Pleomorphic dermal sarcoma: a more aggressive neoplasm than previously estimated

Tardío, Juan C.; Pinedo, Fernando; Aramburu, J. A.; Suárez-Massa, Dolores; Pampín, A.; Requena, Luis; Santonja, Carlos

doi:10.1111/cup.12603

Cited by 104 publications

(136 citation statements)

References 40 publications

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“…Surgical excision margins were evaluated by macroscopic assessment of the surgeon and documented in the medical records. The mitotic index was evaluated as previously described . Clinicopathologic data were collected from the medical records of the patients and analysed using the statistical analysis software package IBM SPSS, version 20.0 (Chicago, IL, USA).…”

Section: Methodssupporting

confidence: 93%

“…Pleomorphic dermal sarcomas (PDS) are frequent sarcomas of the skin of intermediate grade malignant potential developing in UV‐exposed locations. PDS locally recur in up to 28% and have the potential for distant metastasis and disease‐specific death in up to 20% of cases …”

Section: Introductionmentioning

confidence: 99%

“…Despite the fact that PDS have a noteworthy potential to recur as well as to metastasize, there are no clinical guidelines for the follow‐up of these patients. There are only a few small descriptive case series, which reported associations between incomplete excision, necrosis, lymphovascular as well as perineural invasion of the PDS and its propensity to metastasis . However, due to the small sample size and limited patient follow‐up, statistical analysis of prognostic factors was not conducted in these studies.…”

Section: Introductionmentioning

confidence: 99%

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Extended surgical safety margins and ulceration are associated with an improved prognosis in pleomorphic dermal sarcomas

Persa

Loquai

Wobser

et al. 2019

Acad Dermatol Venereol

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Background Pleomorphic dermal sarcomas (PDS) are frequent UV‐induced sarcomas of the skin of intermediate grade malignant potential. Despite the fact that PDS have a noteworthy potential to recur (up to 28%) as well as to metastasize (up to 20%), there are no specific clinical guidelines with respect to follow‐up these patients. Moreover, little is known about clinical, histological or molecular prognostic factors in PDS. Objective The aim of the present study was to identify risk factors to predict relapse in a large multicentre sample cohort of PDS which could aid to optimize personalized treatment recommendations regarding surgical safety margins and adjuvant radiotherapy. Methods Patients with a diagnosis of PDS were selected from nine European institutions based on the histopathologic criteria described by Fletcher. Clinicopathologic and follow‐up data were collected and statistically analysed calculating univariate hazard ratios with 95% confidence intervals by use of the Cox proportional‐hazards model and a significance level of P < 0.05. Patients with an incomplete excision of the tumour were excluded. Results Univariate Cox regression analysis of possible prognostic factors for progression‐free survival (PFS) performed in 92 patients revealed that an excision margin of <2 cm is significantly associated with relapse of PDS [hazard ratio 4.478 (95% CI 1.536–13.055), P = 0.006]. Ulceration of the tumour was associated with a significantly better prognosis [0.396 (0.174–0.904), P = 0.028] whereas adjuvant radiotherapy did not reach statistical significance to improve prognosis in patients with PDS [0.775 (0.231–2.593), P = 0.679]. Gender, age, immunosuppression, intratumoural necrosis, tumour location, vertical thickness or horizontal diameter did not significantly influence PFS in PDS. Conclusion We identified surgical safety margins of <2 cm and absence of ulceration as risk factors for relapse in patients with PDS. These findings may be implemented into both the primary treatment as well as the further monitoring of patients with PDS.

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Section: Methodssupporting

confidence: 93%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Extended surgical safety margins and ulceration are associated with an improved prognosis in pleomorphic dermal sarcomas

Persa

Loquai

Wobser

et al. 2019

Acad Dermatol Venereol

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“…The prognosis of PDS remains better than that of undifferentiated pleomorphic sarcomas of other body sites. Local recurrence rates, usually in the setting of incomplete excision, have been reported to be in the range of 20–28% . The incidence of metastatic disease now appears to be higher than initially suggested, with distant metastases occurring in 10% and 20% of cases in two recent studies …”

Section: Refinements In Classification Of Cutaneous Mesenchymal Neoplmentioning

confidence: 98%

“…Pleomorphic dermal sarcoma (PDS), also known as unclassified pleomorphic sarcoma of skin, and formerly designated cutaneous malignant fibrous histiocytoma, is a relatively uncommon dermal mesenchymal neoplasm that shows some clinical and histological similarities to AFX, but that differs significantly with regard to clinical behaviour. AFX is confined to the dermis, whereas tumours with similar histological features to those of AFX but that show extension into subcutaneous tissue are designated PDS and are associated with risks of local recurrence and distant metastasis of up to 30% and 20%, respectively . When strict diagnostic criteria are applied, AFX is a benign tumour with a low risk of local recurrence and no metastatic potential …”

Section: Refinements In Classification Of Cutaneous Mesenchymal Neoplmentioning

confidence: 99%

Advances in the clinicopathological and molecular classification of cutaneous mesenchymal neoplasms

Costigan

Doyle

2016

Histopathology

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In recent years, there have been several important refinements in the classification of cutaneous mesenchymal neoplasms, including the description of new tumour types, along with the identification of novel and recurrent molecular genetic findings. In addition to providing new insights into tumour biology, many of these advances have had significant clinical consequences with regard to diagnostics, management, and prognostication. Newly described entities include pseudomyogenic haemangioendothelioma, haemosiderotic fibrolipomatous tumour, and fibroblastic connective tissue naevus, which are reviewed in the context of the principal differential diagnoses and significant clinical implications. Genetic characterization of several soft tissue tumour types that occur in the skin has resulted in the identification of diagnostically useful markers: ALK gene rearrangement with corresponding ALK protein expression by immunohistochemistry in epithelioid fibrous histiocytoma; the WWTR1-CAMTA1 fusion gene with CAMTA1 protein expression in epithelioid haemangioendothelioma; MYC amplification and overexpression in radiation-associated angiosarcoma; and EWSR1 gene rearrangement in cutaneous myoepithelial tumours. Finally, the classification of intradermal smooth muscle tumours and unclassified/pleomorphic dermal sarcoma has been refined, resulting in both improved classification and improved prognostication. Many of the tumour types listed above are encountered not only by specialist dermatopathologists, but also by practising general surgical pathologists, and this review should therefore provide a widely applicable update on the histological and molecular classification of cutaneous mesenchymal neoplasms, along with the appropriate use of ancillary diagnostic tests, in particular immunohistochemistry, in the evaluation of such lesions and their histological mimics.

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Connective Tissue

2018

Atlas of Dermatopathology

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Pleomorphic dermal sarcoma: a more aggressive neoplasm than previously estimated

Abstract: Our data suggest that PDS may be a more aggressive neoplasm than previously estimated.

Cited by 104 publications

References 40 publications

Extended surgical safety margins and ulceration are associated with an improved prognosis in pleomorphic dermal sarcomas

Extended surgical safety margins and ulceration are associated with an improved prognosis in pleomorphic dermal sarcomas

Advances in the clinicopathological and molecular classification of cutaneous mesenchymal neoplasms

Connective Tissue

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