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Dermatofibrosarcoma

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https://www.readbyqxmd.com/read/28627712/acral-dermatofibrosarcoma-protuberans-with-myoid-differentiation-a-report-of-two-cases
#1
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/28622423/-congenital-dermatofibrosarcoma-protuberans-clinically-mimicking-a-melanocytic-nevus-treated-with-serial-excisions
#2
J Laske, M Sergon, T Mentzel, S Beissert, J Maschke
A 9-year-old female patient was referred to our department with the clinical diagnosis of a congenital melanocytic nevus on the back for serial excisions. The child's parents confirmed that the lesion existed since birth. Only little changes in color and size were observed over the years. The lesion was painless. This article is protected by copyright. All rights reserved.
June 16, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
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/28595245/massively-metastatic-dermatofibrosarcoma-protuberans-with-fibrosarcomatous-transformation-years-after-surgical-resection
#6
Teo Soleymani, Catherine Ni, Anna Laury, Jashin J Wu
No abstract text is available yet for this article.
June 6, 2017: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/28591457/cutaneous-sarcomas
#7
Judith Kohlmeyer, Susanne Annette Steimle-Grauer, Rüdiger Hein
Cutaneous sarcomas comprise a heterogeneous group of mesenchymal spindle cell tumors of the dermis and subcutis, one of the best-known entities being dermatofibrosarcoma protuberans. Other sarcomas addressed in this review include atypical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, leiomyosarcoma, liposarcoma, and angiosarcoma. With the exception of dermatofibrosarcoma protuberans, which has its peak incidence in middle-aged adults, cutaneous sarcomas usually occur in elderly individuals starting in the sixth or seventh decade of life...
June 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28591446/kutane-sarkome
#8
Judith Kohlmeyer, Susanne Annette Steimle-Grauer, Rüdiger Hein
Cutaneous sarcomas comprise a heterogeneous group of mesenchymal spindle cell tumors of the dermis and subcutis, one of the best-known entities being dermatofibrosarcoma protuberans. Other sarcomas addressed in this review include a typical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, leiomyosarcoma, liposarcoma, and angiosarcoma. With the exception of dermatofibrosarcoma protuberans, which has its peak incidence in middle-aged adults, cutaneous sarcomas usually occur in elderly individuals starting in the sixth or seventh decade of life...
June 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28584757/dermatofibrosarcoma-protuberans-in-a-9-year-old-child
#9
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/28573714/wide-local-excision-vs-mohs-t%C3%A3-bingen-technique-in-the-treatment-of-dermatofibrosarcoma-protuberans-a-two-centre-retrospective-study-and-literature-review
#10
F Veronese, P Boggio, R Tiberio, M Gattoni, P Fava, V Caliendo, E Colombo, P Savoia
BACKGROUND: Dermatofibrosarcoma Protuberans is a rare, low-grade mesenchymal skin tumour, characterized by slow infiltrative growth and common local recurrence, with infrequent distant metastases. OBJECTIVE: The aim of this study is to better clarify clinico-pathological characteristics of this tumour and to evaluate the cure rates of Mohs Tübingen technique (MTT) and wide local excision. Eventually, we perform a literature review to compare our experience with published data...
June 2, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28553740/extragastrointestinal-stromal-tumor-of-the-abdominal-subcutaneous-tissue-report-of-a-very-rare-case-at-an-unusual-site
#11
Xue He, Nannan Chen, Li Lin, Congyang Wang, Yan Wang
Extragastrointestinal stromal tumors (EGISTs) are rare tumors that arise outside the digestive tract. We report a case of an EGIST arising in the subcutaneous tissue of the abdominal wall, which at this site can often be misdiagnosed as dermatofibrosarcoma protuberans. The tumor was surgically resected from a 72-year-old male Chinese Han patient, and pathological examination revealed spindle-shaped tumor cells with eosinophilic cytoplasm and an oval nucleus. Immunohistochemically, the tumor cells showed strong cytoplasmic positivity for CD34, c-KIT (CD117), and DOG1...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28551623/the-immunological-roles-of-periostin-tumor-associated-macrophage-axis-in-development-of-dermatofibrosarcoma-protuberans
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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/28513108/dermatofibrosarcoma-protuberans-of-the-scalp-therapeutic-challenges
#18
Davendran Kanesen, Zaitun Zakaria, Ewe S Ch'ng, Puneet Nandrajog, Regunath Kandasamy
No abstract text is available yet for this article.
May 16, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28509521/dermatofibrosarcoma-protuberans-when-the-age-makes-the-difference
#19
Giovanni Paolino, Dario Didona, Ugo Bottoni, Federico Romaniello, Paola Corsetti, Antonio G Richetta
BACKGROUND: Dermatofibrosarcoma protuberans is a malignant tumor that affects exclusively the skin. It is a low- grade malignant tumor of subcutaneous tissues, characterized by a local recurrence but it seldom metastasizes. This study aims to evaluate the impact of different clinical parameters on disease free survival and overall survival of dermatofibrosarcoma protuberans patients. METHODS: A retrospective study of data including 17 cases of dermatofibrosarcoma protuberans (11 male, 6 female) retrieved from the files of the Dermatology Clinics of La Sapienza, University of Rome...
May 16, 2017: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/28493803/soft-tissue-sarcomas-of-the-abdomen-and-pelvis-radiologic-pathologic-features-part-2-uncommon-sarcomas
#20
Angela D Levy, Maria A Manning, Markku M Miettinen
Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies. Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification. GIST is the most common intraperitoneal sarcoma. Liposarcoma is the most common retroperitoneal sarcoma, and leiomyosarcoma is the second most common. GIST, liposarcoma, and leiomyosarcoma account for the majority of sarcomas encountered in the abdomen and pelvis and are discussed in part 1 of this article...
May 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
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