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https://www.readbyqxmd.com/read/29878374/hormone-receptors-analysis-in-chinese-patients-with-dermatofibrosarcoma-protuberans
#1
Tian Meng, Xiao-Hua Shi, Sha-Fei Wu, Yu-Feng Luo, Xiao-Jun Wang, Xiao Long
BACKGROUND AND OBJECTIVES: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare skin tumor. Clinical observations indicated that DFSP has a more aggressive behavior during pregnancy, which suggest there might be a hormonal influence on this tumor. We evaluated the expression of estrogen receptor (ER) and progesterone receptor (PR) in DFSP patients. METHODS: In our present case series, patients with histopathological-confirmed DFSP at a single institution were identified...
June 7, 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29863809/evaluation-of-pan-trk-immunohistochemistry-in-infantile-fibrosarcoma-lipofibromatosis-like-neural-tumor-and-histologic-mimics
#2
Yin P Hung, Christopher D M Fletcher, Jason L Hornick
AIMS: Infantile fibrosarcoma is characterized by intersecting fascicles of spindle cells and ETV6-NTRK3 gene fusion in most cases. Given histologic overlap with other spindle-cell tumors, the diagnosis can be challenging and often requires molecular confirmation. A recently developed pan-TRK antibody shows promise for identifying tumors with NTRK fusions. The purpose of this study was to evaluate the potential diagnostic utility of pan-TRK immunohistochemistry for infantile fibrosarcoma...
June 4, 2018: Histopathology
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
#3
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
#4
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
#5
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/29606823/recurrent-dermatofibrosarcoma-protuberans-of-scalp-in-a-distant-location-10-years-after-primary-excision
#6
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
#7
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/29509956/relapse-in-dermatofibrosarcoma-protuberans-a-histological-and-molecular-analysis
#8
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...
April 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29492209/egfr-is-involved-in-dermatofibrosarcoma-protuberans-progression-to-high-grade-sarcoma
#9
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
#10
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/29468850/an-unusual-clinical-presentation-of-myxoid-dermatofibrosarcoma-protuberans-with-a-prominent-vasculature-a-potential-pitfall-in-the-diagnosis-of-myxoid-soft-tissue-tumors
#11
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
#12
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/29408302/the-role-of-small-molecule-platelet-derived-growth-factor-receptor-pdgfr-inhibitors-in-the-treatment-of-neoplastic-disorders
#13
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/29309822/fluorescence-microscopy-for-the-evaluation-of-elastic-tissue-patterns-within-fibrous-proliferations-of-the-skin-on-hematoxylin-eosin-stained-slides
#14
Robert Borucki, David M Perry, Dan R Lopez-Garcia, Viktoryia Kazlouskaya, Dirk M Elston
BACKGROUND: Diagnosis of fibrous tumors can be challenging and expensive due to the use of special stains. OBJECTIVE: Determine the usefulness of fluorescence microscopy in the evaluation of elastic pattern on H&E stained slides. METHODS: A total of 228 slides were evaluated by fluorescence microscopy for elastic tissue patterns and sensitivity and specificity determined for relevant comparisons. RESULTS: Fluorescence microscopy was found to be useful especially in the case of distinguishing dermatofibroma (DF) vs dermatofibrosarcoma protuberans (DFSP) and also dermatomyofibroma (DMF) vs other fibrous tumors...
January 5, 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29240584/dermatofibrosarcoma-protuberans-of-distal-extremities-and-acral-sites-a-clinicopathologic-analysis-of-27-cases
#15
Kabeer K Shah, Jonathan B McHugh, Andrew L Folpe, Rajiv M Patel
Dermatofibrosarcoma protuberans (DFSP) of the distal extremities and acral sites are extremely rare and incompletely characterized. Twenty-seven DFSP occurring in these sites were retrieved from our collective archives and reevaluated. Tumors occurred in 16 males and 11 females. Median age at presentation was 42.5 years (range, 7 to 78 y). Lesions involved the foot (18 with 6 in the toes and 2 on the plantar foot), distal ankle (4), hand (4 with 2 in the thumbs), and wrist (1). All cases showed predominantly classic DFSP morphology and were diffusely CD34 positive...
March 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29175214/survival-in-patients-with-primary-dermatofibrosarcoma-protuberans-national-cancer-database-analysis
#16
Oleksandr Trofymenko, Jeremy S Bordeaux, Nathalie C Zeitouni
BACKGROUND: The predictors of mortality, second surgery, and postoperative radiation therapy for treating dermatofibrosarcoma protuberans (DFSP) are not well described. OBJECTIVE: We sought to determine the impact of patient demographics, tumor characteristics, and treatment site and modality on survival after primary DFSP. METHODS: A retrospective analysis of data from the National Cancer Database was performed for patients diagnosed with DFSP during 2003-2012...
June 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29140881/pdgfb-rearrangements-in-dermatofibrosarcoma-protuberans-of-the-vulva-a-study-of-11-cases-including-myxoid-and-fibrosarcomatous-variants
#17
Khadijeh Jahanseir, Deyin Xing, Patricia T Greipp, William R Sukov, Gary L Keeney, Brooke E Howitt, J Kenneth Schoolmeester
Dermatofibrosarcoma protuberans (DFSP) is a low-grade fibroblastic sarcoma that tends to arise in young to middle age adults and involve the trunk and proximal extremities. Rare examples of vulvar DFSP have been reported, including myxoid, myoid, and fibrosarcomatous variants, but detection of the characteristic t(17;22)(q22;q13) that produces COL1A1-PDGFB gene fusion has not been evaluated in a large series of primary vulvar tumors. The clinical, morphologic, immunohistochemical, and molecular cytogenetic features of 11 cases were examined...
November 14, 2017: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/29058523/orbitofacial-dermatofibrosarcoma-protuberans-with-intranasal-extension
#18
Siti Nurhuda Sharudin, Siow Wei Tan, Nor Fadhilah Mohamad, Suresh Kumar Vasudevan, Hanizah Khairan, Y C Mun, Jothi Shanmuganathan
A 25-year-old Chinese woman presented with recurrent painless swelling over the left medial canthus region for 3 months and intranasal mass for an indeterminate duration. Initial incision biopsy of the mass was reported as nodular fasciitis but the lesion recurred 3 weeks later. Intraoperative findings during repeat biopsy showed a mass extending from the deep dermal tissue into the anterior orbit and polyp-like nasal mass. Histopathology findings were that of dermatofibrosarcoma protuberans (DFSP). The mass recurred 4 months later without orbital or intranasal recurrence...
October 23, 2017: Orbit
https://www.readbyqxmd.com/read/29026517/dermatofibrosarcoma-protuberans-coexisting-in-a-patient-with-a-vascular-malformation-a-rare-coincidence
#19
Parthena Deskoulidi, Michael Sofopoulos, Pantelis Diamantopoulos, Thaleia Nikolaidou, Nikolaos Maltzaris, Maria Theodorakopoulou, Christos Klonaris, Niki Arnogiannaki, Maria Kotrotsiou, Spiros Stavrianos
Dermatofibrosarcoma protuberans with fibrosarcomatous differentiation (DFSP-FS) is a rare soft tissue tumor with more aggressive behavior and it is not clear what causes this type of skin cancer. We describe the case of a 48-year-old woman who was born with a vascular malformation in the sternal region and presented suddenly with a soft tissue sarcoma (DFSP-FS) in the same territory. She was initially treated by embolization as the sarcoma was misdiagnosed but the tumor within 6 months seemed to be growing rapidly and reached a giant dimension with ulceration and required surgical intervention...
October 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28988501/dermatofibrosarcoma-protuberans-and-gastrointestinal-stromal-tumor-as-models-for-targeted-therapy-in-soft-tissue-sarcomas
#20
REVIEW
Hanna Koseła-Paterczyk, Piotr Rutkowski
The development of novel targeted treatment in soft tissue sarcomas (STS) is important since many sarcoma subtypes are resistant to chemotherapy and effective therapeutic options are limited. Areas covered: This review discusses the molecular background and treatment in two STS types which became a model for targeted therapy - gastrointestinal stromal tumor (GIST) and dermatofibrosarcoma protuberans (DFSP). DFSP is characterized, by chromosomal translocation which results in the formation of COL1A1-PDGFB fusion gene causing platelet-derived growth factor receptor beta(PDGFRB) signaling activation in tumor cells...
December 2017: Expert Review of Anticancer Therapy
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