scholarly journals Dermatofibrosarcoma Protuberans: A Systematic Review of the Literature

Author(s):  
Alaa Alnooh ◽  
Yasser Al-Qubaisy ◽  
Nujud Alshammari ◽  
Ibrahim Alhowshan

Background: Dermatofibrosarcoma Protuberans (DFSP) is a rare slow growing cutaneous soft tissue sarcoma, that is characterized to be locally invasive with high recurrence rate. However, it has poor metastatic capacity. Objective: We are conducting this literature review to gather evidence-based medicine knowledge regarding Dermatofibrosarcoma Protuberans. Moreover, this study highlights the gaps of knowledge in regard to this disease entity. Methods: The literature search was conducted by searching the keywords “Dermatofibrosarcoma Protuberans” and “soft tissue sarcomas” in PubMed and Web of Science databases. Results: 1,769 potentially relevant results showed matched titles, of which 13 articles met the requirements and were included in the literature review. Limitations: Vague nonspecific clinical manifestations, makes it challenging to establish an early diagnosis and seek an appropriate counseling.  In addition, it is difficult to diagnose due to the slow growing behavior and the tumor’s benign appearance. As a result of all of that there is no enough cases reported in the literature. Conclusion: This review highlights the need of high index of suspicion to help reach an early and proper diagnosis and to raise awareness. Dermatofibrosarcoma Protuberans needs a specialized center in order to identify and recognize the disease. The knowledge and awareness of the disease is low since it is rare.

2015 ◽  
Vol 14 (1) ◽  
pp. 64-66
Author(s):  
Magno Almeida Nogueira ◽  
Guilherme Campelo Lopes dos Santos ◽  
Roberto Iglesias Lopes ◽  
Octavio Henrique Arcos Campos ◽  
Marcos Francisco Dall'Oglio ◽  
...  

Urethral tumors are rare and aggressive. They usually affect men (2:1) and occur more commonly in white (85% of cases). Soft tissue sarcomas are a heterogeneous group of tumors that arise from embryonic mesoderm. It represents 1% of all cases of urinary tract malignancies and rarely primary affect the ureter. We report a case of male urethral sarcoma. To date, only two similar cases have been published in literature.


2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
Matthew F. Ryan ◽  
Brandon Allen

Lipomas are slow-growing benign soft-tissue tumors which are typically asymptomatic and occur in approximately 1% of the population. A lipoma is considered to be of excessive size when it is greater than 10 cm in length (in any dimension) or weighs over 1000 g (Kransdorf (1995)). We describe a case of a man presenting with a giant posterior neck mass which greatly reduced the sagittal range of cervical spine. A discussion of the pathophysiology of lipomas and a literature review regarding giant lipomas versus malignancy follows.


2021 ◽  
Vol 13 (3) ◽  
pp. 16-26
Author(s):  
A. A. Fedenko ◽  
A. A. Tararykova

Soft tissue sarcomas are a rare heterogeneous group of malignant tumors with mesenchymal nature. Soft tissue sarcomas accounts for less than 1 % of all cancers. Low efficiency of chemotherapy for soft tissue sarcomas, especially in advanced disease, is a burning issue. Trabectedin is one of the active agents approved for the treatment of advanced soft tissue sarcomas after the failure of standard chemotherapy. The article provides a literature review of the latest world-wide data of the effectiveness and role of trabectedin in the modern approach of soft tissue sarcomas treatment.


2020 ◽  
Vol 13 (1) ◽  
pp. e232614 ◽  
Author(s):  
Durga Sowgandhi Chilukuri ◽  
Prithviraj Premkumar ◽  
Balasubramanian Venkitaraman ◽  
Jagadesh Chandra Bose Soundararajan

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue malignancy which is locally aggressive, slow growing. It has a very low metastatic potential and has high risk of local recurrence. We report a 65-year-old man with recurrent DFSP of thigh with pancreatic metastasis. Apart from our patient, only four other cases of pancreatic metastasis of DFSP have been reported. Our patient had a solitary metastasis to pancreas and was treated with distal pancreaticosplenectomy. Outcome of the patient was good. We present this case report to emphasise that resection may be considered for solitary metastasis of DFSP and can be managed successfully.


2019 ◽  
Vol 9 ◽  
Author(s):  
Francesco Pierantoni ◽  
Marco Maruzzo ◽  
Antonella Brunello ◽  
Benedetta Chiusole ◽  
Grazia Pusole ◽  
...  

2019 ◽  
Vol 6 (3) ◽  
pp. 6
Author(s):  
Daniel Alexander Reikher ◽  
Mark Feldman

Clinical manifestations of cancer can be categorized as resulting from direct tissue injury from the primary tumor, distant metastatic spread, or aberrant biological activity, also known as a paraneoplastic syndrome. Soft tissue sarcomas are a rare group of malignant tumors of mesenchymal origin which typically present with direct tissue injury, exerting their harmful potential by compression and mass effect. We describe a rare case of an occult retroperitoneal soft tissue sarcoma presenting with marantic endocarditis. To date, there is a paucity of available medical literature relating sarcoma to marantic endocarditis.


2001 ◽  
Vol 110 (10) ◽  
pp. 985-987 ◽  
Author(s):  
Ishrat Hakim

This is the first case report of atypical fibroxanthoma in the otolaryngology literature. Atypical fibroxanthoma is a rare fibrohistiocytic tumor that occurs on actinically damaged skin. The typical presentation is a nodular lesion in sun-exposed areas of the head and neck in an elderly patient. Differentiating atypical fibroxanthoma from other soft tissue sarcomas, such as dermatofibrosarcoma protuberans and malignant fibrous histiocytoma, can be difficult. Treatment should be wide local excision with a 1-cm margin. In cosmetically important regions in which tissue preservation is important, Mohs' surgery should be considered.


2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
S. D. Nelen ◽  
F. J. Vogelaar ◽  
F. Gilissen ◽  
J. C. Van der Linden ◽  
K. Bosscha

Introduction. Soft tissue sarcomas (STSs) represent 1 percent of all adult malignancies and sarcomas only rarely spread to the regional lymph nodes.Case Presentation. We present a case of a woman with a dermatofibrosarcoma protuberans and a sarcoma not therwise specified of the lower extremity. The patient had no distant metastasis during follow-up, but did develop a regional lymph nodemetastasis (RLNM) in the groin. We reviewed the literature about RLNM in STSs.Discussion. Reviewing the literature we see that within specific histological types RLNM occurs as often as distant metastasis. Furthermore RLNM occurs in over 10% for specific histological types and in 24% of all patients with a soft tissue sarcoma of the lower extremity. Except for radical lymphadenectomy with a 5-year survival rate of 46% there is no appropriate treatment.Conclusion. The risk for a RLNM in certain histological types and anatomical locations might transcend the risk for a distant lung metastasis.


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