Merkel cell carcinoma: Report of 10 cases and review of the literature
Merkel cell carcinoma: Report of 10 cases and review of the literature
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DOI：
10.1067/mjd.2000.106505
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发表时间：
2000-11-01
影响因子：
13.8
通讯作者：
Wright, J
中科院分区：
文献类型：
--
作者：
Akhtar, S;Oza, KK;Wright, J
背景资料：默克尔细胞癌是一种罕见的原发性神经内分泌皮肤肿瘤，通常发生在老年患者的头颈部或四肢。由于回顾性数据的局限性，最佳的治疗是不好defined.Objective：我们的目的是提出的临床过程和治疗的10例MCC和审查发表的文献上MCC.Method：我们进行了回顾性分析，并获得了详细的临床资料，所有10例患者治疗MCC在我们的机构从1986年至1998年。医学文献也审查了自然史和治疗建议，使用MEDLINE search.Results：5名男性和5名女性接受了治疗，1986年和1998年之间的MCC（5 IA期疾病，4 IB期，1 II期）。平均年龄为70.3岁（范围：47-86岁）。肿瘤位于头颈部7例，四肢3例。10例患者中有5例复发（复发前平均时间，5.7个月）（范围，2周至20个月）; 1例患者局部复发，1例患者区域淋巴结复发，3例患者局部和区域淋巴结复发。4例患者发生全身转移。还观察到长期存活（6至> 164个月）; 4名患者死于MCC。初次手术后，9例患者在某个时候接受了放疗，3例患者还接受了化疗。10例患者中有5例既往接受过治疗或并存恶性肿瘤。在一名患者中，MCC发生在先前照射的区域。回顾875例病例，显示男女比例为1.5：1;肿瘤的位置如下：头颈部，47%;四肢，40%;躯干，8%;未知原发部位，5%。局部复发率为25%，区域淋巴结转移率为52%，远处转移率为34%，死亡率为34%。早期局部处理较小的病灶可获得良好的长期生存率。目前尚不清楚预防性淋巴结清扫和/或放射和辅助放射是否会增加生存率。治疗局部复发后可获得长期生存。化疗的作用仍有争议，应考虑在晚期疾病患者和那些不被认为是手术的候选人。
Background: Merkel cell carcinoma (MCC) is a rare primary neuroendocrine skin tumor that usually arises in the head and neck or the extremities of elderly patients. Because of the limitation of retrospective data, optimal treatment is not well defined.Objective: Our purpose was to present the clinical course and treatment of 10 patients with MCC and review the published literature on MCC.Method: We conducted a retrospective analysis and obtained detailed clinical information for all 10 patients treated for MCC at our institution from 1986 through 1998. The medical literature was also reviewed for natural history and treatment recommendations using MEDLINE search.Results: Five men and 5 women received their treatment between 1986 and 1998 for MCC (5 had stage IA disease, 4 stage IB, 1 stage II). The mean age was 70.3 years (range, 47-86 years). Seven tumors were located on the head and neck and 3 on extremities. Five of 10 patients had a relapse (mean time before recurrence, 5.7 months) (range, 2 weeks-20 months); one patient had local recurrence, one had regional lymph node recurrence, and 3 had both local and regional lymph node recurrence. In 4 patients systemic metastases developed. Long survival is also noted (6 to > 164 months); 4 patients died of MCC. After initial surgery, 9 patients received radiotherapy at some point and 3 patients also received chemotherapy Five of 10 patients had 13 previously treated or coexisting malignant neoplasms. In one patient MCC developed in a previously irradiated field. Review of 875 cases showed a male/female ratio of 1.5:1; location of tumors was as follows: head and neck, 47%; extremities, 40%; trunk, 8%; unknown primary site, 5%. Local recurrence was observed in 25%, regional lymph node involvement in 52%, distant metastasis in 34%, and MCC was a cause of death in 34%.Conclusion: MCC has a high incidence of locoregional recurrence with distant metastases that is more common with higher stage lesions. Early local management of smaller lesions results in good long-term survival. It is not known whether prophylactic lymph node dissection and/or radiation and adjuvant radiation increases survival. Long survival can be achieved after treating locoregional recurrence. The role of chemotherapy is still controversial and should be considered in patients with advanced disease and those not thought to be candidates for surgery.