Lombardo Alyssa M, Sheetz Tyler, Carrau Ricardo L, Zynger Debra L, Singer Eric A
Division of Urologic Oncology, The Ohio State University Comprehensive Cancer Center, Columbus, OH.
Department of Otolaryngology, The Ohio State University Comprehensive Cancer Center, Columbus, OH.
J Kidney Cancer VHL. 2024 Aug 30;11(3):45-50. doi: 10.15586/jkcvhl.v11i3.306. eCollection 2024.
Adenoid cystic carcinoma (ACC) is a rare tumor, accounting for 1% of all head and neck cancers, with an aggressive nature characterized by local recurrence, delayed metastasis, and survival of less than 50% at 10 years. This is a case of biopsy-proven ACC to the kidney, 1 of 29 known occurrences, managed by metastasectomy by robotic-assisted nephrectomy, with plans for resection of lung metastasis. Thirteen years after diagnosis of sinonasal ACC treated with resection, the patient presented with shortness of breath. This prompted a CT scan of the chest, which led to the incidental finding of left renal mass and pulmonary lesion. Literature suggests improved disease-specific survival in locoregional recurrence treated with surgery versus radiation; in patients with metastasis to the lung, metastasectomy offers greater survival benefit than supportive therapy. But, this is not significantly better than chemotherapy or radiation alone. While the optimal therapeutic approach remains to be identified in distant metastatic ACC, metastasectomy remains a viable option for patients who have potentially completely resectable metastatic tumors, appropriate performance status, and adequate affected-organ function. Preoperative counseling should include discussion on partial nephrectomy with prioritization of nephron-sparing but potential for increased perioperative risk versus radical nephrectomy to ensure negative margins and expedite timeline to systemic therapy.
腺样囊性癌(ACC)是一种罕见肿瘤,占所有头颈癌的1%,具有侵袭性,其特征为局部复发、延迟转移,10年生存率低于50%。这是一例经活检证实的肾ACC病例,为已知的29例病例之一,通过机器人辅助肾切除术行转移灶切除术治疗,并计划切除肺转移灶。鼻窦ACC切除术后13年,患者出现呼吸急促。这促使进行胸部CT扫描,结果意外发现左肾肿块和肺部病变。文献表明,与放疗相比,手术治疗局部区域复发可提高疾病特异性生存率;对于发生肺转移的患者,转移灶切除术比支持性治疗具有更大的生存获益。但是,这并不比单纯化疗或放疗明显更好。虽然远处转移性ACC的最佳治疗方法仍有待确定,但对于具有潜在可完全切除的转移性肿瘤、合适的身体状况和足够的受累器官功能的患者,转移灶切除术仍然是一种可行的选择。术前咨询应包括讨论部分肾切除术,优先保留肾单位,但围手术期风险可能增加,与根治性肾切除术相比,以确保切缘阴性并加快全身治疗的时间线。