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    1917 results match your criteria Dermatofibrosarcoma Protuberans

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    Nonepithelial Tumors and Tumor-like Lesions of the Skin and Subcutis in Children.
    Pediatr Dev Pathol 2018 Mar-Apr;21(2):150-207
    3 Department of Pathology, University of Virginia, Charlottesville, Virginia.
    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. Read More

    Recurrent Dermatofibrosarcoma Protuberans of Scalp in a Distant Location 10 Years after Primary Excision.
    Ann Dermatol 2018 Apr 21;30(2):226-228. Epub 2018 Feb 21.
    Department of Dermatology, Korea University Ansan Hospital, Ansan, Korea.
    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. Read More

    A Rare Case of Dermatofibrosarcoma Protuberans of the Thumb in an 80-year-old Woman.
    Cureus 2018 Jan 2;10(1):e2016. Epub 2018 Jan 2.
    Pathology, Medical Faculty Trakia University.
    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. Read More

    Giant cells in soft tissue tumors! Is it a clue to diagnosis or cytologists mystery??? An unusual case report.
    J Cancer Res Ther 2018 Jan-Mar;14(2):444-446
    Department of General Surgery, KS Hegde Medical Academy, KS Hegde Medical Academy, Nitte University, Mangalore, Karnataka, India.
    Giant cells in soft tissue (ST) tumors are rare, pose great challenges to treating clinicians, and diagnosing pathologists. Common lesion with giant cells includes benign conditions such as nodular fasciitis to highly malignant lesions such as giant cell variant of malignant fibrous histiocytoma and extraskeletal osteosarcoma. Giant cell tumors of ST, extension of bony lesion to the ST are also rare possibilities. Read More

    PTEN Mutation Identified in Patient Diagnosed with Simultaneous Multiple Cancers.
    Cancer Res Treat 2018 Feb 27. Epub 2018 Feb 27.
    Department of Surgery, Uijeongbu St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Uijeongbu, Korea.
    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. Read More

    Relapse in dermatofibrosarcoma protuberans: A histological and molecular analysis.
    J Surg Oncol 2018 Mar 6. Epub 2018 Mar 6.
    Skin Cancer Department, AC Camargo Cancer Hospital, São Paulo, Brazil.
    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. Read More

    [Management for locally advanced dermatofibrosarcoma protuberans in Togo].
    Bull Cancer 2018 Apr 2;105(4):333-334. Epub 2018 Mar 2.
    Centre hospitalier universitaire Sylvanus-Olympio, Chirurgie générale-viscérale, Cancérologie chirurgicale, clinique médico-chirurgicale, 08BP 57 Lomé, Togo; Centre hospitalier universitaire Kara, CHU de SO, Service d'anatomie pathologique, Lomé, Togo.

    EGFR is involved in dermatofibrosarcoma protuberans progression to high grade sarcoma.
    Oncotarget 2018 Feb 3;9(9):8478-8488. Epub 2018 Jan 3.
    Pathology Department, Hôpital St Louis, APHP, Paris, France.
    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. Read More

    Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years.
    Open Access Maced J Med Sci 2018 Jan 10;6(1):35-37. Epub 2018 Jan 10.
    Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior, Sofia, Bulgaria.
    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. Read More

    An unusual clinical presentation of myxoid dermatofibrosarcoma protuberans with a prominent vasculature: A potential pitfall in the diagnosis of myxoid soft tissue tumors.
    J Cutan Pathol 2018 Feb 22. Epub 2018 Feb 22.
    University of California, David Geffen School of Medicine, Los Angeles, California.
    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. Read More

    A Rare Case of Misdiagnosis: Recurrence of Dermatofibrosarcoma Protuberans That Was Treated Surgicallyas a Keloid.
    Med Arch 2018 Feb;72(1):74-75
    Plastic and Reconstructive Surgery, Nevsehir Public Hospital, Nevsehir, Turkey.
    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. Read More

    Differential Diagnosis of Benign Spindle Cell Lesions.
    Surg Pathol Clin 2018 Mar 9;11(1):91-121. Epub 2017 Dec 9.
    Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Anatomic Pathology, University of Catania, Via S. Sofia 87, Catania 95123, Italy. Electronic address:
    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. Read More

    The role of small molecule platelet-derived growth factor receptor (PDGFR) inhibitors in the treatment of neoplastic disorders.
    Pharmacol Res 2018 Mar 3;129:65-83. Epub 2018 Feb 3.
    Blue Ridge Institute for Medical Research, 3754 Brevard Road, Suite 116, Box 19, Horse Shoe, NC, 28742-8814, United States. Electronic address:
    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. Read More

    Impact of Pathologist Involvement in Sarcoma and Rare Tumor Patient Support Groups on Facebook: A Survey of 542 Patients and Family Members.
    Arch Pathol Lab Med 2018 Jan 29. Epub 2018 Jan 29.
    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. Read More

    Clinical Features and Treatment of Fibrous Histiocytomas of the Tongue: A Systematic Review.
    Int Arch Otorhinolaryngol 2018 Jan 2;22(1):94-102. Epub 2017 May 2.
    Division of Dermatology, Creighton University School of Medicine, Omaha, Nebraska, United States.
     Benign fibrous histiocytomas are common lesions of the skin that rarely affect the tongue. Such cases are available in the literature exclusively as case reports. Similarly, malignant fibrous histiocytoma, now classified as undifferentiated pleomorphic sarcoma, is exceedingly rare in the tongue and not fully understood. Read More

    Histological types of soft-tissue sarcomas at the lagos university teaching hospital.
    Niger Postgrad Med J 2017 Oct-Dec;24(4):205-209
    Department of Anatomic and Molecular Pathology, Lagos University Teaching Hospital, Lagos, Nigeria.
    Objective: There is scanty data on histologically diagnosed soft-tissue sarcomas in the Nigerian literature. This is due to paucity of facilities for ancillary testing as well as a dearth of specialist soft tissue pathologists. Knowledge however of the common soft-tissue sarcomas is vital for the establishment of an effective sarcoma service. Read More

    [Extrapleural solitary fibrous tumor with uncommon histology: a clinicopathologic analysis of 7 cases].
    Zhonghua Bing Li Xue Za Zhi 2018 Jan;47(1):51-56
    Department of Pathology, Zhejiang Provincial People's Hospital, People's Hospital of Hangzhou Medical College, Hangzhou 310014, China.
    To investigate the clinicopathologic characteristics, immunophenotypes, and differential diagnostic features of extra-pleural solitary fibrous tumor (SFT) with uncommon histology. Seven cases of extra-pleural SFT with uncommon histology were collected during January 2015 and December 2016 in Zhejiang Provincal People's Hospital; the clinical and radiologic features, histomorphology, immunophenotype and prognosis were analyzed. EnVision method was used for immunohistochemical staining of STAT6, CD34 and other differential diagnosis associated markers. Read More

    Suitability of the Cellient cell block method for diagnosing soft tissue and bone tumors.
    Diagn Cytopathol 2018 Apr 10;46(4):299-305. Epub 2018 Jan 10.
    Department of Pathology and Medical Biology, University Medical Center Groningen, University of Groningen, P.O. Box 30.001, RB Groningen, 9700, The Netherlands.
    Background: The diagnosis of tumors of soft tissue and bone (STB) heavily relies on histological biopsies, whereas cytology is not widely used. Cellient cell blocks often contain small tissue fragments. In addition to Hematoxylin and Eosin (H&E) interpretation of histological features, immunohistochemistry (IHC) can be applied after optimization of protocols. Read More

    Fluorescence microscopy for the evaluation of elastic tissue patterns within fibrous proliferations of the skin on hematoxylin-eosin-stained slides.
    J Am Acad Dermatol 2018 Jan 5. Epub 2018 Jan 5.
    Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, SC. Electronic address:
    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. Read More

    Late-Stage Erythema Elevatum Diutinum Mimicking a Fibroblastic Tumor: A Potential Pitfall.
    Am J Dermatopathol 2017 Dec 28. Epub 2017 Dec 28.
    Dermatopathology Department, Friedrichshafen Dermatopathologie, Friedrichshafen, Germany.
    Erythema elevatum diutinum (EED) is a rare dermatosis with evolving histopathological features that vary according to the age of the lesions, with a variable fibrosis and a fascicled proliferation of spindle cells in late phases. The authors present an otherwise healthy 57-year-old woman with multiple indurated nodules on the inner aspect of both feet. Skin biopsy showed storiform interlacing bundles of spindled cells with plump nuclei and some areas with neutrophils and leukocytoclasia. Read More

    Reconstructive methods in Mohs micrographic surgery in Uruguay: A bidirectional descriptive cohort analysis.
    Actas Dermosifiliogr 2018 Apr 14;109(3):254-261. Epub 2017 Dec 14.
    Cátedra de Dermatología Prof. Dr. Miguel Martínez, Hospital de Clínicas Dr. Manuel Quintela, Universidad de la República, Montevideo, Uruguay.
    Background And Objectives: The primary goal of Mohs micrographic surgery (MMS) is to completely excise a cancerous lesion and a wide range of reconstructive techniques of varying complexity are used to close the resulting wound. In this study, we performed a descriptive analysis of patients who underwent MMS, with a focus on wound closure methods.

    Material And Methods: We conducted a bidirectional descriptive cohort analysis of all MMS procedures performed by a single surgeon between November 2013 and April 2016. Read More

    [What's new in instrumental dermatology?]
    Ann Dermatol Venereol 2017 Dec;144 Suppl 4:IVS10-IVS15
    Service de dermatologie générale et oncologique, CHU Ambroise-Paré 9, avenue Charles-de-Gaulle, 92200 Boulogne-Billancourt, 75007 Paris, France; cabinet médical, 4 bis, avenue de Ségur, 75007 Paris, France. Electronic address:
    This "What's new in instrumental dermatology" dedicated skin surgeryis based upon a 2015-2017 literature analysis. The excision of skin cancers is an important part of surgical dermatology. Will artificial intelligence and new drug be able to face the increasing need for therapy? Wrong-site surgery is due to multiple factors. Read More

    Dermatofibrosarcoma Protuberans of Distal Extremities and Acral Sites: A Clinicopathologic Analysis of 27 Cases.
    Am J Surg Pathol 2018 Mar;42(3):413-419
    Departments of Pathology.
    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. Read More

    Survival in patients with primary dermatofibrosarcoma protuberans: National cancer database analysis.
    J Am Acad Dermatol 2017 Nov 23. Epub 2017 Nov 23.
    Department of Dermatology, University of Arizona College of Medicine, Phoenix, Arizona. Electronic address:
    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. Read More

    PDGFB Rearrangements in Dermatofibrosarcoma Protuberans of the Vulva: A Study of 11 Cases Including Myxoid and Fibrosarcomatous Variants.
    Int J Gynecol Pathol 2017 Nov 14. Epub 2017 Nov 14.
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minneapolis (K.J., P.T.G., W.R.S., G.L.K., J.K.S.) Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland (D.X.) Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts (B.E.H.).
    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. Read More

    Orbitofacial dermatofibrosarcoma protuberans with intranasal extension.
    Orbit 2017 Oct 23:1-5. Epub 2017 Oct 23.
    e Department of Otorhinolaryngology , Hospital Sultanah Aminah , Johor Bahru , Johor , Malaysia.
    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. Read More

    Dermatofibrosarcoma protuberans coexisting in a patient with a vascular malformation-a rare coincidence.
    J Surg Case Rep 2017 Oct 9;2017(10):rjx192. Epub 2017 Oct 9.
    Department of Plastic Surgery, St. Savvas Cancer Hospital of Athens, 171 Alexandras Avenue, Athens 11522, Greece.
    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. Read More

    Dermatofibrosarcoma protuberans and gastrointestinal stromal tumor as models for targeted therapy in soft tissue sarcomas.
    Expert Rev Anticancer Ther 2017 12 13;17(12):1107-1116. Epub 2017 Oct 13.
    a Department of Soft Tissue/Bone Sarcoma and Melanoma , Maria Sklodowska-Curie Institute Oncology Center , Warsaw , Poland.
    Introduction: 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. Read More

    Unforeseen clonal evolution of tumor cell population in recurrent and metastatic dermatofibrosarcoma protuberans.
    PLoS One 2017 4;12(10):e0185826. Epub 2017 Oct 4.
    Laboratory of Molecular Pathology and Cancer Genomics, College of Pharmacy, Seoul National University, Seoul, Korea.
    Dermatofibrosarcoma protuberans (DFSP) is a very rare soft tissue sarcoma, generally of low-grade malignancy. DFSP is locally aggressive with a high recurrence rate, but metastasis occurs rarely. To investigate the mechanism of metastasis in DFSP, we analyzed the whole exome sequencing data of serial tumor samples obtained from a patient who had a 10-year history of recurrent and metastatic DFSP. Read More

    Involvement of platelet-derived growth factor ligands and receptors in tumorigenesis.
    J Intern Med 2018 Jan 17;283(1):16-44. Epub 2017 Oct 17.
    Department of Genetics and Pathology, Uppsala University, Uppsala, Sweden.
    Platelet-derived growth factor (PDGF) isoforms and their receptors have important roles during embryogenesis, particularly in the development of various mesenchymal cell types in different organs. In the adult, PDGF stimulates wound healing and regulates tissue homeostasis. However, overactivity of PDGF signalling is associated with malignancies and other diseases characterized by excessive cell proliferation, such as fibrotic conditions and atherosclerosis. Read More

    [A pink nodule on the face].
    Pan Afr Med J 2017 18;27:205. Epub 2017 Jul 18.
    Service de Dermatologie, Centre Hospitalier Universitaire Ibn Sina, Rabat, Maroc.
    We report the case of a 32-year old female patient, with no notable medical history, requiring dermatology consultation for evaluation of a nodule on her right cheek which had evolved over the past 10 years. Clinical examination showed a reddish dermal-based nodule with a smooth surface. The lesion measured 1cm in diameter and was located at the level of the right cheek (A). Read More

    Orbital dermatofibrosarcoma protuberans with frontal and ethmoid sinus involvement: A case report and brief review of literature.
    Indian J Ophthalmol 2017 Sep;65(9):892-894
    Department of Radiation Oncology, Safdarjung Hospital, New Delhi, India.
    Dermatofibrosarcoma protuberans is a soft tissue sarcoma that is dermal in origin. The incidence is <0.1% of all malignancies and 1% of soft tissue sarcoma. Read More

    Discovery of Human-Similar Gene Fusions in Canine Cancers.
    Cancer Res 2017 11 7;77(21):5721-5727. Epub 2017 Sep 7.
    Univ. Rennes 1, CNRS, Institut de Geéneétique et de Deéveloppement de Rennes (IGDR), UMR6290, Rennes, France.
    Canine cancers represent a tremendous natural resource due to their incidence and striking similarities to human cancers, sharing similar clinical and pathologic features as well as oncogenic events, including identical somatic mutations. Considering the importance of gene fusions as driver alterations, we explored their relevance in canine cancers. We focused on three distinct human-comparable canine cancers representing different tissues and embryonic origins. Read More

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