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Tony Ht Sung, Annie Cw Tam, Jennifer Ls Khoo
Dermatofibrosarcoma Protuberans (DFSP) is a rare malignant soft-tissue neoplasm which is often misdiagnosed due to its indolent clinical course and non-specific radiological appearances. An observation case series was conducted with retrospective review of clinical and radiological data of DFSP patients presenting to a major tertiary hospital in Hong Kong for radiological assessment between November 2006 and February 2016. Seven patients with confirmed histological diagnosis of DFSP were included. Tumour sizes at presentation ranged from 1 to 5 cm, most commonly (n = 6) occurred over chest wall and abdominal wall...
October 18, 2016: Journal of Medical Imaging and Radiation Oncology
Garrett C Lowe, Oluwakemi Onajin, Christian L Baum, Clark C Otley, Christopher J Arpey, Randall K Roenigk, Jerry D Brewer
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with slow infiltrative growth and local recurrence if inadequately excised. OBJECTIVE: To compare long-term outcomes after Mohs micrographic surgery (MMS) and wide local excision (WLE). MATERIALS AND METHODS: Records of patients with DFSP surgically treated with WLE or MMS from January 1955 through March 2012 were retrospectively reviewed. RESULTS: Mean follow-up for patients treated with MMS (n = 67) and WLE (n = 91) was 4...
October 4, 2016: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
Min Jung Kim, Min Seok Hur, Byung Gon Choi, Soo Young Kim, Yang Won Lee, Yong Beom Choe, Kyu Joong Ahn
Dermatofibrosarcoma protuberans (DFSP) is a rare disease of dermal fibroblastic origin that accounts for less than 5% of all soft tissue sarcomas in adults. DFSP grows slowly and is an asymptomatic lesion at the initial diagnosis. Herein, we report a case of multiple pedunculated nodules as a variant of DFSP. A 47-year-old man presented with a 7-month history of multiple well-circumscribed, firm, pedunculated nodules on the inguinal area. Histopathologic examination results showed densely packed uniform spindle cells with a storiform and cartwheel pattern, and positivity for CD34...
October 2016: Annals of Dermatology
J Rubió-Casadevall, A M Hernandez-Pujol, M C Ferreira-Santos, G Morey-Esteve, L Vilardell, G Osca-Gelis, N Vilar-Coromina, R Marcos-Gragera
INTRODUCTION: We present an epidemiological study focused on Non-melanoma skin cancers (NMSC), including squamous cell carcinoma (SCC), basal cell carcinoma (BCC), Merkel cell carcinoma (MCC), dermatofibrosarcoma protuberans (DFS) and adnexal and skin appendages neoplasm (ASAN), a neoplasm understudied in cancer registries. MATERIAL AND METHODS: We analyze trends of incidence and survival of NMSC registered with the Cancer Registry of Girona, Spain. RESULTS: We found 14389 cases of NMSC, accounting 3,474 SCC, 10729 BCC, 33 MCC, 61 DFSP and 71 ASAN...
September 14, 2016: Cancer Epidemiology
Marcella Tazzari, Valentina Indio, Barbara Vergani, Loris De Cecco, Francesca Rini, Tiziana Negri, Chiara Camisaschi, Marco Fiore, Silvia Stacchiotti, G Paolo Dagrada, Paolo G Casali, Alessandro Gronchi, Annalisa Astolfi, Maria A Pantaleo, Antonello Villa, Claudia Lombardo, Flavio Arienti, Silvana Pilotti, Licia Rivoltini, Chiara Castelli
Dermatofibrosarcoma protuberans (DFSP), although rare, is the most frequent skin sarcoma. Here, we focus on DFSP carrying the fibrosarcomatous transformation (FS-DFSP). FS-DFSP responds to imatinib (IM); however, tumor relapse often occurs. In a series of 21 pre- and post-treatment FS-DFSP samples, the present study explored the events that occur at the tumor site during IM therapy. Gene expression profile (GEP) and immunohistochemistry (IHC) analyses documented the occurrence of IM-induced senescence phenotype in the tumor cells and showed the accumulation of activated CD3(+) T cells and CD163(+)CD14(+) myeloid cells expressing the CD209 marker in post-therapy lesions...
September 5, 2016: Journal of Investigative Dermatology
John T Mullen
Dermatofibrosarcoma protuberans (DFSP) is a rare dermal soft tissue sarcoma characterized by a typically indolent clinical course. The greatest clinical challenge in management of DFSP is achieving local control. There is vigorous debate in the literature as to the optimal surgical approach to these tumors. The choice between wide local excision and Mohs micrographic surgery for DFSP should be governed by the attainment of three goals: (1) to completely excise the tumor with negative margins, tantamount to cure; (2) to preserve function, optimize cosmesis, and minimize morbidity of resection; and (3) to minimize cost and inconvenience to the patient and the health care system at large...
October 2016: Surgical Oncology Clinics of North America
Toshiharu Tatai, Daisuke Gomi, Toshirou Fukushima, Takashi Kobayashi, Nodoka Sekiguchi, Akiyuki Sakamoto, Shigeru Sasaki, Tomonobu Koizumi, Kenji Sano
We herein encountered a case of abdominal wall dermatofibrosarcoma protuberans (DFSP) that developed pulmonary and pancreatic metastases 5 years after complete resection. Because specific rearrangements of the platelet-derived growth factor beta (PDGFB) locus by a novel fluorescence in situ hybridization method was detected, the patient was treated with imatinib mesylate at 400 mg/day. A partial response was achieved by imatinib without any specific toxicity. Although metastatic DFSP is an extremely rare disease, an evaluation of PDGFB fusion is essential and imatinib mesylate should be considered as an optimal therapeutic choice in patients with metastatic or locally advanced DFSP...
2016: Internal Medicine
Chuan Ma, Yang Sun, Xin Yang, Qian Zhang, Chunlei Zhang, Ligang Cui
Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon malignant soft tissue tumor. Although metastasis is rare, it has significant topo-aggressive characteristics with a high recurrence rate. The common treatment of DFSP is surgical resection. This study reported a case of a 22 years old woman who presented DFSP with single irregular red plaque in the right occipital area. The plaque is about 2 cm × 2 cm in diameter with an irregular and unclear border. In order to minimize the range of resection area, the contrast-enhanced ultrasound (CEUS) was used to precisely identify the tumor affected area before the surgery treatment...
August 29, 2016: Dermatologic Therapy
Jeffrey Reha, Steven C Katz
Dermatofibrosarcoma protuberans (DFSP) is a rare superficial soft tissue sarcoma. Its rarity precludes large prospective studies. Clinical diagnosis requires an high index of suspicion. Effective management requires an appreciation of tumor biology and the nature of the characteristic infiltrative growth pattern. DFSP tends to recur locally, with a low risk of dissemination. Aggressive surgical resection with widely negative margins is essential to management. Radiotherapy may be indicated in special circumstances...
October 2016: Surgical Clinics of North America
Kathryn L Kreicher, Kord S Honda, David E Kurlander, Jeremy S Bordeaux
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which the exact etiology is unknown. Case reports exist of DFSP appearing and growing rapidly during pregnancy, suggesting a hormonal role. OBJECTIVE: Our goal was to determine the expression of estrogen receptors (ERs) and progesterone receptors (PRs) in patients with DFSP. METHODS: Archived formalin-fixed, paraffin-embedded tissue from patients with DFSP in the past 20 years at a single institution were analyzed for ER and PR using immunohistochemistry...
August 16, 2016: Journal of the American Academy of Dermatology
Aaron M Mullane, Alexis Ricci, Subhasis Misra, Nail Aydin
INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare, malignant, soft tissue neoplasm of the dermis. Tumor recurrence is common following resection, and can be locally devastating if not identified in a timely manner. We report a unique case of this rare tumor. This case poses the question of an association between basal cell carcinoma (BCC) and DFSP, and presents the possible need for increased awareness of DFSP for healthcare providers and patients with a history of non-melanoma skin cancers as well as surgical or burn scars...
2016: International Journal of Surgery Case Reports
Gimena Castro Pérez, Cintia Arias, Paula Luna, Irene Sorín, Luis Daniel Mazzuoccolo
Dermatofibrosarcoma protuberans (DFSP) is a malignant fibrohistiocytic tumor that appears exclusively on the skin. It is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence but seldom metastasizes. It may rarely occur on the head and neck accounting for less than one percent of total head and neck malignancies. We present a man with a giant DFSP on the face. Oncological, functional, and aesthetic aspects are set forth.
2016: Case Reports in Dermatological Medicine
Barkha Gupta, Nidhi Verma, Nita Khurana, Sudhir Kumar Jain
Lesions on the scalp are frequently encountered in clinical practice. Commonly diagnosed lesions include epidermal inclusion cysts (EICs), dermoid cysts and lipomas. Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant skin tumour occurring mostly in the extremities. However, its occurrence on the scalp is unusual, with an incidence of <5%. This lesion is rarely known to undergo fibrosarcomatous transformation. We present two such rare cases of DFSP of the scalp, with fibrosarcomatous transformation, masquerading clinically as EIC...
2016: BMJ Case Reports
Raghava Kashyap, Vamshi Krishna Muddu, Sameera Anantamakula, Satya Sri
Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive tumor with distant metastases being unusual. We present a case of metastatic DFSP treated with imatinib showing complete metabolic response to treatment.
July 2016: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
Melissa H Kinney, Sally M Knox
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a tendency towards local recurrence. A 26-year-old woman presented with a 3 × 2 cm raised, purple-hued lesion on her left breast. Excisional biopsy identified the lesion as a DFSP. She underwent two additional operations to achieve widely clear margins. Operative excision is the primary treatment of dermatofibrosarcoma protuberans of the breast.
July 2016: Proceedings of the Baylor University Medical Center
Imran Ahmad, Mohd Altaf Mir, Lalit Mohan Bariar, Nishat Afroz
Dermatofibrosarcoma protuberans (DFSP) is very rare tumor of dermis layer of skin with the incidence of only 1 case per million per year. DFSP rarely leads to a metastasis (Less than 5% have metastasis), but DFSP can recur locally. We publish a rare case of a recurrent dermatofibrosarcoma protuberans and its management with radical excision and interval skin grafting.
January 2016: World Journal of Plastic Surgery
Keiko Hayakawa, Seiichi Matsumoto, Keisuke Ae, Taisuke Tanizawa, Tabu Gokita, Yuki Funauchi, Noriko Motoi
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) may recur locally but rarely metastasizes. Fibrosarcomatous transformation in dermatofibrosarcoma protuberans (FS-DFSP) is said to have worse prognosis compared with ordinary DFSP (O-DFSP). Since DFSP rarely metastasizes, there have been few reports summarizing data on distant metastasis cases at single institution. The aim of this retrospective study is to review DFSP cases in order to analyze risk factors for metastasis. PATIENTS AND METHODS: This retrospective study involved 67 patients...
September 2016: Journal of Orthopaedics and Traumatology: Official Journal of the Italian Society of Orthopaedics and Traumatology
Maressa C Criscito, Kathryn J Martires, Jennifer A Stein
Importance: There is limited information regarding the influence of patient demographics, tumor characteristics, and treatment type on the survival of patients with dermatofibrosarcoma protuberans (DFSP). Objective: To assess prognostic factors and to evaluate the influence of treatment modality on overall survival of patients with DFSP. Design, Setting, and Participants: We examined DFSP using data for 3686 patients with histologically confirmed cases of DFSP diagnosed between 1972 and 2012 from the 18 US regional registries of the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Program, with linkage to demographic data from the US Census Bureau for median household income (MHI)...
June 4, 2016: JAMA Dermatology
Silvia Stacchiotti, Annalisa Astolfi, Alessandro Gronchi, Andrea Fontana, Maria A Pantaleo, Tiziana Negri, Monica Brenca, Marcella Tazzari, Milena Urbini, Valentina Indio, Chiara Colombo, Stefano Radaelli, Silvia Brich, Angelo P Dei Tos, Paolo G Casali, Chiara Castelli, Gian Paolo Dagrada, Silvana Pilotti, Roberta Maestro
UNLABELLED: Dermatofibrosarcoma protuberans (DFSP) is a rare and indolent cutaneous sarcoma. At times, a fibrosarcomatous transformation marked by a more aggressive clinical behavior may be present. We investigated the natural history and the molecular bases of progression from classic DFSP to the fibrosarcomatous form (FS-DFSP), looking, retrospectively, at the outcome of all patients affected by primary DFSP treated at our institution from 1993 to 2012 and analyzing the molecular profile of 5 DFSPs and 5 FS-DFSPs by an integrated genomics approach (whole transcriptome sequencing, copy number analysis, FISH, qRT-PCR, IHC)...
September 2016: Molecular Cancer Research: MCR
Aliya Sarhan Al Barwani, Sawsan Taif, Reem Ahmed Al Mazrouai, Khamis Salim Al Muzahmi, Asif Alrawi
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tumor which originally represents a cutaneous sarcoma. It grows slowly and presents usually as nodular superficial lesion on the trunk or the extremities. Although these tumors are locally aggressive with high rate of recurrence following surgery; the prognosis is considered excellent when it is effectively treated. The radiological appearance of this tumor has rarely been studied and findings infrequently discussed in the literature probably because many lesions underwent resection before imaging...
2016: Journal of Clinical Imaging Science
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