Oncological Safety and Recurrence in the Surgical Treatment of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma of the Scalp
by
, , ,
Johannes Zeller
1,*
,
Jurij Kiefer
1,
David Braig
1,2,
Oscar Winninger
1,
Daniel Kraus
1,
Salome Hagelstein
1 and
Steffen U. Eisenhardt
1
1
Department of Plastic and Hand Surgery, Medical Faculty of the University of Freiburg, University of Freiburg Medical Center, 79106 Freiburg, Germany
2
Division of Hand, Plastic and Aesthetic Surgery, University Hospital, LMU Munich, 80336 Munich, Germany
*
Author to whom correspondence should be addressed.
Cancers 2022, 14(12), 2869; https://doi.org/10.3390/cancers14122869
Submission received: 6 April 2022
/
Revised: 6 June 2022
/
Accepted: 9 June 2022
/
Published: 10 June 2022
(This article belongs to the Special Issue Soft and Hard Tissue Sarcoma—Current and Future Concepts in Diagnosis and Treatment)
Simple Summary
Atypical fibroxanthoma and pleomorphic dermal sarcoma represent two tumors on the spectrum of a rare dermal sarcoma entity. Close clinical presentation and nearly identical histologic features but distinct prognoses make proper treatment strategies challenging. We performed a retrospective analysis of 32 patients with AFX or PDS in the scalp to provide guidance regarding the extent of radical excision to achieve stable oncological outcomes and whether radical tumor resection on the scalps required complex soft-tissue reconstruction. Compared to AFX, PDS shows a more aggressive growth pattern with frequent satellite metastases and distant metastases. These require extensive resections for local control to achieve long-term remission in most PDS patients. Despite the limited elasticity of the scalp, plastic reconstructive procedures can obtain reliable soft tissue reconstruction, even for complete scalp defects. Due to their rarity, managing these tumors requires an interdisciplinary setting in a specialized sarcoma center.
Abstract
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.
