Pratihar Sarbartha Kumar, Khanna Ashish, Chakraborty Arnab, Vasudeo Vivek, Saurabh Nikhil, Kumar Bhuwan, Ali Mujahid, Singh Amitabh, Rawal Sudhir Kumar
Department of Urooncology and Robotic surgery, Rajiv Gandhi Cancer Institute and Research Centre, Sector 5 Rohini, New Delhi, 110085 India.
Department of Surgical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Sector 5 Rohini, New Delhi, India.
Indian J Surg Oncol. 2023 Sep;14(3):556-560. doi: 10.1007/s13193-023-01766-0. Epub 2023 May 17.
Cutaneous radiation-associated angiosarcoma (cRAA) is a rare and aggressive secondary cutaneous angiosarcoma (cAS) with poor survival. cRAA has been mostly reported in breast carcinoma patients. Owing to its rarity, there is scanty literature available and no treatment guidelines. To the best of our knowledge, this is the first report of cRAA after multimodality treatment of carcinoma penis. A sixty-eight-year-old gentleman, a known case of carcinoma penis, underwent total penectomy with perineal urethrostomy and bilateral radical inguinopelvic lymph node dissection 6 years ago. He received adjuvant radiotherapy to the pelvis and bilateral groin. He presented with a bleeding plaque-like lesion with ulceration over the left lower abdomen (within previous radiation field) which rapidly progressed in size over the past 2 months. On examination, the lesion bled profusely on touch. Contrast MRI was suggestive of lobulated exophytic enhancing cutaneous lesion free from underlying muscle. Wedge biopsy was suggestive of cutaneous angiosarcoma. He underwent wide local excision with local perforator flap reconstruction from the right lower abdomen. Histopathology was suggestive of cutaneous angiosarcoma which showed immunoexpression of CD31, ERG1, cMYC suggestive of cRAA. cRAA is a very aggressive disease with 5-year survival of 15-34%. To the best of our knowledge, this is the first ever reported case of cRAA of lower abdomen after multimodality management of carcinoma penis. It masquerades with other benign and less aggressive radiation-induced skin lesions. cMYC immunoexpression is specific for secondary cAS. Wide local resection with negative margin provides the best outcome.
皮肤辐射相关血管肉瘤（cRAA）是一种罕见且侵袭性强的继发性皮肤血管肉瘤（cAS），生存率低。cRAA主要在乳腺癌患者中报道。由于其罕见性，相关文献稀少且没有治疗指南。据我们所知，这是阴茎癌多模式治疗后发生cRAA的首例报告。一名68岁男性，已知患有阴茎癌，6年前接受了全阴茎切除术、会阴尿道造口术和双侧根治性腹股沟盆腔淋巴结清扫术。他接受了盆腔和双侧腹股沟的辅助放疗。他出现了一个左下腹部（在先前放疗区域内）有溃疡的出血性斑块样病变，在过去2个月内迅速增大。检查时，病变触诊时大量出血。对比增强磁共振成像提示为分叶状外生性增强的皮肤病变，不累及深层肌肉。楔形活检提示为皮肤血管肉瘤。他接受了广泛局部切除，并采用右下腹部局部穿支皮瓣重建。组织病理学提示为皮肤血管肉瘤，显示CD31、ERG1、cMYC免疫表达，提示为cRAA。cRAA是一种非常侵袭性的疾病，5年生存率为15% - 34%。据我们所知，这是阴茎癌多模式治疗后下腹部cRAA的首例报告病例。它与其他良性且侵袭性较小的放射性皮肤病变相似。cMYC免疫表达对继发性cAS具有特异性。切缘阴性的广泛局部切除可提供最佳治疗效果。
