Atypical fibroxanthoma and pleomorphic dermal sarcoma: A reappraisal

Cesinaro, Anna Maria; Gallo, Graziana; Tramontozzi, Sabrina; Migaldi, Mario

doi:10.1111/cup.13787

Cited by 23 publications

(31 citation statements)

References 20 publications

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“…The resection was planned based on the clinical appearance, comorbidities, imaging, and pathology reports. The ultimate goal, however, must be clear margins [ 23 ], as incomplete initial resection causes local recurrence [ 24 , 25 ].…”

Section: Discussionmentioning

confidence: 99%

Oncological Safety and Recurrence in the Surgical Treatment of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma of the Scalp

Zeller

Kiefer

Braig

et al. 2022

Cancers

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Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are two distinct designations for a rare dermal sarcoma entity. These tumors arise predominantly in the sun-damaged skin of elderly patients. Although both AFX and PDS have a similar clinical presentation and nearly identical genetic features, they significantly differ in prognosis. Here we present a retrospective single-center chart review analyzing the outcomes of patients treated for dermal sarcoma. The radicality of the tumor-resection extent and soft-tissue reconstructive options were assessed. Patients between January 2010 and August 2021 were included. We recorded resection margins, tumor recurrence, overall survival, number of operations until complete tumor resection, and reconstructive procedures; any complications were recorded. Furthermore, we analyzed a subgroup of patients with satellite metastases. A total of 32 patients met the inclusion criteria (30 male, 2 female, median age of 77.5 years (interquartile range (IQR) 74–81)). Histopathology revealed AFX in 14 patients and PDS in 18 patients. Margin-free resection was achieved in 31 cases, and 27 patients were remission free over the reported period. The local recurrence rate was 5, and distant metastasis was detected in four cases. Of all the PDS cases, nine presented with satellite metastasis. No AFX had satellite metastases. Due to their rarity, managing these tumors requires an interdisciplinary setting in a specialized sarcoma center.

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Section: Discussionmentioning

confidence: 99%

Oncological Safety and Recurrence in the Surgical Treatment of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma of the Scalp

Zeller

Kiefer

Braig

et al. 2022

Cancers

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“…This notion is further supported by recent studies that have failed to show any difference between AFX and PDS for genetic mutations, oncogene expression profiles, and DNA methylation profiling. [5][6][7] More than half a century ago, Helwig believed AFX was benign or a pseudosarcoma. 8,9 Although some authors still hold this benign conviction, it is clear that AFX is better considered as a low-grade malignancy.…”

Section: Pleomorphic Dermal Sarcoma Does Not Represent An Entity Diff...mentioning

confidence: 99%

Pleomorphic Dermal Sarcoma Does Not Represent an Entity Different From Atypical Fibroxanthoma

Chen

Asgari

Chen³

2022

The American Journal of Dermatopathology

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Palisaded neutrophilic and granulomatous dermatitis/ interstitial granulomatous dermatitis overlap: a striking clinical and histologic presentation with "burning rope sign" and subsequent mirror-image contralateral recurrence. Am J Dermatopathol. 2017;39:e141-e146. 6. Chu P, Connolly MK, LeBoit PE. The histopathologic spectrum of palisaded neutrophilic and granulomatous dermatitis in patients with collagen vascular disease.

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“…AFX/DUPS: As for all PDS, initial clear resection margins are the gold standard of surgery, as incomplete initial resection causes local recurrence [ 59 , 60 ]. No guidelines on surgical management and follow-up exist.…”

Section: Treatment Algorithms and Strategiesmentioning

confidence: 99%

A Multidisciplinary Approach to Complex Dermal Sarcomas Ensures an Optimal Clinical Outcome

Trøstrup

Bigdeli

Krogerus

et al. 2022

Cancers

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Primary dermal sarcomas (PDS) belong to a highly clinically, genetically and pathologically heterogeneous group of rare malignant mesenchymal tumours primarily involving the dermis or the subcutaneous tissue. The tumours are classified according to the mesenchymal tissue from which they originate: dermal connective tissue, smooth muscle or vessels. Clinically, PDS may mimic benign soft tissue lesions such as dermatofibromas, hypertrophic scarring, etc. This may cause substantial diagnostic delay. As a group, PDS most commonly comprises the following clinicopathological forms of dermal sarcomas: dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), dermal undifferentiated pleomorphic sarcoma (DUPS), leiomyosarcoma (LMS), and vascular sarcomas (Kaposi’s sarcoma, primary angiosarcoma, and radiation-induced angiosarcoma). This clinical entity has a broad spectrum regarding malignant potential; however, local aggressive behaviour in some forms causes surgical challenges. Preoperative, individualised surgical planning with complete free margins is pivotal along with a multidisciplinary approach and collaboration across highly specialised surgical and medical specialties. The present review gives a structured overview of the most common forms of dermal sarcomas including surgical recommendations and examples for advanced reconstructions as well as the current adjunctive medical treatment strategies. Optimal aesthetic and functional outcomes with low recurrence rates can be achieved by using a multidisciplinary approach to complex dermal sarcomas. In cases of extended local tumour invasion in dermal sarcomas, advanced reconstructive techniques can be applied, and the interdisciplinary microsurgeon should be an integral part of the sarcoma board.

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Atypical fibroxanthoma and pleomorphic dermal sarcoma: A reappraisal

Cited by 23 publications

References 20 publications

Oncological Safety and Recurrence in the Surgical Treatment of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma of the Scalp

Oncological Safety and Recurrence in the Surgical Treatment of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma of the Scalp

Pleomorphic Dermal Sarcoma Does Not Represent an Entity Different From Atypical Fibroxanthoma

A Multidisciplinary Approach to Complex Dermal Sarcomas Ensures an Optimal Clinical Outcome

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