Current Update on the Molecular Biology of Cutaneous Sarcoma: Dermatofibrosarcoma Protuberans

Iwasaki, Takeshi; Yamamoto, Hidetaka; Oda, Yoshinao

doi:10.1007/s11864-019-0628-3

Cited by 28 publications

(40 citation statements)

References 74 publications

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“…This finding supports a prognostic role of cell proliferation in DFSF and has potential clinical implications not only as a risk stratification marker but also as a potential therapeutic target [15]. While there is a scarcity of published data on the prognostic value of Ki-67 immunostaining in DFSP, an association between Ki-67 and histology type has been suggested [12,16,17]. Agarwal et al [12] have shown that dermatofibromas have a higher proliferation index when compared to the superficial/peripheral portion of DFSP.…”

Section: Discussionsupporting

confidence: 62%

The Ki-67 proliferation index predicts recurrence-free survival in patients with dermatofibrosarcoma protuberans

Adem

Yazıcı

Özşen

et al. 2020

Bosn J of Basic Med Sci

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Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue sarcoma that originates from the dermis or subcutaneous tissue in the skin. While its prognosis is generally favorable, disease recurrence is relatively frequent. Because morbidity after repeated surgery may be significant, an optimized prediction of recurrence-free survival (RFS) has the potential to improve current management strategies. The purpose of this study was to investigate the prognostic value of the Ki-67 proliferation index with respect to RFS in patients with DFSP. We retrospectively analyzed data from 45 patients with DFSP. We calculated the Ki-67 proliferation index as the percentage of immunostained nuclei among the total number of tumor cell nuclei regardless of the intensity of immunostaining. We constructed univariate and multivariate Cox proportional hazards regression models to identify predictors of RFS. Among the 45 patients included in the study, 8 developed local recurrences and 2 had lung metastases (median follow-up: 95.0 months; range: 5.2−412.4 months). The RFS rates at 60, 120, and 240 months of follow-up were 83.8%, 76.2%, and 65.3%, respectively. The median Ki-67 proliferation index was 14%. Notably, we identified the Ki-67 proliferation index as the only independent predictor for RFS in multivariate Cox proportional hazards regression analysis (hazard ratio = 1.106, 95% confidence interval = 1.019−1.200, p = 0.016). In summary, our results highlight the potential usefulness of the Ki-67 proliferation index for facilitating the identification of patients with DFSP at higher risk of developing disease recurrences.

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Section: Discussionsupporting

confidence: 62%

The Ki-67 proliferation index predicts recurrence-free survival in patients with dermatofibrosarcoma protuberans

Adem

Yazıcı

Özşen

et al. 2020

Bosn J of Basic Med Sci

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“…Likewise, lack of comprehensive follow-up data made it difficult to undertake survival statistics. Despite these limitations, apart from the population being a black race, other known poorer prognostic factors have been documented in the present study are a predominant male gender and large tumour size [ [2,3,6].…”

Section: Discussionmentioning

confidence: 88%

“…Dermatofibrosarcoma protuberance is the most common dermal sarcoma of unresolved histogenesis and seen more frequently among middle-aged individuals [1,2]. It is a rare neoplasm with an incidence of about 4.1 per million person years among the American population [3].…”

Section: Introductionmentioning

confidence: 99%

“…It, however, rarely metastasizes. It is, therefore, regarded as a low-grade sarcoma with excellent 10 year survival of 99% [2,6].Studies on dermatofibrosarcoma protuberance from Nigeria are few [7], hence the need to undertake this study. More so, cases of missed diagnosis are frequent with patients often presenting as a recurrence before accurate diagnosis is made [8,10].…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Dermatofibrosarcoma protuberance in a black African cohort – a clinicopathologic study

Ogun

Ezenkwa

Ayandipo

2020

ecancer

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Background: Dermatofibrosarcoma protuberance (DFSP) is the commonest, yet rare, dermal sarcoma globally. There are few reports in the literature of this neoplasm in Nigerians and indeed in sub-Saharan Africa. This study documents our institutional practice observation and compares it with those from other regions of the world. Methods and materials: This study was a retrospective review of all cases of histologically diagnosed DFSP at the University College Hospital, Ibadan, Nigeria, spanning a period of 27 years (January 1989-December 2016). Data on patient age, gender, tumour location, size, tumour recurrence and metastasis status were obtained from clinical and surgical pathology archival files and records. Results: Sixty-nine cases of DFSP were recorded over the period reviewed with a malefemale ratio of 1.6:1. The mean age of the study population was 39.6 years. The youngest patient was 5-year old, while the oldest was 86 years and the modal age group was the 4th decade. The trunk was the commonest anatomic tumour location. Recurrences were seen in seven cases with recurrence interval ranging from 6 to 240 months. The correlation between tumour size and age was non-significant (r = −0.183; p = 0.182). There was fibrosarcoma-like transformation in three cases (4.3%) studied. Conclusion: Dermatofibrosarcoma protuberance is rare in our population and occurs more commonly in males and on the trunk. Recurrence can occur beyond the recommended follow-up period of 10 years.

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Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease—Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)

Krewer

Rolle

Kościelniak

et al. 2020

Journal of Surgical Oncology

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Background Dermatofibrosarcoma protuberans (DFSP) is a rare low‐grade tumor. Little is known about best treatment of primary and relapsed disease (RD). Methods Treatment and outcome of 40 patients with DFSP prospectively registered within the CWS‐96 and ‐2002P trials and the registry SoTiSaR (1996‐2016) were analysed. Results Median age was 8 years (range, 0.64‐17.77). Fluorescence in situ hybridization analysis to detect COL1A1‐PDGFB fusion genes was positive in 86% (12/14) of evaluated patients. Primary resection was performed in all patients. Patients had IRS group I (n = 28), II (n = 9), and III (n = 2); not available (n = 1). To achieve complete remission (CR), a secondary resection was performed in 18 patients resulting in microscopically complete (R0, n = 34/40) and microscopically incomplete (R1, n = 5/40) resection. All patients achieved CR. The 5‐year event‐free survival (EFS) and overall survival was 86% (±12; CI, 95%) and 100% (±0; CI, 95%), respectively. R0 resection/IRS I was significantly favorable for the 5‐year EFS. Local relapse occurred after a median time of 1.1 years (range, 0.04‐5.1) in 15% (6/40) after CR. All patients with RD underwent resection and achieved CR. Three patients had fibrosarcomatous DFSP, two were alive after R0 resection. Conclusion Complete surgical resection is mandatory to prevent relapse of DFSP.

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Current Update on the Molecular Biology of Cutaneous Sarcoma: Dermatofibrosarcoma Protuberans

Cited by 28 publications

References 74 publications

The Ki-67 proliferation index predicts recurrence-free survival in patients with dermatofibrosarcoma protuberans

The Ki-67 proliferation index predicts recurrence-free survival in patients with dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberance in a black African cohort – a clinicopathologic study

Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease—Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)

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