1,977 results match your criteria Dermatofibrosarcoma Protuberans


Patterns of infiltration and local recurrences of various types of cutaneous sarcomas following three-dimensional histology.

J Dtsch Dermatol Ges 2018 Dec;16(12):1434-1442

Department of Dermatology, Eberhardt Karls University, Tübingen Germany.

Background: Cutaneous sarcomas are rare and characterized by pathogenetic heterogeneity. Knowledge about local infiltration patterns and recurrence rates may be useful in improving patient care and outcomes. The objective of the present study was to compare these two characteristics in sarcomas that had been treated using the identical surgical procedure. Read More

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December 2018

Cutaneous Sarcomas.

Hematol Oncol Clin North Am 2019 Feb;33(1):87-101

Department of Dermatology, Brigham and Women's Hospital, 221 Longwood Avenue, Boston, MA 02115, USA; Cutaneous Oncology Program, Dana Farber Cancer Institute, Boston, MA 02215, USA. Electronic address:

Cutaneous sarcomas are rare malignancies that may present with a variety of clinical manifestations. This article focuses on 4 of the most common cutaneous sarcomas (Kaposi sarcoma, cutaneous angiosarcoma, dermatofibrosarcoma protuberans, and cutaneous leiomyosarcoma) and reviews clinical, diagnostic, and therapeutic aspects of these rare skin malignancies. Read More

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February 2019

Clinical Utility of In Situ Hybridization Assays in Head and Neck Neoplasms.

Head Neck Pathol 2018 Nov 22. Epub 2018 Nov 22.

Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney, Australia.

Head and neck pathology present a unique set of challenges including the morphological diversity of the neoplasms and presentation of metastases of unknown primary origin. The detection of human papillomavirus and Epstein-Barr virus associated with squamous cell carcinoma and newer entities like HPV-related carcinoma with adenoid cystic like features have critical prognostic and management implications. In salivary gland neoplasms, differential diagnoses can be broad and include non-neoplastic conditions as well as benign and malignant neoplasms. Read More

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November 2018
3 Reads

[Alternative therapy option for plastic-aesthetic treatment of defects after resection in the upper third of the facial region in the sense of subtotal scalping instead of local flap plastics].

Hautarzt 2018 Nov 22. Epub 2018 Nov 22.

Klinik für Mund‑, Kiefer- und Gesichtschirurgie, Universitätsklinikum Münster, Waldeyerstr. 30, 48149, Münster, Deutschland.

In the presented case, the resulting defect size after resection of a dermatofibrosarcoma protuberans exceeded the treatment capability with local flaps in the region of the exposed facial skin. Through the use of conventional wound healing in combination with a meshed split-thickness skin graft, plastic aesthetic soft tissue treatment with an aesthetically satisfactory result was possible. Read More

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November 2018

Dermatofibrosarcoma Protuberans in a Male Infant.

Urology 2018 Nov 10. Epub 2018 Nov 10.

Department of Urology, University of Kentucky, Lexington, KY; Department of Surgery, Division of Urology, University of Colorado, Aurora, CO. Electronic address:

Dermtofibrosarcoma protuberans is a rare cutaneous malignancy known to be locally aggressive. It is uncommonly seen in the pediatric population and can be difficult to distinguish from other benign skin lesions. We present a case of dermatofibrosarcoma protuberans of the penis in a 6-month-old child managed with surgical resection. Read More

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November 2018
5 Reads

Establishment of novel patient-derived models of dermatofibrosarcoma protuberans: two cell lines, NCC-DFSP1-C1 and NCC-DFSP2-C1.

In Vitro Cell Dev Biol Anim 2018 Nov 8. Epub 2018 Nov 8.

Department of Innovative Seeds Evaluation, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.

Dermatofibrosarcoma protuberans (DFSP) is a common type of dermal sarcoma, characterized by the presence of the unique collagen type I alpha 1 chain (COL1A1)-PDGFB translocation, which causes constitutive activation of the platelet-derived growth factor β (PDGFB) signaling pathway. Patients with DFSP exhibit frequent local recurrence, and novel therapeutic approaches are required to achieve better clinical outcomes. Patient-derived cancer cell lines are essential in the preclinical research. Read More

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November 2018
4 Reads

Bednar Tumour Occurring after Malignant Melanoma Excision.

Case Rep Pathol 2018 1;2018:7694272. Epub 2018 Oct 1.

Anatomic Pathology, University of Rome Tor Vergata, Viale Oxford 81, 00133 Rome, Italy.

We report the case of a seventy-four-year-old man with a slow-growing 2 cm mass on the back that arose near the surgical scar of previously excised melanoma, invasive to a Breslow depth of 3 mm. Preoperative clinical diagnosis was "in-transit" melanoma metastasis. After surgical excision, histopathologic examination revealed a dermal nodular proliferation of spindle cells arranged in storiform pattern, with mild pleomorphism, infiltrating around appendages and into the subcutaneous tissue. Read More

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October 2018
5 Reads

Clinical and radiographic presentation of pelvic sarcoma in children.

SICOT J 2018 19;4:44. Epub 2018 Oct 19.

Department of Orthopaedics, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

Introduction: Pelvic sarcomas may present with vague symptoms. The aim of this study was to examine the characteristics and clinical presentations of different types of pelvic sarcoma in children.

Methods: This is a retrospective cohort study of patients up to 21 years of age with the diagnosis of pelvic sarcoma between January 2000 and June 2013. Read More

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October 2018
2 Reads

Expression of Proton-sensing G-Protein-coupled receptors in selected skin tumors.

Exp Dermatol 2018 Oct 19. Epub 2018 Oct 19.

Department of Dermatology.

Background: In humans, there are four known proton-sensing G-Protein-coupled receptors (pH-GPCRs): GPR4 (GPR19), TDAG8 (GPR65, T-cell death-associated gene 8), OGR1 (GPR68, ovarian cancer GPCR1), and G2A (GPR132, G2 accumulation protein). They are known to be involved in sensing changes of extracellular proton-concentrations in the acidic microenvironment of tumors, which leads to altered cell proliferation, migration, metastasis, immune cell function and inflammation. However, little is known about the expression of pH-GPCRs in the skin and especially skin cancers. Read More

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October 2018
3 Reads

Jellyfish-like sonographic pattern can help recognition of dermatofibrosarcoma protuberans. Report of 3 new cases and review of the literature.

Australas J Dermatol 2018 Oct 14. Epub 2018 Oct 14.

Department of Dermatology, Hospital Universitario La Paz, Madrid, Spain.

High-frequency ultrasonography (HFUS) can help improve dermatofibrosarcoma protuberans (DFSP) recognition. We present three cases of DFSP in which a "jellyfish-like" sonographic pattern was a useful adjunct in formulating the diagnosis. In addition, we review all DFSP ultrasound images available in the literature. Read More

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October 2018
2 Reads

An audit of the diagnosis and reporting of soft tissue sarcomas at the Lagos University Teaching Hospital.

Niger J Clin Pract 2018 Oct;21(10):1330-1336

Department of Anatomic and Molecular Pathology, Lagos University Teaching Hospital, Lagos, Nigeria.

Background: : The effective management of patients with cancer is predicated on the right diagnoses and other relevant parameters included in the pathology report. This is particularly important in soft tissue pathology where arriving at the right diagnosis is often challenging. The aim of this study, therefore, was to perform an audit of sarcoma diagnosis and reporting in our institution. Read More

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October 2018

[Dermatofibrosarcoma: Management].

Bull Cancer 2018 Nov 5;105(11):1094-1101. Epub 2018 Oct 5.

Centre Oscar Lambret, département de cancérologie générale, 3, rue F. Combemale, 59020 Lille, France.

Dematofibrosarcoma protuberans (DFSP) are very rare (1 to 4 incident cases per million of inhabitants). The local spreading of DFSP is underestimated. The histological diagnosis is challenging but we now know a specific marker (translocation t(17;22)(q22;q13) (COL1A1;PDGFB)). Read More

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November 2018
2 Reads

Dermatofibrosarcoma of The Breast - Case Report and Review of Literature.

Eur J Breast Health 2018 Oct 1;14(4):234-237. Epub 2018 Oct 1.

Department of Clinical Oncology and Nuclear Medicine, Sohag University Hospital, Sohag, Egypt.

Dermatofibrosarcoma protuberans is a rare form of soft tissue sarcoma that tends to invade and recur locally. The most common site of this disease is head, neck and extremities; however, the involvement of the breast has been reported. We present a case with a dermatofibrosarcoma of the breast that is composed of low-grade spindle cells which are positive for CD43. Read More

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October 2018
2 Reads

Dermatofibrosarcoma Protuberans of the Breast-a Rare Entity.

Indian J Surg Oncol 2018 Sep 10;9(3):351-354. Epub 2017 Aug 10.

3Department Of Endocrine Surgery, Christian Medical College Vellore, Tamil Nadu, 632004 India.

Dermatofibrosarcoma protuberans (DFSP) represents about 1% of soft-tissue sarcomas with an estimated incidence of 0.8 to 5.0 cases per million per year. Read More

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September 2018
4 Reads

Histopathological pattern of skin cancer in Western region of Saudi Arabia. An 11 years experience.

Saudi Med J 2018 Oct;39(10):994-998

Department of Pathology, Taibah University, Madinah, Kingdom of Saudi Arabia. E-mail.

Objectives: To determine the histopathological pattern of skin cancer in Madinah region of Kingdom of Saudi Arabia (KSA). Methods: This is a retrospective study including all cases with a histological diagnosis of skin cancer diagnosed at the Department of Pathology, King Fahad Hospital, Madinah, Saudi Arabia, between January 2006 and December 2017. Data included  age, gender, site and histology of the tumor were collected from histopathological reports and analyzed using Statistical Package for the Social Sciences  (SPSS) Version 21. Read More

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October 2018
6 Reads

First reported case of dermatofibrosarcoma in siblings.

Authors:
H Smith M Lam A Patel

Clin Exp Dermatol 2018 Sep 28. Epub 2018 Sep 28.

Dermatology Department, Gateway A, The Treatment Centre, Circle Nottingham, Lister Road, Nottingham, NG7 2FT, UK.

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September 2018
7 Reads

Pediatric dermatologic surgery: our experience.

G Ital Dermatol Venereol 2018 Sep 24. Epub 2018 Sep 24.

Department of Dermatology, ASST degli Spedali Civili, Spedali Civili di Brescia, Brescia, Italy.

Background: Surgical treatment for pediatric skin disorders are used for diagnostic and therapeutic reasons. We underline these procedures are usually easy and uncomplicated without the need for general anesthesia. Objective of this study was to share our experience in the field of pediatric dermatologic surgery. Read More

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September 2018
5 Reads

Metastasis of dermatofibrosarcoma diagnosed by capsule endoscopy.

Rev Esp Enferm Dig 2018 Sep 21. Epub 2018 Sep 21.

Aparato Digestivo, Hospital Clínico San Carlos.

In the study of obscure gastrointestinal bleeding, which includes iron-deficiency anemia, the capsule endoscopy is a valuable diagnostic tool. In the different series the presence of tumors reaches 16% as the cause of it. We present the case of a rare tumor with metastatic extension in the small intestine in which the capsule endoscopy was key to the diagnosis and survival of the patient. Read More

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September 2018
7 Reads

Collagen Features of Dermatofibrosarcoma Protuberans Skin Base on Multiphoton Microscopy.

Technol Cancer Res Treat 2018 Jan;17:1533033818796775

1 College of Photonic and Electronic Engineering, Fujian Normal University, Fujian Provincial Key Laboratory of Photonic Technology, Key Laboratory of Optoelectronic Science and Technology for Medicine, Ministry of Education, Fuzhou, China.

Dermatofibrosarcoma protuberans is a rare, low-grade skin fibroblastic tumor which tends to recur locally due to its high misdiagnosis. Dermatofibrosarcoma protuberans usually spreads through the intracutaneous and subcutaneous layers into the deep dermis layer in which the main component is collagen. Therefore, alterations in collagen shape and content are important for accurate diagnosis of dermatofibrosarcoma protuberans. Read More

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January 2018
5 Reads

Recurrent Dermatofibrosarcoma with Pigmentation and Myoid Differentiation.

Sultan Qaboos Univ Med J 2018 May 9;18(2):e228-e230. Epub 2018 Sep 9.

Department of Surgery, Maulana Azad Medical College, New Delhi, India.

Dermatofibrosarcomas (DFSP) are rare low-grade tumours with various subtypes and usually occur among middle-aged adults. However, myoid differentiation is very rare. We report a 44-year-old woman who presented to the Lok Nayak Jai Prakash Hospital, New Delhi, India, in 2017 with a recurrent pigmented DFSP presenting as an arm swelling. Read More

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May 2018
2 Reads

Dermatofibrosarcoma Protuberans Secondary to a Decorative Tattoo: An Isotattootopic Response?

Indian J Dermatol 2018 Sep-Oct;63(5):439-440

Department of Surgery and Translational Medicine, Division of Dermatology and Venereology, University of Florence, Florence, Italy. E-mail:

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September 2018
1 Read

Nodular fasciitis, a forgotten entity.

Int J Dermatol 2018 Sep 6. Epub 2018 Sep 6.

Department of Dermatology, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina.

Background: Nodular fasciitis is a benign pseudosarcomatous, self-limited, and reactive process. Based on its clinical and histological features - a fast-growing, solitary tumor with high cellularity and mitotic count - nodular fasciitis is considered to be a benign mimic of sarcoma.

Methods: We present four cases of nodular fasciitis and a review of the literature. Read More

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September 2018
8 Reads

Novel KHDRBS1-NTRK3 rearrangement in a congenital pediatric CD34-positive skin tumor: a case report.

Virchows Arch 2018 Sep 6. Epub 2018 Sep 6.

Department of Dermatology, Unit of Pediatric Dermatology - CHRU Tours, Tours, France.

Cutaneous spindle-cell neoplasms in adults as well as children represent a frequent dilemma for pathologists. Along this neoplasm spectrum, the differential diagnosis with CD34-positive proliferations can be challenging, particularly concerning neoplasms of fibrohistiocytic and fibroblastic lineages. In children, cutaneous and superficial soft-tissue neoplasms with CD34-positive spindle cells are associated with benign to intermediate malignancy potential and include lipofibromatosis, plaque-like CD34-positive dermal fibroma, fibroblastic connective tissue nevus, and congenital dermatofibrosarcoma protuberans. Read More

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September 2018
8 Reads
2.650 Impact Factor

A novel case of an aggressive superficial spindle cell sarcoma in an adult resembling fibrosarcomatous dermatofibrosarcoma protuberans and harboring an EML4-NTRK3 fusion.

J Cutan Pathol 2018 Dec 26;45(12):933-939. Epub 2018 Sep 26.

Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, Lebanon, New Hampshire.

A subset of soft tissue sarcomas often harbors recurrent fusions involving protein kinases. While some of these fusion events have shown utility in arriving at a precise diagnosis, novel fusions in otherwise difficult to classify sarcomas continue to be identified. We present a case of a 40-year-old female who noted a lower back nodule in 2010 that was initially labeled as a dermatofibrosarcoma protuberans with fibrosarcomatous transformation. Read More

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December 2018
5 Reads

Recurrent dermatofibrosarcoma protuberans: challenging a surgeon's dexterity for the 'tricky' margins.

Ecancermedicalscience 2018 13;12:858. Epub 2018 Aug 13.

Department of General Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi 110 029, India.

Soft tissue tumours represent 0.2%-1% of all breast malignancies. [Al Tarakji M, Toro A, and Di Carlo I, (2015) 158 https://doi. Read More

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Review of the medical literature and assessment of current utilization patterns regarding the use of two common fluorescence in situ hybridization assays in the diagnosis of dermatofibrosarcoma protuberans and clear cell sarcoma.

J Cutan Pathol 2018 Dec 27;45(12):905-913. Epub 2018 Sep 27.

Departments of Dermatology and Pathology, University of Michigan Medical Center, Ann Arbor, Michigan.

Background: Dermatofibrosarcoma protuberans (DFSP) is a tumor of intermediate malignancy, which in selected circumstances can pose difficulty in diagnosis. Clear cell sarcoma (CCS) is a very rare aggressive soft tissue sarcoma that can be difficult to distinguish histologically from melanoma.

Methods: The current literature on t(17;22) COL1A1-PDGFB fluorescence in situ hybridization (FISH) assay in DFSP was reviewed. Read More

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December 2018
8 Reads

Giant fibrous hamartoma of infancy: pitfall of CD34 positive dermal mesenchymal tumor.

Dermatol Online J 2018 Jun 15;24(6). Epub 2018 Jun 15.

Dermatology Department, Hospital de Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisboa.

Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue tumor with a triphasic organoid histologic appearance. The authors present a case of a 21-month-old healthy girl with a slowly growing flesh-colored subcutaneous plaque 12cm in size on the lower back, with overlying hypertrichosis. A punch biopsy revealed a proliferation of spindle cells infiltrating the dermis and hypodermis organized in a dense storiform pattern with a strong diffuse positivity for CD34. Read More

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June 2018
1 Read

Imatinib-induced drug reaction with eosinophilia and systemic symptoms in solid tumors: a patient with dermatofibrosarcoma protuberans and successful desensitization management.

Anticancer Drugs 2018 Oct;29(9):919-923

Center for Sarcoma and Bone Oncology, Dana Farber Cancer Institute.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe, potentially life-threatening drug-induced hypersensitivity reaction, characterized by cutaneous eruptions, fever, diffuse lymphadenopathy, along with hypereosinophilia, and elevated liver function tests, which in severe cases may lead to fulminant hepatic failure and death. Although DRESS syndrome has been associated with over 50 different drugs including imatinib, it has never been reported in association with imatinib treatment in solid tumors. We recently treated a patient with metastatic dermatofibrosarcoma protuberans, a rare cutaneous mesenchymal tumor characterized by constitutive activation of the PDGFβ receptor and high sensitivity to imatinib therapy, who had a DRESS reaction to imatinib. Read More

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October 2018
11 Reads

Advanced Dermatofibrosarcoma Protuberans Treatment With Imatinib: Experience From a Dedicated Sarcoma Medical Oncology Clinic in India.

J Glob Oncol 2018 Jul(4):1-7

Sameer Rastogi, Ekta Dhamija, Adarsh Barwad, Atul Sharma, and Rambha Panday, All India Institute of Medical Sciences; and Aditi Aggarwal, Maulana Azad Medical College, New Delhi, India.

Purpose Advanced dermatofibrosarcoma protuberans (DFSP) is an exceptionally uncommon disease with scarce literature, especially from developing countries. Molecular testing is unfortunately not available in India, and expert diagnosis by a sarcoma pathologist is available only in tertiary care centers. Materials and Methods We retrospectively analyzed consecutive patients with inoperable DFSP (on the basis of expert histopathology only) who presented to our sarcoma medical oncology clinic from January 2016 to July 2017. Read More

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July 2018
9 Reads

STAT6 expression in spindle cell lesions of the breast: An immunohistochemical study of 48 cases.

Pathol Res Pract 2018 Oct 17;214(10):1544-1549. Epub 2018 Jul 17.

Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Azienda Ospedaliero-Universitaria "Policlinico-Vittorio Emanuele", Anatomic Pathology Section, School of Medicine, University of Catania, 95123 Catania, Italy.

The diagnosis of spindle cell lesions of the breast parenchyma is challenging. Some of these lesions share the expression of CD34, posing differential diagnostic problems, especially in core biopsies. Recently, antibodies against the STAT6 C-terminal, are being used in paraffin-embedded tissues as a surrogate for identifying the NAB2-STA6 fusion gene which is considered a specific molecular marker for solitary fibrous tumor. Read More

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October 2018
10 Reads

Mohs micrographic surgery for dermatofibrosarcoma protuberans: comparison of frozen and paraffin techniques.

J Eur Acad Dermatol Venereol 2018 Dec 20;32(12):2171-2177. Epub 2018 Aug 20.

Department of Dermatology, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.

Background: Due to the propensity for local recurrence, Mohs micrographic surgery (MMS) has been suggested for the treatment of dermatofibrosarcoma protuberans (DFSP) and it has shown improved clinical outcomes. Recently, some authors suggested that MMS using paraffin-embedded sections (paraffin MMS) is superior in DFSP treatment compared with the conventional frozen MMS method. However, there have been no studies comparing frozen and paraffin MMS for the treatment of DFSP. Read More

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December 2018
1 Read

Dermatofibrosarcoma Protuberans of Breast.

J Coll Physicians Surg Pak 2018 Aug;28(8):645-647

Department of Oncology, Jinnah Postgraduate Medical Centre, Karachi.

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant neoplasm of skin, subcutaneous tissue and dermis, with more potential of local infiltration, appears most frequently on the trunk, followed by extremities, head, neck, and extremely rare in breast. It often occurs in young adults and middle-aged adults. The standard of treatment for this disease is local surgical excision with 2-3 cm wide margins. Read More

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August 2018
4 Reads
0.320 Impact Factor

Congenital fibroblastic connective tissue nevi: Unusual and misleading presentations in three infantile cases.

Pediatr Dermatol 2018 Sep 19;35(5):644-650. Epub 2018 Jul 19.

Services de Chirurgie Maxillo-Faciale et Plastique, Assistance Publique-Hôpitaux de Paris, Hôpital Universitaire Necker Enfants-Malades, Université Sorbonne Paris Cité, Université Paris Descartes, Paris, France.

Background: Fibroblastic connective tissue nevi (FCTN) are benign skin conditions characterized by bland spindle cells infiltrating the reticular dermis and the upper subcutis with preservation of adnexal structures. A subset of FCTN expresses CD34, which may cause difficulties in the differential diagnosis, in particular with dermatofibrosarcoma (DFSP). We aim to study clinical and histological main features of congenital FCTN to better understand their heterogeneity. Read More

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September 2018
4 Reads

Dermatofibrosarcoma protuberans with a novel COL6A3-PDGFD fusion gene and apparent predilection for breast.

Genes Chromosomes Cancer 2018 09 14;57(9):437-445. Epub 2018 Aug 14.

Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York.

Dermatofibrosarcoma protuberans is a locally aggressive superficial mesenchymal neoplasm. It typically occurs in adulthood, and has been reported to have a slight male predilection. Tumors have a characteristic histopathologic appearance, including: storiform architecture, infiltrative "honeycomb" growth within subcutaneous adipose tissue, and immunoreactivity for CD34. Read More

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September 2018
7 Reads
4.040 Impact Factor

Dermatofibrosarcoma protuberans: A clinical analysis.

Oncol Lett 2018 Aug 24;16(2):1855-1862. Epub 2018 May 24.

Department of Plastic Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a high incidence of misdiagnosis. DFSP has a high rate of recurrence but a low rate of metastasis. In the present study, retrospective analyses were performed on the clinical features, differential diagnosis and treatment of patients with DFSP to improve our understanding of the disease and allow more effective treatment measures to be implemented. Read More

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Acellular dermal matrix for breast reconstruction after surgery for giant dermatofibrosarcoma protuberans.

Ann Ital Chir 2018 Jul 12;7. Epub 2018 Jul 12.

Introduction: Dermatofibrosarcoma protuberans (DFSP) is a rare neoplasm that exceptionally can affect the breast, always originating from skin and dermis, so imposing large sacrifice of skin. Only few cases have been reported of intraparenchymal DFS. We describe a unique case of giant intraparenchymal DFS that required removal of all the gland and reconstructive surgery. Read More

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July 2018
12 Reads

Lipofibromatosis-like neural tumour: a clinicopathological study of ten additional cases of an emerging novel entity.

Pathology 2018 Aug 27;50(5):519-523. Epub 2018 Jun 27.

Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China. Electronic address:

We present our experience with ten cases of lipofibromatosis-like tumour (LPF-NT) to further characterise this newly described neoplasm. There were six males and four females with a mean age of 12.8 years (range 2-37 years). Read More

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August 2018
6 Reads

Alternative PDGFD rearrangements in dermatofibrosarcomas protuberans without PDGFB fusions.

Mod Pathol 2018 Nov 28;31(11):1683-1693. Epub 2018 Jun 28.

Cancer Research Center of Lyon, Université Claude Bernard Lyon 1, CNRS 5286, INSERM U1052, Lyon, France.

Dermatofibrosarcoma protuberans is underlined by recurrent collagen type I alpha 1 chain-platelet-derived growth factor B chain (COL1A1-PDGFB) fusions but ~ 4% of typical dermatofibrosarcoma protuberans remain negative for this translocation in routine molecular screening. We investigated a series of 21 cases not associated with the pathognomonic COL1A1-PDGFB fusion on routine fluorescence in situ hybridization (FISH) testing. All cases displayed morphological and clinical features consistent with the diagnosis of dermatofibrosarcoma protuberans. Read More

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November 2018
3 Reads

Management of dermatofibrosarcoma protuberans of the face using lower trapezius musculocutaneous pedicle flap reconstruction: a case report.

J Surg Case Rep 2018 Jun 15;2018(6):rjy089. Epub 2018 Jun 15.

Implantlogy (Cambridge Academy, UK), Dental School, Beirut Arab University (BAU), Beirut, Lebanon.

Dermatofibrosarcoma protuberans (DFSP) is a rare neoplasm which represents <0.1% of all tumors but it is considered the most common skin sarcoma. It is a slow-growing tumor that arises from the dermis and invades deeper tissues. Read More

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Lipofibromatosis-like neural tumor: Case report of a unique infantile presentation.

JAAD Case Rep 2018 Mar 4;4(2):185-188. Epub 2018 Feb 4.

Department of Dermatology, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts.

A 14-month-old boy presented with a slow-growing, asymptomatic back plaque, which was biopsied and found to have S100 positivity, sparse CD34 staining, and no significant mitotic activity, nuclear pleomorphism, or necrosis; genetic workup found gene fusion, overall consistent with lipofibromatosis-like neural tumor (LPF-NT). LPF-NT is rare, with 14 cases previously reported, and our patient is the first report of this diagnosis in infancy. This case report and literature review includes comparison of similar diagnoses including lipofibromatosis, low-grade malignant peripheral nerve sheath tumor, infantile fibrosarcoma, and dermatofibrosarcoma protuberans and serves to aid detection of LPF-NT presenting in pediatric patients by highlighting similarities and differences that should prompt consideration. Read More

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March 2018
16 Reads

[Unusual giant cell fibroblastoma: A case report].

Pan Afr Med J 2017 24;28:263. Epub 2017 Nov 24.

Service d'Anatomie Pathologique, Hôpital d'Enfants, 18, Avenue Attine, Secteur 7, Hay Riad, Rabat, Maroc.

Giant cell fibroblastoma (GCF) is a rare superficial tumor occurring in male infants. We report the case of a 3-month old infant examined for a mass in his right arm. Arm MRI objectified a subcutaneous mass. Read More

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June 2018
2 Reads

Hormone receptors analysis in Chinese patients with dermatofibrosarcoma protuberans.

J Surg Oncol 2018 Jul 7;118(1):157-166. Epub 2018 Jun 7.

Department of Plastic and Aesthetic Surgery, Peking Union Medical College Hospital, Beijing, China.

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. Read More

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July 2018
2 Reads

Evaluation of pan-TRK immunohistochemistry in infantile fibrosarcoma, lipofibromatosis-like neural tumour and histological mimics.

Histopathology 2018 Oct 5;73(4):634-644. Epub 2018 Jul 5.

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Aims: Infantile fibrosarcoma is characterised by intersecting fascicles of spindle cells and ETV6-NTRK3 gene fusion in most cases. Given histological overlap with other spindle-cell tumours, the diagnosis can be challenging and often requires molecular confirmation. A recently developed pan-TRK antibody shows promise for identifying tumours with NTRK fusions. Read More

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October 2018
50 Reads

Dermatofibrosarcoma protuberans: A retrospective study of clinicopathologic features and related Akt/mTOR, STAT3, ERK, cyclin D1, and PD-L1 expression.

J Am Acad Dermatol 2018 Nov 22;79(5):843-852. Epub 2018 May 22.

Department of Pathology, Seoul National University Boramae Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea. Electronic address:

Background: Little is known regarding oncoproteins other than platelet-derived growth factor subunit B in dermatofibrosarcoma protuberans (DFSP). Moreover, the risk factors for worse prognosis are controversial.

Objective: We sought to determine the clinicopathologic features and key factors for adverse outcome in DFSP, including the implication of expression of protein kinase B (Akt)/mammalian target of rapamycin (mTOR), signal transducer and activator of transcription 3 (STAT3), extracellular signal regulated kinase (ERK), cyclin D1, and programmed death ligand 1 (PD-L1). Read More

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November 2018
14 Reads
4.449 Impact Factor

Microphthalmia-associated transcription factor (MiTF): Promiscuous staining patterns in fibrohistiocytic lesions is a potential pitfall.

Pathol Res Pract 2018 Jun 11;214(6):821-825. Epub 2018 May 11.

Department of Pathology & Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA. Electronic address:

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. Read More

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June 2018
14 Reads
1.560 Impact Factor

A favorable outcome of advanced dermatofibrosarcoma protuberans under treatment with sunitinib after imatinib failure.

Onco Targets Ther 2018 1;11:2439-2443. Epub 2018 May 1.

Melanoma and Sarcoma Medical Oncology Unit, Sun Yat-sen University Cancer Center, Guangzhou, People's Republic of China.

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. Read More

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May 2018
5 Reads

Evaluation of p16 protein expression and CDKN2A deletion in conventional and fibrosarcomatous dermatofibrosarcoma protuberans.

Pathology 2018 Jun 7;50(4):474-475. Epub 2018 May 7.

Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, United States. Electronic address:

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