Seo Natsuki, Nuri Takashi, Asaka Akinori, Kuwabara Hiroko, Hirose Yoshinobu, Ueda Koichi
From the Department of Plastic and Reconstructive Surgery, Osaka Medical and Pharmaceutical University, Takatsuki, Osaka, Japan.
Department of Pathology, Osaka Medical and Pharmaceutical University, Takatsuki, Osaka, Japan.
Plast Reconstr Surg Glob Open. 2025 Jul 17;13(7):e6965. doi: 10.1097/GOX.0000000000006965. eCollection 2025 Jul.
A malignant peripheral nerve sheath tumor is a rare and aggressive soft-tissue sarcoma that originates from Schwann cells or neural crest cells. Although 60% of cases are associated with neurofibromatosis type 1, approximately 10% occur secondary to radiotherapy. We report a malignant peripheral nerve sheath tumor that developed after radiotherapy for a keloid. A 35-year-old woman presented with a 7 × 9 cm left shoulder lesion 11 years after surgical treatment of a keloid followed by radiotherapy (20 Gy). Four years earlier, she had undergone a second operation with postoperative radiation (25 Gy) to address keloid recurrence. Biopsy was concerning for malignancy, the tumor was resected with a 2-cm margin, and reconstruction was performed using a latissimus dorsi flap. The lesion exhibited erosion and hemorrhage not seen in benign keloids, and histological examination of the surgical specimen revealed high-grade sarcoma. No recurrence has been observed during follow-up. The development of secondary malignancy after radiotherapy for keloids is exceedingly rare-only 6 cases have been reported, including ours. However, clinicians must be vigilant in all keloid patients undergoing radiotherapy. Rapid enlargement, erosion, or bleeding in previously treated keloids should raise suspicion for malignant transformation. Proper radiation protocols and protective measures are essential to mitigate the risk. Early recognition and surgical intervention are critical for achieving favorable outcomes.
恶性外周神经鞘瘤是一种罕见且侵袭性的软组织肉瘤,起源于施万细胞或神经嵴细胞。虽然60%的病例与1型神经纤维瘤病相关,但约10%继发于放射治疗。我们报告一例在瘢痕疙瘩放疗后发生的恶性外周神经鞘瘤。一名35岁女性在瘢痕疙瘩手术治疗并放疗(20 Gy)11年后,出现一个7×9 cm的左肩肿物。4年前,她因瘢痕疙瘩复发接受了二次手术及术后放疗(25 Gy)。活检提示恶性,肿瘤行2 cm切缘切除,并用背阔肌皮瓣进行重建。该肿物表现出良性瘢痕疙瘩未见的糜烂和出血,手术标本的组织学检查显示为高级别肉瘤。随访期间未观察到复发。瘢痕疙瘩放疗后继发恶性肿瘤极为罕见——包括我们的病例在内仅报告了6例。然而,临床医生对所有接受放疗的瘢痕疙瘩患者都必须保持警惕。既往治疗的瘢痕疙瘩迅速增大、糜烂或出血应怀疑发生恶变。适当的放疗方案和保护措施对于降低风险至关重要。早期识别和手术干预对于取得良好预后至关重要。