Zeller Johannes, Kiefer Jurij, Braig David, Winninger Oscar, Kraus Daniel, Hagelstein Salome, Eisenhardt Steffen U
Department of Plastic and Hand Surgery, Medical Faculty of the University of Freiburg, University of Freiburg Medical Center, 79106 Freiburg, Germany.
Division of Hand, Plastic and Aesthetic Surgery, University Hospital, LMU Munich, 80336 Munich, Germany.
Cancers (Basel). 2022 Jun 10;14(12):2869. doi: 10.3390/cancers14122869.
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.
非典型纤维黄色瘤（AFX）和多形性皮肤肉瘤（PDS）是一种罕见的皮肤肉瘤实体的两种不同命名。这些肿瘤主要发生在老年患者受阳光损伤的皮肤中。尽管AFX和PDS具有相似的临床表现和几乎相同的基因特征，但它们在预后方面有显著差异。在此，我们进行了一项回顾性单中心病历审查，分析了皮肤肉瘤患者的治疗结果。评估了肿瘤切除范围的根治性和软组织重建方案。纳入了2010年1月至2021年8月期间的患者。我们记录了切缘、肿瘤复发、总生存期、直至肿瘤完全切除的手术次数以及重建手术；记录了任何并发症。此外，我们分析了一组有卫星转移的患者亚组。共有32例患者符合纳入标准（30例男性，2例女性，中位年龄77.5岁（四分位间距（IQR）74 - 81））。组织病理学显示14例为AFX，18例为PDS。31例实现了切缘阴性切除，27例患者在报告期内无复发。局部复发率为5%，4例检测到远处转移。在所有PDS病例中，9例出现卫星转移。没有AFX出现卫星转移。由于这些肿瘤罕见，在专门的肉瘤中心进行跨学科治疗很有必要。
