Epigenetics of Cutaneous Sarcoma

Epigenetic changes influence various physiological and pathological conditions in the human body. Recent advances in epigenetic studies of the skin have led to an appreciation of the importance of epigenetic modifications in skin diseases. Cutaneous sarcomas are intractable skin cancers, and there are no curative therapeutic options for the advanced forms of cutaneous sarcomas. In this review, we discuss the detailed molecular effects of epigenetic modifications on skin sarcomas, such as dermatofibrosarcoma protuberans, angiosarcoma, Kaposi’s sarcoma, leiomyosarcoma, and liposarcoma. We also discuss the application of epigenetic-targeted therapy for skin sarcomas.

Dermatofibrosarcoma Protuberans: A Systematic Review of the Literature

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.

A comparison of clinical outcomes between additional excision after unplanned and planned excisions in patients with soft-tissue sarcoma of the limb

Aims Patients with soft-tissue sarcoma (STS) who undergo unplanned excision (UE) are reported to have worse outcomes than those who undergo planned excision (PE). However, others have reported that patients who undergo UE may have similar or improved outcomes. These discrepancies are likely to be due to differences in characteristics between the two groups of patients. The aim of the study is to compare patients who underwent UE and PE using propensity score matching, by analyzing data from the Japanese Bone and Soft Tissue Tumor (BSTT) registry. Methods Data from 2006 to 2016 was obtained from the BSTT registry. Only patients with STS of the limb were included in the study. Patients with distant metastasis at the initial presentation and patients with dermatofibrosarcoma protuberans and well-differentiated liposarcoma were excluded from the study. Results A total of 4,483 patients with STS of the limb were identified before propensity score matching. There were 355 patients who underwent UE and 4,128 patients who underwent PE. The five-year disease-specific survival (DSS) rate was significantly better in the patients who received additional excision after UE than in those who underwent PE. There was no significant difference in local recurrence-free survival (LRFS) between the two groups. After propensity score matching, a new cohort of 355 patients was created for both PE and UE groups, in which baseline covariates were appropriately balanced. Reconstruction after tumour excision was frequently performed in patients who underwent additional excision after UE. There were no significant differences in DSS and LRFS between the patients who underwent PE and those who had an additional excision after UE. Conclusion Using propensity score matching, patients with STS of the limb who underwent additional excision after UE did not experience higher mortality and local failure than those who underwent PE. Reconstruction may be necessary when additional excision is performed. Cite this article: Bone Joint J 2021;103-B(12):1809–1814.