwani
Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma that arises from the dermis and invades deeper tissues. Usually, it behaves as an intermediate grade malignancy but in rare instances, it can metastasize. Frequent occurrences have been observed in trunk and extremities but involvement of the breast has rarely been reported. Therefore, dermatofibrosarcoma protuberans (DFSP) breast often masquerades primary breast malignancy on clinical and radiological grounds. Histomorphology and immunohistochemistry are helpful in making a definitive diagnosis...
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28544597/metastasis-of-soft-tissue-sarcomas-in-lymph-node-a-cytomorphological-study
#10
Jahnavi Gandhi, Shailee Mehta, Trupti Patel, Amisha Gami, Majal Shah, Dhaval Jetly
BACKGROUND: Soft-tissue sarcoma (STS) rarely metastasizes to lymph node compared to carcinoma. Fine-needle aspiration cytology (FNAC) carries a pivotal role in diagnosis of metastatic tumor to lymph node. This study highlights the role of FNAC in diagnosis of STS metastasis to lymph node. METHOD: A retrospective study over a period by 4 years carried out. FNAC of enlarged lymph node was performed in patients with STS. Cytology smears were examined in conjunction with clinical details...
May 24, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28528910/the-management-of-soft-tissue-tumours-of-the-abdominal-wall
#11
REVIEW
H G Smith, D Tzanis, C Messiou, C Benson, J A van der Hage, M Fiore, S Bonvalot, A J Hayes
BACKGROUND: Soft tissue tumours of the abdominal wall account for approximately 10% of all soft tissue tumours. Tumours at this site comprise a heterogeneous group of pathologies with distinct clinical behaviours and responses to treatment. The management of these tumours has largely been extrapolated from studies of soft tissue tumours at other sites. This review aims to summarise the existing data relating to abdominal wall tumours and suggest principles for managing soft tissue tumours at this site...
May 6, 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28515919/programmed-death-ligand-1-expression-is-associated-with-fibrosarcomatous-transformation-of-dermatofibrosarcoma-protuberans
#12
Kenji Tsuchihashi, Hitoshi Kusaba, Yuichi Yamada, Yuta Okumura, Hozumi Shimokawa, Masato Komoda, Keita Uchino, Tomoyasu Yoshihiro, Nobuhiro Tsuruta, Fumiyasu Hanamura, Kyoko Inadomi, Mamoru Ito, Kosuke Sagara, Michitaka Nakano, Kenta Nio, Shuji Arita, Hiroshi Ariyama, Kenichi Kohashi, Ryuji Tominaga, Yoshinao Oda, Koichi Akashi, Eishi Baba
Dermatofibrosarcoma protuberans (DFSP) is a locally invading tumor, characterized by the presence of the collagen type I α 1 (COL1A1)-platelet-derived growth factor (PDGF) β fusion gene. We herein report the case of a 31-year-old man with a history of resection of an abdominal wall DFSP. The patient presented with chest pain and a computed tomography scan revealed a large mass in the posterior mediastinum and another mass in the right lung. The mediastinal mass was a sarcomatous lesion expressing the COL1A1-PDGFβ fusion gene, suggesting that it represented a metastasis of the DFSP following fibrosarcomatous (FS) transformation...
May 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28442848/dermatofibrosarcoma-protuberance-of-the-breast-a-diagnostic-challenge
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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...
June 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
#20
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
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