Merkel细胞癌合并原位鳞状细胞癌一例并文献复习

Merkel细胞癌是一种罕见的、具有高度侵袭性的皮肤神经内分泌癌,好发于老年人的日光暴露部位,尤其是头颈部(41%～50%),其次是四肢(32%～38%)。Merkel细胞癌可与鳞状细胞癌、鲍温病、基底细胞癌等皮肤肿瘤合并发生。我们报道一例发生在非光暴露部位的Merkel细胞癌合并原位鳞状细胞癌,并对相关文献进行复习。     

Cutaneous undifferentiated small (Merkel) cell carcinoma,that developed synchronously with multiple actinic keratoses,squamous cell carcinomas and basal cell carcinoma

Merkel Cell Carcinoma (MCC) is an uncommon undifferentiated neuroendocrine tumor, arising in skin mainly on sun-exposed areas. We present an unusual case of primary cutaneous undifferentiated small cell carcinoma that co-existed with six other lesions; 2 actinic keratoses, 3 squamous-cell carcinomas and a basal-cell carcinoma. HE stained sections revealed MCC located in the mid-dermis, co-existing with severe actinic keratosis. Immunohistochemically, the tumor cells reacted to cytokeratin 20, epithelial membrane antigen, chromogranin and neuron specific enolase. This is an unusual case of cutaneous MCC co-existing with six other different lesions. The concurrent development of MCC, squamous-cell and basal-cell carcinoma in the same patient indicates the pluripotent epidermal stem cell origin of these tumors. Further research is needed to enlighten the factors inducing this divergent differentiation.     

皮肤基底细胞癌和皮肤鳞状细胞癌270例回顾性分析

 目的:总结我院皮肤基底细胞癌(BCC)和皮肤鳞状细胞癌(SCC)临床与组织病理资料,以期提高BCC与SCC的诊断率。方法：回顾性分析2014年1月1日-2018年12月31日间我院皮肤科门诊经组织病理切片确诊的170例BCC与100例SCC患者的临床与病理资料。结果：BCC与SCC年度发病整体均呈逐渐上升趋势。BCC、SCC男女患病比例分别为0.8∶1、1∶1，好发部位均为曝光部位(头面颈部和四肢)，临床诊断与组织病理诊断符合率分别为62.4%与30.0%。临床诊断上，BCC易与脂溢性角化病(SK)、色素痣混淆；SCC易与BCC、鲍温病、光线性角化病(AK)混淆。结论：BCC和SCC为临床常见的非黑素性皮肤肿瘤，但易误诊和漏诊。临床医生对于可疑病灶应尽早行皮损组织病理检查。     

Surgical margins of excision for basal cell carcinoma and squamous cell carcinoma

In excising basal and squamous cell carcinomata, the surgical margin that is wide enough to completely remove the tumor an acceptable percentage of the time and narrow enough to minimize removal of excessive normal tissue must be selected. This task can be reliably accomplished with comprehensive knowledge of factors that affect subclinical tumor extension such as tumor appearance, diameter, histology, location, treatment status, and, in the case of squamous cell carcinoma, vertical invasion depth and involvement of subcutaneous fat. Information regarding these factors along with specific recommendations about excisional margins for basal cell and squamous cell carcinomata is presented.     

Merkel细胞癌多瘤病毒阳性的皮肤原发性Merkel细胞癌二例

【摘要】 目的 报道Merkel细胞癌多瘤病毒阳性的Merkel细胞癌2例。 方法 对诊治的2例Merkel细胞癌进行光镜观察及免疫组化标记,聚合酶链反应（PCR）检测Merkel细胞癌多瘤病毒并测序。 结果 2例均为男性,例1右下肢胫前肿物1年余,皮肤科检查见右胫前密集粉红色结节,融合成10 cm × 8 cm肿块,质硬,部分表面糜烂伴渗出及结痂,肿块周围亦可见多个大小不一的红色结节,活动性差。例2左膝肿物6月余,皮肤科检查见左膝内侧5 cm × 4 cm紫蓝色结节型肿物,质硬,边界不清,活动性差。2例患者皮损组织病理表现相似,肿瘤细胞大小一致,细胞核大、深染,染色质细腻,核分裂象易见;胞质少,红染。免疫组化：广谱细胞角蛋白（pan-CK）、细胞角蛋白20（CK20）、突触素（Syn）、嗜铬素（CgA）和神经元特异性烯醇化酶（NSE）均阳性,Ki-67（≥60%）阳性;细胞角蛋白7（CK7）、S100蛋白、HMB45、CD34、甲状腺转录因子1（TTF-1）和白细胞共同抗原（LCA）表达均阴性。2例Merkel细胞癌均经PCR检测到Merkel细胞癌多瘤病毒,而5例皮肤T细胞淋巴瘤、2例正常人皮肤和2例T细胞淋巴瘤细胞系MAC1和MAC2A均未检测到Merkel细胞癌多瘤病毒。 结论 Merkel细胞癌具有特征性的临床和组织病理表现,免疫组化标记、PCR检测Merkel细胞癌多瘤病毒对明确诊断具有重要作用。 【关键词】 癌,Merkel细胞; 多瘤病毒属     

痣样基底细胞癌综合征一例

痣样基底细胞癌综合征是一种罕见的常染色体显性遗传病,以泛发性皮肤基底细胞癌和多器官发育异常为主要临床特征。本文报告1例痣样基底细胞癌综合征患者,并结合相关文献对该病的发病率、发病机制、诊断标准、治疗方法等进行讨论。     

Insights into the Merkel cell phenotype from Merkel cell carcinoma cell lines

Merkel cell carcinoma (MCC) of the skin is an aggressive form of skin cancer and is being seen with increasing incidence in Queensland. We have recently established a number of MCC cell lines and characterized these for growth, morphology, expression of neuroendocrine markers and radiation sensitivity. As a result, cell lines were grouped into four classes by their morphology in a similar way to small cell lung cancer (SCLC) cell lines. Types I and II cell lines grew slowly as tight spherical clusters suspended in the medium, with Type II cell lines less densely packed than the Type I cell lines. Type III cell lines grew as flat 2-dimensional clusters and had shorter doubling times and Type IV cell lines grew as adherent monolayers and had the shortest doubling times. Expression of neuroendocrine markers distinguished those with a classic phenotype from those with a variant one. Mainly morphological Types I and II retained the classic phenotype while Classes III and IV had a variant phenotype. The range of surviving fraction at 2 Gray (SF2 0.2-0.45) seen in MCC cell lines was not as high as seen in SCLC cell lines but the variant ones tended to be more radiation resistant. Examination of POU proteins showed that neuroendocrine phenotype was linked with expression of brn-2, and growth in suspension with brn-3c.     

Merkel cell carcinoma in situ: New insights into the cells of origin

We report a case of a 71-year-old male who presented with a small skin-coloured plaque on his cheek. Histopathology demonstrated an intraepidermal carcinoma with follicular involvement. No evidence of dermal invasion was seen. Immunohistochemical studies showed areas of positive staining for CK20, EMA and synaptophysin. Histopathology findings were found to be most consistent with a diagnosis of intraepidermal carcinoma with features of Merkel cell carcinoma in situ, in combination with a squamous cell carcinoma in situ, with follicular involvement. Recent advances and findings suggest Merkel cell polyomavirus MCPyV-positive Merkel cell carcinoma and MCPyV-negative Merkel cell carcinoma have different cells of origin from different germ layers.     

SGK1在日光性角化病、基底细胞癌及鳞状细胞癌中的表达

目的：检测SGK1在日光性角化病（AK）、基底细胞癌（BCC）及鳞状细胞癌（SCC）中的表达。方法：采用免疫组化SABC法检测SGK1在25例正常皮肤(NS)、25例AK、28例BCC、28例皮肤鳞状细胞癌标本中的表达。结果：NS、AK、BCC和SCC标本中,SGK1阳性细胞率分别为（40.03±14.42）%,（36.63±14.28）%,（52.82±18.73）%和（52.58±20.13）%。BCC组和SCC组分别与NS组比较,差异均有统计学意义（Ps<0.05）。各组SGK1染色阳性细胞率>50%的标本分别为6例（24%）,3例（12%）,16例（57.14%）和14例（50%）,BCC组和SCC组分别与NS组比较,差异均有统计学意义（Ps<0.05）。结论：SGK1的高表达可能与基底细胞癌及鳞状细胞癌的发病有关。     

色素性基底细胞癌一例

患者女,67岁.主因左耳后皮疹20年,溃烂2年余就诊.患者自述20年前左耳后长出一绿豆大小皮肤色丘疹,无自觉症状,数年后丘疹略增大,表面变粗糙,当地医院诊断刺瘊,自行数次用头发捆勒祛除之,未见效,皮疹增长缓慢无不适感.近5年常令其子用烟头烫,2年来丘疹反复糜烂、渗液、结痂,基底渐增大,色变黑,皮疹增大至鸽卵大小,中心溃烂不愈,无痛、痒感.     

梭形细胞鳞状细胞癌一例

患者女,62岁.下唇斑块伴轻度瘙痒2年就诊.2年前无明显诱因下唇中部出现一硬化、增厚的黄豆大小斑疹,轻度瘙痒,少许片状脱屑,无糜烂、渗液及结痂.     

Basal cell carcinoma of the vulva

Basal cell carcinoma (BCC), the most common human malignancy, accounting for 75% of all non-melanoma skin cancer, is uncommon on unexposed skin such as the perianal and genital regions. We describe a woman with BCC of the vulva treated with local resection. All margins of excision were free of disease. The patient recovered without complication and there have been no recurrences after 2 years of follow-up. Approximately 200 cases of BCC on perianal and genital skin have been reported in the literature. Although the aetiology of vulvar BCC is not known, early diagnosis is important. Because BCC in these sites sometimes seems innocuous, biopsy of all suspect lesions is advisable.     

角化棘皮瘤与鳞状细胞癌增殖特性的比较研究

用PC10免疫组化染色观察了增殖细胞核抗原（PCNA）在角化棘皮瘤（KA）和鳞癌（SCC）中的表达。在KA中，PCNA阳性细胞主要分布于基底层和基底上层，而在SCC中，则广泛分布于肿瘤细胞团中。PCNA阳性指数在KA组中为22．3％～40．8％，平均32．6％，而在SCC组中则高达41．0％～80．9％，平均63．2％。两组间有显著性差异（P＜0．01），而且两组间个例PCNA阳性指数没有重叠。这种增殖特性的显著差异提示KA和SCC是两种性质不同的肿瘤。而PCNA阳性指数可能对鉴别KA和SCC有帮助。     

Usefulness of ulceration and hyperkeratosis as clinical predictors of Merkel cell polyomavirus‐negative and combined Merkel cell carcinoma: A retrospective study

Merkel cell carcinoma is a rare neuroendocrine carcinoma of the skin that is associated with Merkel cell polyomavirus (MCPyV). The clinical appearance and demographic characteristics of this tumor have been described using the mnemonic AEIOU: asymptomatic, expanding rapidly, immune suppression, older than 50 years, and ultraviolet‐exposed fair skin. In addition, MCC can be categorized based on morphology as pure MCC or combined MCC that exhibits neuroendocrine and other phenotypic elements. There is limited information regarding the clinical characteristics and prognosis of combined MCC. This retrospective study aimed to identify factors, such as ulceration or hyperkeratosis, that could predict MCPyV status and morphological variants. Twenty patients with MCC were divided into groups based on MCPyV status and morphology: MCPyV‐positive or MCPyV‐negative MCC and pure or combined MCC. The patients’ MCPyV status was immunohistochemically determined using the CM2B4 antibody to the MCPyV large T‐antigen. The patients’ clinicopathological characteristics were evaluated to identify predictors of MCPyV‐negative MCC and combined MCC. The presence of ulceration/hyperkeratosis predicted the presence of MCPyV‐negative MCC (80% of cases) and combined MCC (50% of cases). None of the 10 patients with MCPyV‐positive MCC had ulceration/hyperkeratosis. The clinical presence of ulceration/hyperkeratosis may help guide the diagnosis of MCPyV‐negative MCC and combined MCC.     

色素性基底细胞癌

报告1例色素性基底细胞癌。患者女,68岁。右侧腋下黑色条状斑块10余年。皮肤科检查:右侧腋下约3.0 cm×0.2 cm大黑色斑块,边界尚清,其上散在分布数个米粒大黑色丘疹,斑块中部可见糜烂、渗液,渗液周边可见炎症性红斑,无触痛。皮损组织病理检查:表皮层局灶瘤细胞巢,表皮至真皮层可见一肿块,由嗜碱性基底样细胞组成,可见细胞异形性及有丝分裂象,在肿块周边细胞呈栅栏状排列,可见收缩间隙。诊断:色素性基底细胞癌。     

Cutaneous metastatic breast carcinoma with clear cell features

Breast carcinoma remains one of the most common sources of skin metastases in women. Cutaneous breast carcinoma metastases have variable clinical and histopathologic presentations that can make diagnosis challenging. We report a unique case of metastatic breast carcinoma with prominent clear cell features, thus mimicking a xanthomatous process. Dermatopathologists should be aware of this entity given its resemblance to other clear cell infiltrates and neoplasms.