原发性肛门直肠黑色素瘤:多模式管理在一系列的四个病例和文献回顾

Nishant Lohia, Manoj Prashar, S. Harish, Sankalp Singh, Anand Subramananiam, S. Viswanath, R. Ranjan, G. Trivedi
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引用次数: 0

摘要

肛肠黑色素瘤是一种非常罕见的侵袭性粘膜黑色素细胞恶性肿瘤,占所有肛肠肿瘤的1%。只有少数病例被报道。手术切除仍是主要的治疗方法。由于缺乏随机试验,尚无明确的管理策略。我们在此报告4例肛门直肠黑色素瘤的病例系列。4例均行腹会阴切除术(APR)及淋巴结清扫术。4例患者中2例接受替莫唑胺(TMZ)辅助化疗,1例接受TMZ联合顺铂辅助双重化疗,4例患者在接受辅助化疗前因非肿瘤性疾病死亡。在第一例和第三例中,淋巴结清扫仅限于盆腔淋巴结。然而,在第二个病例中,进行了广泛的淋巴结清扫,以双侧骨盆和腹股沟淋巴结清扫和主动脉旁淋巴结清扫的形式进行。第四个病例也接受了广泛的淋巴结清扫,以双侧骨盆和主动脉旁淋巴结清扫的形式。然而,第一位患者在辅助TMZ的第一个周期结束时发生转移,并在手术3个月后死亡。第二例患者可以完成所有6个周期的辅助TMZ治疗,并在辅助TMZ治疗完成1年后出现骨骼和脑转移。他还接受了脑转移的姑息性全脑放疗。手术后18个月,他终于死于疾病。第三例完成了所有6个辅助TMZ周期,并且仍然存活,无病间隔约为1年。第4例术后1个月左右死于非肿瘤性死亡。
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Primary anorectal melanoma: Multimodality management in a series of four cases and review of literature
Anorectal melanoma is a very rare and aggressive mucosal melanocytic malignancy, accounting for 1% of all anorectal cancers. There have only been a few cases reported. Surgical resection remains the mainstay of treatment. No definitive management strategies exist because of the absence of randomized trials. We here report a case series on four cases of anorectal melanoma. All four cases underwent abdominoperineal resection (APR) and nodal dissection. Two out of four cases received adjuvant temozolomide (TMZ), one case received adjuvant doublet chemotherapy with TMZ and cisplatin, and the fourth case succumbed to nononcological disease before he could be subjected to adjuvant chemotherapy. In the first and third case, nodal dissection was limited to pelvic nodes only. However, in the second case, extensive nodal dissection in the form of bilateral pelvic and inguinal lymph nodal dissection and para-aortic lymph nodal dissection was performed. The fourth case also was subjected to extensive nodal dissection in form of bilateral pelvic and para-aortic lymph node dissection. The first patient however became metastatic at the end of first cycle of adjuvant TMZ and succumbed to his illness post 3 months of surgery. The second patient could complete all six cycles of adjuvant TMZ, and after 1 year of completion of adjuvant TMZ, he presented with skeletal and brain metastases. He also received palliative whole-brain radiotherapy for brain metastases. He finally succumbed to his disease 18 months after surgery. The third case completed all six cycles of adjuvant TMZ and is still alive with disease-free interval of around 1 year. The fourth case succumbed to nononcological death around 1 month after surgery.
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