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Dermatofibrosarcoma protuberans

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
Marina Venturini, Arianna Zanca, Ausilia Maria Manganoni, Laura Pavoni, Giulio Gualdi, PierGiacomo Calzavara-Pinton
No abstract text is available yet for this article.
November 2016: Journal of the American Academy of Dermatology
Suk Hyun Bae, Ji Young Lee
Dermatofibrosarcoma protuberans is an uncommon malignant mesenchymal tumor of the dermis, which most commonly appears on the body and extremities. A preoperative diagnosis of dermatofibrosarcoma protuberans is extremely difficult, because it is a very rare entity and its appearance is often similar to that of benign breast lesion. Here, we presented a case of breast dermatofibrosarcoma protuberans with associated radiological mammography, ultrasound, magnetic resonance imaging and fludeoxyglucose-positron emission tomography computerized tomography (FDG-PET CT) features...
April 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
J Wu, K X Du, J Y He, X H Liao, J Li
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Adriana Martín-Fuentes, Esther De Eusebio-Murillo, Consuelo Sánchez Herreros, Adrian Ballano-Ruiz, Esther Jiménez Blázquez, Jesús Cuevas-Santos
BACKGROUND: Dermatofibrosarcoma protuberans is a rare malignant tumor with a high rate of recurrence after surgery. Moh's micrographic surgery allows examination of all surgical margins to ensure complete removal. OBJECTIVE: To evaluate the use of Moh's micrographic surgery using paraffin-embedded sections for the treatment of dermatofibrosarcoma protuberans. METHODS: We performed a retrospective analysis of 33 patients with dermatofibrosarcoma protuberans treated in our department with paraffin-embedded micrographic surgery between January 2002 and June 2015...
September 19, 2016: Indian Journal of Dermatology, Venereology and Leprology
Chenthuran Deivaraju, H Thomas Temple, Norman Block, Philip Robinson, Andrew V Schally
AIM: Luteinizing hormone releasing hormone (LHRH) is a neurohormone, secreted by the hypothalamus, which regulates the secretion of gonadotropins, luteinizing hormone (LH) and follicle stimulating hormone (FSH) from the pituitary. LHRH acts by binding to receptors located in the pituitary gland. These receptors (LHRH receptors) have also been found in the cytoplasm of many tumor cells that involve both the reproductive and non-reproductive organs. These receptors have been demonstrated in prostate and breast cancers, endometrial carcinomas, renal cell carcinoma, lymphoma, carcinoma of liver, pancreas and skin...
August 17, 2016: Hormone Molecular Biology and Clinical Investigation
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
Eugene Y Fukudome, Deepak R Bharadia, Douglas L Helm, Indranil Sinha
Large posterior upper trunk defects can be challenging to reconstruct. Trapezius or latissimus dorsi myocutaneous flaps are principally utilized for reconstruction; however, some defects may not be amenable to this standard approach. Here, we describe a patient with a full-thickness skin and subcutaneous tissue loss of the upper back and inferior cervical region after dermatofibrosarcoma protuberans resection. A large, extended V-Y flap was used for closure of this wound secondary to its location, size, and orientation...
May 2016: Plastic and Reconstructive Surgery. Global Open
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
Gayathri P Amonkar, Asha Rupani, Ajay Shah, Ramesh Deshpande
Bednar tumor is an uncommon variant of dermatofibrosarcoma protuberans. Also known as pigmented dermatofibrosarcoma protuberans, this tumor is of intermediate grade. It is seen in adults and has a predisposition to affect the shoulder region. We report a rare case of Bednar tumor in a 40-year-old female patient. The diagnosis of Bednar tumor must be considered while reporting pigmented subcutaneous spindle cell lesions.
April 2016: Dermatopathology (Basel, Switzerland)
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
Ravin Ratan, Shreyaskumar R Patel
Soft tissue sarcoma is a term used to describe a heterogeneous group of many rare tumors. Since the initial description of activity of doxorubicin, several additional agents have been brought to bear in the treatment of these diseases. Despite 2 recent drug approvals, doxorubicin and ifosfamide remain the most effective chemotherapy drugs available for the treatment of majority of these tumors. Optimal dosing and administration influence outcomes because of the steep dose-response curves associated with these agents...
October 2016: Cancer
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