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Dermatofibrosarcoma protuberans

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https://www.readbyqxmd.com/read/29792909/dermatofibrosarcoma-protuberans-a-retrospective-study-of-clinicopathological-features-and-related-akt-mtor-stat3-erk-cyclin-d1-and-pd-l1-expression
#1
Sunyoung Park, Soyun Cho, Minji Kim, Ji Ung Park, Eui Cheol Jeong, Euno Choi, Jeong Hwan Park, Cheol Lee, Mee Soo Chang
BACKGROUND: Little is known regarding oncoproteins other than PDGFβ in dermatofibrosarcoma protuberance (DFSP). Moreover, the risk factors for worse prognosis are controversial. OBJECTIVE: We sought to determine the clinicopathological features and key factors for adverse outcome in DFSP including the implication of Akt/mTOR, STAT3, ERK, cyclinD1, and PD-L1 expression. METHODS: Clinicopathological and immunohistochemical analyses were performed for 44 DFSPs having wide local excision along with 92 dermatofibromas as controls...
May 21, 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29773427/microphthalmia-associated-transcription-factor-mitf-promiscuous-staining-patterns-in-fibrohistiocytic-lesions-is-a-potential-pitfall
#2
Sambit K Mohanty, Shivani Sharma, Dinesh Pradhan, Shivani R Kandukuri, Navid Farahani, Catherine Barry, Julie M Wu, David Frishberg, Bonnie Balzer
Microphthalmia-associated transcription factor (MiTF) is used as a marker of melanocytic differentiation. However, MiTF immunoexpression has also been observed in histiocytes, macrophages, smooth muscle cells and fibroblasts, which raise the concern of fibrohistiocytic (FH) lesions being misdiagnosed as melanoma based on MiTF immunoreactivity. MiTF has been known to be positive in FH tumors, but this is the first study evaluating ninety-three fibrohistiocytic neoplasms to understand and delineate the staining pattern of MiTF in these tumors...
May 11, 2018: Pathology, Research and Practice
https://www.readbyqxmd.com/read/29760553/a-favorable-outcome-of-advanced-dermatofibrosarcoma-protuberans-under-treatment-with-sunitinib-after-imatinib-failure
#3
Wei Xiao, Yi Que, Ruiqing Peng, Ya Ding, Jingjing Zhao, Xizhi Wen, Desheng Weng, Xiaoshi Zhang, Yuanxiang Guan, Xing Zhang
While traditional cytotoxic agents play a limited role in advanced dermatofibrosarcoma protuberans (DFSP), the treatment of sunitinib for patients with advanced DFSP after imatinib failure is not well defined. The objective of this case report was to analyze the relationship between molecular mechanisms and clinical outcomes of sunitinib treatment in patients with advanced DFSP after imatinib failure. In this case report, a 37-year-old man suffered from advanced DFSP progression after surgical operation, microwave ablation, and chemotherapy...
2018: OncoTargets and Therapy
https://www.readbyqxmd.com/read/29723383/an-unusual-variant-of-atrophic-dermatofibrosarcoma-protuberans
#4
Simone Silva da Costa Aragão, Everson José Dos Santos Leite, Alberto Eduardo Cox Cardoso, Ricardo Luiz Simões Houly
Dermatofibrosarcoma protuberans is an uncommon neoplasm that is most often seen in young adults. The most common clinical presentation is the protruding form; however, other subtypes are known, such as the atrophic. In 2012 there were only 33 reports of this variant in the literature. Many cases of Dermatofibrosarcoma protuberans in children are only discovered in adulthood because they were not diagnosed early. Due the high morbidity, we raise the need for attention from the dermatologist to recognize uncommon neoplasms in the clinical practice...
March 2018: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29682829/expression-of-aurora-kinase-a-expression-in-dermatofibrosarcoma-protuberans
#5
Sanayo Kanamori, Ikko Kajihara, Saori Kanazawa-Yamada, Saki Otsuka-Maeda, Hironobu Ihn
No abstract text is available yet for this article.
April 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29607758/nonepithelial-tumors-and-tumor-like-lesions-of-the-skin-and-subcutis-in-children
#6
Louis P Dehner, Alejandro A Gru
This overview of mesenchymal tumors presenting in the skin and/or subcutis in children brings together the range of neoplasms and hamartomas which are seen in this age-group. It is not surprising from the perspective of the pediatric or general surgical pathologist that vascular anomalies, including true neoplasms and vascular malformations, are the common phenotypic category. Since there is considerable morphologic overlap among these lesions, clinicopathologic correlation may be more important than for many of the other mesenchymal tumors...
March 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29606823/recurrent-dermatofibrosarcoma-protuberans-of-scalp-in-a-distant-location-10-years-after-primary-excision
#7
Chang Min Kim, Tae Jun Park, Bo Young Kim, Seung Hyun Chun, Il-Hwan Kim
Dermatofibrosarcoma protuberans (DFSP) is a slow growing low-grade cutaneous sarcoma. Local recurrence after excision is common due to the poorly defined periphery that renders histological control of surgical margin difficult, Mohs micrographic surgery is the optimal method for treatment. A 41 years old male patient, who had a previous history of DFSP, came to our dermatology clinic for evaluation of an asymptomatic firm flesh-colored nodule on the forehead. Total excision biopsy was done and the mass was histologically proved as DFSP...
April 2018: Annals of Dermatology
https://www.readbyqxmd.com/read/29531869/a-rare-case-of-dermatofibrosarcoma-protuberans-of-the-thumb-in-an-80-year-old-woman
#8
Georgi P Georgiev, Svetoslav A Slavchev, Julian Ananiev
Dermatofibrosarcoma protuberans (DFSP) has been described as a rare, locally invasive, malignant fibroblastic tumor with a high rate of recurrence that usually affects middle-aged patients. Herein, we describe a rare case of a pedunculated dermatofibrosarcoma protuberans of the right thumb in an 80-year-old woman treated with excision through the base of the pedicle. We also make a brief literature review concerning this tumor.
January 2, 2018: Curēus
https://www.readbyqxmd.com/read/29517116/dermatofibrosarcoma-protuberans-a-rare-lesion-on-breast
#9
Anuradha Bishnoi, Dipankar De, Davinder Parsad, Uma N Saikia
No abstract text is available yet for this article.
March 8, 2018: Breast Journal
https://www.readbyqxmd.com/read/29510612/pten-mutation-identified-in-patient-diagnosed-with-simultaneous-multiple-cancers
#10
Hye Sung Won, Eun Deok Chang, Sae Jung Na, In Yong Whang, Dong Soo Lee, Sun Hyong You, Yong Seok Kim, Jeong Soo Kim
PTEN hamartoma tumor syndrome is a spectrum of disorders characterized by unique phenotypic features including multiple hamartomas caused by mutations of the tumor suppressor gene PTEN. Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome are representative diseases, and both have several common clinical features and differences. Because PTEN mutations are associated with an increased risk of malignancy including breast, thyroid, endometrial, and renal cancers, cancer surveillance is an important element of disease management...
February 27, 2018: Cancer Research and Treatment: Official Journal of Korean Cancer Association
https://www.readbyqxmd.com/read/29509956/relapse-in-dermatofibrosarcoma-protuberans-a-histological-and-molecular-analysis
#11
André S Molina, João P Duprat Neto, Eduardo Bertolli, Isabela W da Cunha, José H T G Fregnani, Pedro H M Figueiredo, Fernando A Soares, Mariana P Macedo, Clovis A Pinto Lopes, Ivan D de Abranches Oliveira Santos Filho
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare low grade tumor with a locally aggressive behavior and low metastatic potential. OBJECTIVES: To evaluate the factors that are associated with relapse in DFSP. Methods Retrospective analysis of medical records from 61 patients with dermatofibrosarcoma. Fluorescence in situ hybridization was used to detect translocations. RESULTS: Of 61 patients, 6 experienced a relapse. No patient with resection margins greater than 3 cm had a recurrence...
March 6, 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29492209/egfr-is-involved-in-dermatofibrosarcoma-protuberans-progression-to-high-grade-sarcoma
#12
Amélie Osio, Shuo Xu, Morad El Bouchtaoui, Christophe Leboeuf, Guillaume Gapihan, Christine Lemaignan, Guilhem Bousquet, Céleste Lebbé, Anne Janin, Maxime Battistella
Dermatofibrosarcoma protuberans (DFSP), amounting to 6% of all soft tissue sarcomas, has a slow growth rate, contrasting with a likelihood for local recurrence and a 10-20% evolution to higher-grade sarcoma, or "transformed DFSP" (DFSP-T). At molecular level, the characteristic COL1A1-PDGFB rearrangement, leading to sustained PDGFR signaling, is not linked to the evolutive potential. Here, we studied EGFR, another tyrosine kinase receptor, using laser-microdissection to select the different histologic components of DFSP (DFSP center, DFSP infiltrative periphery, DFSP-T higher-grade sarcoma), in 22 patients followed over 3 to 156 months...
February 2, 2018: Oncotarget
https://www.readbyqxmd.com/read/29483976/dermatofibrosarcoma-protuberans-retrospective-single-center-analysis-over-16-years
#13
Uwe Wollina, Dana Langner, Jacqueline Schönlebe, Katlein França, Torello Lotti, Georgi Tchernev
Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and Allergology between 08/2001 and 08/2017. The diagnosis had been confirmed by histology and immunohistology in all cases. We identified four adults and a pediatric patient with DFSP. All patients were treated by wide surgical excision and controlled by three-dimensional histologic margin control...
January 25, 2018: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29474191/a-unique-case-of-dermatofibrosarcoma-protuberans-with-melanocytic-differentiation
#14
Alejandro A Gru, Mark R Wick, Hongyan Dai
No abstract text is available yet for this article.
February 13, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29468850/an-unusual-clinical-presentation-of-myxoid-dermatofibrosarcoma-protuberans-with-a-prominent-vasculature-a-potential-pitfall-in-the-diagnosis-of-myxoid-soft-tissue-tumors
#15
Patrick Tran, Gregory P Henderson, Michael McLemore
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor that arises primarily on the trunk and extremities but seldom on the scalp. Several variants of DFSP have been described, including myxoid DFSP. Although typical DFSP may have focally myxoid areas, myxoid DFSP, in which most of the stroma is myxoid, is rare and can pose diagnostic challenges. Here, we report a case of myxoid DFSP with an unusual clinical presentation that could have been mistaken for a lipoma. Additionally, the myxoid DFSP displayed prominent vasculature in a myxoid stroma, which could have been mistaken for a myxoid liposarcoma...
February 22, 2018: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/29416224/a-rare-case-of-misdiagnosis-recurrence-of-dermatofibrosarcoma-protuberans-that-was-treated-surgicallyas-a-keloid
#16
Murat Ucak
Aim: In this report, we presented the patient with Dermatofibrosarcoma Protuberans (DFSP), removed by considering as the keloid scar in the general surgery clinic with misdiagnosis. Case report: The patient was a 19-year-old female student with no scar or previous trauma history in the lesion area. Pathology report of excisional biopsy revealed as a DFSP, reachedsubcutis and dermis. In staging by CT, there had been no distant metastases. There was a lesion with the size of 2x1...
February 2018: Medical Archives
https://www.readbyqxmd.com/read/29413661/differential-diagnosis-of-benign-spindle-cell-lesions
#17
REVIEW
Gaetano Magro
Spindle cell lesions of the breast cover a wide spectrum of diseases ranging from reactive tumor-like lesions to high-grade malignant tumors. The recognition of the benign spindle cell tumor-like lesions (nodular fasciitis; reactive spindle cell nodule after biopsy, inflammatory pseudotumor/inflammatory myofibroblastic tumor; fascicular variant of pseudoangiomatous stromal hyperplasia) and tumors (myofibroblastoma, benign fibroblastic spindle cell tumor, leiomyoma, schwannoma, spindle cell lipoma, solitary fibrous tumor, myxoma) is crucial to avoid confusion with morphologically similar but more aggressive bland-appearing spindle cell tumors, such as desmoid-type fibromatosis, low-grade (fibromatosis-like) spindle cell carcinoma, low-grade fibrosarcoma/myofibroblastic sarcoma and dermatofibrosarcoma protuberans...
March 2018: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29408302/the-role-of-small-molecule-platelet-derived-growth-factor-receptor-pdgfr-inhibitors-in-the-treatment-of-neoplastic-disorders
#18
REVIEW
Robert Roskoski
Platelet-derived growth factor (PDGF) was discovered as a serum-derived component necessary for the growth of smooth muscle cells, fibroblasts, and glial cells. The PDGF family is a product of four gene products and consists of five dimeric isoforms: PDGF-AA, PDGF-BB, PDGF-CC, PDGF-DD, and the PDGF-AB heterodimer. This growth factor family plays an essential role in embryonic development and in wound healing in the adult. These growth factors mediate their effects by binding to and activating their receptor protein-tyrosine kinases, which are encoded by two genes: PDGFRA and PDGFRB...
March 2018: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/29387771/dermatofibrosarcoma-protuberans-in-pediatric-patients-a-diagnostic-and-management-challenge
#19
Anna H Buteau, Brett H Keeling, Lucia Z Diaz, Margarita Larralade, Paula Luna, Chandra Krishnan, Moise L Levy
No abstract text is available yet for this article.
March 2018: JAAD Case Reports
https://www.readbyqxmd.com/read/29377717/impact-of-pathologist-involvement-in-sarcoma-and-rare-tumor-patient-support-groups-on-facebook-a-survey-of-542-patients-and-family-members
#20
Jasmine Haller, Marjorie Parker David, Nathan E Lee, Sara C Shalin, Jerad M Gardner
CONTEXT: - Patients with rare tumors have difficulty finding reliable information about their disease. Facebook patient support groups allow patients to educate one another. OBJECTIVE: - To investigate how these patients perceive the value of pathologists, both in Facebook groups and real-world patient care. DESIGN: - Survey links were posted in 12 Facebook patient groups: 6 with an active pathologist member (angiosarcoma, epithelioid hemangioendothelioma, epithelioid sarcoma, dermatofibrosarcoma protuberans [×2], and desmoid fibromatosis), and 6 without "active" pathologist involvement (aggressive angiomyxoma, chondrosarcoma, Ewing sarcoma, leiomyosarcoma, liposarcoma, and osteosarcoma)...
January 29, 2018: Archives of Pathology & Laboratory Medicine
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