Heath Michelle, Jaimes Natalia, Lemos Bianca, Mostaghimi Arash, Wang Linda C, Peñas Pablo F, Nghiem Paul
University of Washington, Division of Dermatology, Seattle, Washington 98109, USA.
J Am Acad Dermatol. 2008 Mar;58(3):375-81. doi: 10.1016/j.jaad.2007.11.020.
Merkel cell carcinoma (MCC) is an aggressive skin cancer with a mortality of 33%. Advanced disease at diagnosis is a poor prognostic factor, suggesting that earlier detection may improve outcome. No systematic analysis has been published to define the clinical features that are characteristic of MCC.
We sought to define the clinical characteristics present at diagnosis to identify features that may aid clinicians in recognizing MCC.
We conducted a cohort study of 195 patients given the diagnosis of MCC between 1980 and 2007. Data were collected prospectively in the majority of cases, and medical records were reviewed.
An important finding was that 88% of MCCs were asymptomatic (nontender) despite rapid growth in the prior 3 months (63% of lesions) and being red or pink (56%). A majority of MCC lesions (56%) were presumed at biopsy to be benign, with a cyst/acneiform lesion being the single most common diagnosis (32%) given. The median delay from lesion appearance to biopsy was 3 months (range 1-54 months), and median tumor diameter was 1.8 cm. Similar to earlier studies, 81% of primary MCCs occurred on ultraviolet-exposed sites, and our cohort was elderly (90% >50 years), predominantly white (98%), and often profoundly immune suppressed (7.8%). An additional novel finding was that chronic lymphocytic leukemia was more than 30-fold overrepresented among patients with MCC.
The study was limited to patients seen at a tertiary care center. Complete clinical data could not be obtained on all patients. This study could not assess the specificity of the clinical characteristics of MCC.
To our knowledge, this study is the first to define clinical features that may serve as clues in the diagnosis of MCC. The most significant features can be summarized in an acronym: AEIOU (asymptomatic/lack of tenderness, expanding rapidly, immune suppression, older than 50 years, and ultraviolet-exposed site on a person with fair skin). In our series, 89% of primary MCCs had 3 or more of these findings. Although MCC is uncommon, when present in combination, these features may indicate a concerning process that would warrant biopsy. In particular, a lesion that is red and expanding rapidly yet asymptomatic should be of concern.
默克尔细胞癌（MCC）是一种侵袭性皮肤癌，死亡率为33%。诊断时的晚期疾病是一个不良预后因素，这表明早期检测可能改善预后。尚未发表系统分析来定义MCC的特征性临床特征。
我们试图确定诊断时存在的临床特征，以识别可能有助于临床医生识别MCC的特征。
我们对1980年至2007年间被诊断为MCC的195例患者进行了队列研究。大多数病例的数据是前瞻性收集的，并对病历进行了回顾。
一个重要发现是，尽管在之前3个月内快速生长（63%的病变）且呈红色或粉红色（56%），但88%的MCC是无症状（无压痛）的。大多数MCC病变（56%）在活检时被推测为良性，最常见的单一诊断是囊肿/痤疮样病变（32%）。从病变出现到活检的中位延迟时间为3个月（范围1 - 54个月），中位肿瘤直径为1.8厘米。与早期研究相似，81%的原发性MCC发生在紫外线暴露部位，我们的队列患者年龄较大（90% >50岁），主要为白人（98%），且常存在严重免疫抑制（7.8%）。另一个新发现是，慢性淋巴细胞白血病在MCC患者中的比例高出30多倍。
该研究仅限于在三级医疗中心就诊的患者。并非所有患者都能获得完整的临床数据。本研究无法评估MCC临床特征的特异性。
据我们所知，本研究首次定义了可能作为MCC诊断线索的临床特征。最重要的特征可以用首字母缩略词AEIOU概括：无症状/无压痛、快速扩大、免疫抑制、年龄大于50岁以及白皙皮肤者的紫外线暴露部位。在我们的系列研究中，89%的原发性MCC具有这些发现中的3项或更多。尽管MCC不常见，但当这些特征同时出现时，可能表明存在需要活检的可疑病变。特别是，一个红色且快速扩大但无症状的病变应引起关注。
