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Histopathologic and Immunophenotypic Properties of Primary Cutaneous Large Cell Neuroendocrine Carcinomas 原发性皮肤大细胞神经内分泌癌的组织病理学和免疫表型特征。
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-31 DOI: 10.1111/cup.14853
Myles R. McCrary, Riechelle Garcia, Janice Jiang, Michael B. Morgan

Cutaneous large cell neuroendocrine carcinoma (LCNEC) is a rare and poorly understood malignancy. Here we describe the clinicopathological characteristics of six cutaneous LCNEC case. A retrospective chart and slide review of PCLCNEC cases at a large commercial dermatopathology practice from January 2017 to May 2025 was performed. Patient characteristics, histopathologic features, and immunohistochemical profiles, including Merkel cell polyoma virus large T antigen/MCPyV antigenicity, were summarized. Demographically, PCLCNEC occurred in elderly white patients, presenting as a rapidly progressing nodule in sun-exposed skin. Histologically, the tumors were characterized by predominantly infiltrative growth patterns with varying levels of neuroendocrine architectural and cytologic features, including rosette and trabeculae formation, organoid nesting, and peripheral palisading. All were positive for CK7 and neuroendocrine markers and negative for MCPyV, CK20, and TTF1 expression by immunohistochemistry. Metastasis was identified in one patient, while the remaining five patients had no evidence of disease following wide local excision with a median follow-up period of 25 months. This series contributes valuable insights into the characterization and diagnosis of primary cutaneous LCNEC. In conjunction with previously reported data, we suggest that the morphology and immunophenotype support further inquiry into its potential distinction as a unique cutaneous entity.

摘要皮肤大细胞神经内分泌癌(LCNEC)是一种罕见且了解甚少的恶性肿瘤。本文报告6例皮肤LCNEC病例的临床病理特点。对2017年1月至2025年5月一家大型商业皮肤病理学诊所的PCLCNEC病例进行回顾性图表和幻灯片回顾。总结了患者特征、组织病理学特征和免疫组织化学特征,包括默克尔细胞多瘤病毒大T抗原/MCPyV抗原性。人口统计学上,PCLCNEC发生在老年白人患者中,表现为暴露在阳光下的皮肤上快速发展的结节。组织学上,肿瘤主要表现为浸润性生长模式,具有不同程度的神经内分泌建筑和细胞学特征,包括玫瑰花结和小梁形成、类器官巢和周围栅栏。所有患者的CK7和神经内分泌标志物均呈阳性,MCPyV、CK20和TTF1免疫组化表达均呈阴性。1例患者发现转移,而其余5例患者在广泛局部切除后无疾病证据,中位随访期为25个月。这一系列为原发性皮肤LCNEC的特征和诊断提供了有价值的见解。结合先前报道的数据,我们建议形态学和免疫表型支持进一步调查其作为独特皮肤实体的潜在区别。
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引用次数: 0
CRTC1::MAML2-Positive Hidradenoma With Sebaceous Differentiation CRTC1:: maml2阳性皮脂腺瘤。
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-29 DOI: 10.1111/cup.14862
Megumi Aoki, Keisuke Goto, Kazuyasu Fujii, Mitsuharu Nomoto, Masamichi Goto, Manami Kajiwara, Toshitaka Nagao, Shigeto Matsushita

Hidradenoma can exhibit several cell types, including clear cells, polyhedral eosinophilic cells, squamoid cells, mucinous cells, oxyphilic (oncocytic) cells, and transitional (intermediate) cells. However, sebocytes have not yet been described in hidradenoma. Here, we present a case of CRTC1::MAML2-positive hidradenoma with sebaceous differentiation. In addition, this tumor had two cell-type layers that were morphologically similar to apocrine glandular cells and myoepithelial cells throughout the lesion. A 66-year-old male presented with a 20 mm nodule on his upper lip with a 3-year history. Histopathological examination revealed a multinodular tumor adherent to the overlying epidermis. No surrounding salivary glands were seen. The tumor consisted of eosinophilic ductoglandular cells surrounded by basaloid cells at the outer periphery. Numerous sebocytes were scattered throughout the tumor. Severe nuclear atypia or mitotic figures were not observed. Immunohistochemical examination showed no expression of α-smooth muscle actin, calponin, and S100 protein in the tumor cells. Adipophilin highlighted scattered sebocytic tumor cells. Immunoexpression of MLH1, PMS2, MSH2, and MSH6 was preserved. MAML2 break-apart fluorescence in situ hybridization revealed frequent split signals. Sanger sequencing revealed a CRTC1(e1)::MAML2(e2) fusion. Based on this report, hidradenoma should be included in the differential diagnosis of cutaneous adnexal tumors with sebaceous differentiation other than sebaceous adnexal tumors. In addition, sebocytes should be added to the list of tumor cell types in hidradenoma.

汗腺瘤可表现为几种细胞类型,包括透明细胞、多面体嗜酸性细胞、鳞状细胞、黏液细胞、嗜氧细胞和过渡性细胞。然而,在汗腺瘤中尚未发现脂细胞。在此,我们报告一例CRTC1:: maml2阳性皮脂腺瘤伴皮脂腺分化。此外,该肿瘤具有两种细胞型层,其形态与整个病变的大汗腺细胞和肌上皮细胞相似。66岁男性,上唇有20毫米结节,病史3年。组织病理学检查显示多结节性肿瘤粘附于上覆表皮。周围未见唾液腺。肿瘤由嗜酸性管腺细胞组成,外周由基底细胞包围。大量皮脂细胞散布在肿瘤各处。未见严重的核异型或有丝分裂象。免疫组化检查未见肿瘤细胞中α-平滑肌肌动蛋白、钙钙蛋白、S100蛋白的表达。脂亲蛋白突出了分散的皮脂细胞肿瘤细胞。保留MLH1、PMS2、MSH2和MSH6的免疫表达。MAML2断裂荧光原位杂交显示频繁的分裂信号。Sanger测序显示CRTC1(e1)::MAML2(e2)融合。根据本报告,除皮脂腺性附件肿瘤外,皮脂腺分化的皮肤附件肿瘤应纳入汗腺瘤的鉴别诊断。此外,应将皮脂细胞添加到汗腺瘤的肿瘤细胞类型列表中。
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引用次数: 0
Reaction to Cutaneous Filler Elleva Poly-l-Lactic Acid: Report of Two Cases 皮肤填充物Elleva聚l-乳酸反应2例报告。
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-27 DOI: 10.1111/cup.14857
Pedro Carneiro Marinho, Maria Belen Mendoza Macias, Joaquim Mesquita Filho, Luciana França, Maria Auxiliadora Jeunon Sousa, Thiago Jeunon de Sousa Vargas

The global demand for aesthetic procedures has been steadily increasing, driving the development of new techniques and materials to meet this need. However, these procedures are not without risks, including the formation of subcutaneous nodules following injectable treatments. Histopathology plays a crucial role in identifying skin fillers based on their distinct morphological characteristics. In this article, we present two cases of subcutaneous nodules associated with the application of Elleva poly-l-lactic acid, providing a detailed histopathological analysis.

全球对美容程序的需求一直在稳步增长,推动了新技术和新材料的发展,以满足这一需求。然而,这些手术并非没有风险,包括注射治疗后皮下结节的形成。组织病理学在识别皮肤填充物中起着至关重要的作用,基于其独特的形态特征。在这篇文章中,我们提出了两例皮下结节与Elleva聚l-乳酸的应用有关,提供了详细的组织病理学分析。
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引用次数: 0
PRAME Immunohistochemistry Fails to Highlight Persistent (Recurrent) Melanocytic Nevi PRAME免疫组化不能突出持续性(复发性)黑素细胞痣
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-25 DOI: 10.1111/cup.14859
Meaghan C. Dougher, Michael Ioffreda, Thomas N. Helm, Klaus F. Helm
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引用次数: 0
Plasmablastic Lymphoma With Intravascular Localization in a Kaposi Sarcoma Skin Lesion 卡波西肉瘤皮肤病变伴血管内定位的浆母细胞淋巴瘤
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-23 DOI: 10.1111/cup.14858
Mercedes Sanchez-Diaz, Daniel La Rosa, Bruner García, Karina Feria, Alex Ventura

We report a rare case of plasmablastic lymphoma (PBL) in an HIV-positive patient with nodal and gastric involvement, who also showed intravascular PBL cells within a longstanding Kaposi sarcoma (KS) skin lesion.

我们报告一例罕见的浆母细胞淋巴瘤(PBL),患者为hiv阳性,伴有淋巴结和胃受损伤,同时在长期的卡波西肉瘤(KS)皮肤病变中也发现了血管内PBL细胞。
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引用次数: 0
Diagnostic Utility of PRAME in Rare Melanoma Mimics: A Comparative Analysis Including GNET, MMNST, Epithelioid MPNST, and MITF-Rearranged Melanocytic Tumors PRAME在罕见黑色素瘤模拟物中的诊断应用:包括GNET、MMNST、上皮样MPNST和mitf重排黑色素细胞肿瘤的比较分析。
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-17 DOI: 10.1111/cup.14849
Ahmed Shah, Jeremiah F. Molligan, Carina A. Dehner, Jorge Torres-Mora

Background

Differentiating metastatic melanoma from histologic mimics such as malignant gastrointestinal neuroectodermal tumor (GNET), malignant melanotic nerve sheath tumor (MMNST), and epithelioid malignant peripheral nerve sheath tumor (EMPNST) poses significant diagnostic challenges due to overlapping morphology and immunophenotypes. PRAME is a novel immunohistochemical marker increasingly used to distinguish melanoma from its mimics, but remains underexplored in these rare tumor types.

Methods

PRAME immunohistochemistry was performed on four GNETs, seven MMNSTs, 10 EMPNSTs, 16 metastatic melanomas (including eight undifferentiated melanomas), and two MITF-rearranged melanocytic tumors. PRAME expression was scored from 0 to 4+ based on the percentage of tumor nuclei showing moderate to strong staining.

Results

All GNETs, MMNSTs, and MITF-rearranged tumors were PRAME-negative. EMPNSTs showed variable expression: six were negative (0–1), one equivocal (2+), and three positive (3–4+). Fifteen of 16 melanomas were PRAME-positive. PRAME scores differed significantly among tumor types (p = 1.99 × 10−5). PRAME demonstrated high sensitivity and specificity for distinguishing metastatic melanoma from primary mimickers including GNET and MMNST, but low specificity in EMPNST (71.4%).

Conclusions

PRAME reliably distinguishes metastatic melanoma from GNET and MMNST, supporting its use in this differential. However, its reduced specificity in EMPNST limits its standalone diagnostic value in this context, emphasizing the need for a multimodal approach.

背景:由于形态学和免疫表型的重叠,转移性黑色素瘤与恶性胃肠道神经外胚层肿瘤(GNET)、恶性黑素性神经鞘肿瘤(MMNST)和上皮样恶性周围神经鞘肿瘤(EMPNST)的鉴别具有重大的诊断挑战。PRAME是一种新的免疫组织化学标记物,越来越多地用于区分黑色素瘤和类似物,但在这些罕见的肿瘤类型中仍未得到充分的研究。方法:对4例GNETs、7例MMNSTs、10例EMPNSTs、16例转移性黑色素瘤(包括8例未分化黑色素瘤)和2例mitf重排黑色素细胞瘤进行PRAME免疫组化。根据中度至强烈染色的肿瘤核的百分比,PRAME的表达从0到4+评分。结果:所有GNETs、MMNSTs和mitf重排肿瘤均为prame阴性。EMPNSTs表现为不同的表达:阴性6例(0-1),模棱两可1例(2+),阳性3例(3-4+)。16例黑色素瘤中有15例prame阳性。不同肿瘤类型间PRAME评分差异有统计学意义(p = 1.99 × 10-5)。PRAME在区分转移性黑色素瘤与原发性模拟物(包括GNET和MMNST)方面具有很高的敏感性和特异性,但在EMPNST方面的特异性较低(71.4%)。结论:PRAME可靠地将转移性黑色素瘤与GNET和MMNST区分开来,支持其在这种区分中的应用。然而,其在EMPNST中的特异性降低限制了其在这种情况下的独立诊断价值,强调了多模式方法的必要性。
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引用次数: 0
Cutaneous Acanthamoebiasis: Two Cases Highlighting Diverse Histopathologic Findings 皮肤棘阿米巴病:两例不同的组织病理表现。
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-17 DOI: 10.1111/cup.14854
Nadia Siddiqui, Bicong Wu, Lindsay Gunnell, Evan George, Oliver Chang

Acanthamoeba is a free-living ameba, known to most commonly cause amebic keratitis in contact lens users and granulomatous amebic encephalitis in immunocompromised patients. Cutaneous Acanthamoebiasis as a single-organ manifestation is less common. We report two cases seen in our department. Case one is a 23-year-old hospitalized patient with systemic lupus erythematosus and pancytopenia who acutely developed nodules on the extremities. Biopsy showed lymphohistiocytic inflammation and focal coagulative necrosis in the subcutaneous fat. Acanthamoeba trophozoites and cysts were apparent in H&E-stained sections and highlighted by PAS and GMS stains. PCR studies were positive for Acanthamoeba species. Case two is an 81-year-old with diffuse large B-cell lymphoma on idelalisib, who presented with a three-week history of ulcerating nodules on the extremities. Biopsy from the left arm exhibited a mixed deep dermal infiltrate with neutrophils and histiocytes while biopsy from the right arm exhibited granulomatous inflammation. Acanthamoeba trophozoites and cysts were apparent in H&E and PAS-stained sections, confirmed to be Acanthamoeba by PCR. Cutaneous Acanthamoebiasis should be considered in biopsies of cutaneous nodules from immunocompromised patients. The histopathologic inflammatory changes are variable and non-specific, while the organisms are easily overlooked due to their resemblance to histiocytes. Familiarity with these features is essential for accurate, timely diagnosis.

棘阿米巴是一种自由生活的阿米巴原虫,已知最常引起隐形眼镜使用者阿米巴角膜炎和免疫功能低下患者阿米巴肉芽肿性脑炎。皮肤棘阿米巴病作为单一器官的表现是不常见的。我们报告两个在我们部门看到的病例。病例一是一名23岁的系统性红斑狼疮和全血细胞减少症住院患者,他在四肢急性发展为结节。活检显示皮下脂肪有淋巴组织细胞炎症和局灶性凝固性坏死。h&e染色切片可见棘阿米巴滋养体和囊肿,PAS和GMS染色突出。PCR检测结果为棘阿米巴原虫阳性。病例二为81岁的弥漫性大b细胞淋巴瘤患者,表现为三周的四肢溃疡性结节病史。左臂活检显示中性粒细胞和组织细胞混合深层真皮浸润,右臂活检显示肉芽肿性炎症。H&E和pas染色切片可见棘阿米巴滋养体和囊泡,经PCR证实为棘阿米巴。在免疫功能低下患者的皮肤结节活检中应考虑皮肤棘阿米巴病。组织病理学炎症变化是可变的和非特异性的,而生物体由于其与组织细胞的相似性而容易被忽视。熟悉这些特征对于准确、及时的诊断至关重要。
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引用次数: 0
Demographics of U.S. Dermatopathology Fellowship Applicant Interviewees: A Single Institution Descriptive Analysis 美国皮肤病理学奖学金申请人受访者的人口统计数据:单一机构描述性分析。
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-05 DOI: 10.1111/cup.14855
Peter Chow, Lauren F. Rinehart, Emily H. Smith, Kristin Smith, Gillian Heinecke, M. Yadira Hurley, Nicole Burkemper

Introduction

Matching into a dermatopathology fellowship is increasingly competitive; however, the application process is not standardized, and there is little data to guide a successful match. This study aimed to determine the association of various application variables and a successful match into dermatopathology fellowship.

Methods

A retrospective anonymized data review of dermatopathology fellowship applicants from a single midwestern institution was conducted. Applicant background and demographics were collected and analyzed with various statistical models.

Results

A total of 68 applicant interviewees over a 7-year period were included in the analysis, with 62% of interviewees ultimately matching into dermatopathology fellowship. Most applicants attended an allopathic medical school (90.8%) and were trained in pathology (64.7%). There was no significant correlation in gender, medical school background, residency training background, or research productivity. There was a statistically significant correlation of successful matching with higher mean United States Medical Licensing Examination step 2 scores (p value 0.012), but not for step 1 or 3 scores.

Conclusions

Results of this study show a minimal difference in dermatopathology application traits, suggesting that intangible factors may hold a greater importance for a successful match. This study provides prospective dermatopathology fellowship applicants with a description of application qualities that help to secure an interview.

导读:进入皮肤病理学研究团契的竞争日益激烈;然而,申请过程并不标准化,并且很少有数据来指导成功匹配。本研究旨在确定各种应用变量的关联,并成功匹配到皮肤病理学奖学金。方法:对来自中西部一家机构的皮肤病理学奖学金申请者进行回顾性匿名数据回顾。收集申请人的背景和人口统计数据,并使用各种统计模型进行分析。结果:在7年的时间里,共有68名申请人被纳入分析,62%的受访者最终匹配到皮肤病理学奖学金。大多数申请人就读于对抗疗法医学院(90.8%),并接受过病理学培训(64.7%)。性别、医学院背景、住院医师培训背景或研究效率无显著相关。成功匹配与较高的美国医师执照考试第2步平均分数有统计学意义(p值0.012),但与第1步和第3步分数无统计学意义。结论:本研究结果显示,在皮肤病理学应用特征的微小差异,表明无形因素可能对成功匹配更重要。这项研究提供了潜在的皮肤病理学奖学金申请者与应用质量的描述,有助于确保面试。
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引用次数: 0
Setting A New Standard for Histopathologic Reporting of Primary Cutaneous Melanoma: Progress Made by the Melanoma Reporting Task Force of the American Society of Dermatopathology 为原发性皮肤黑色素瘤的组织病理学报告制定新标准:美国皮肤病理学会黑色素瘤报告工作组取得的进展。
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-08-04 DOI: 10.1111/cup.14836
Adam I. Rubin, Christopher R. Shea, Beth S. Ruben, May P. Chan
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引用次数: 0
Minimum Pathology Reporting Elements for Melanoma: A Review of Reporting Guidelines and Proposal for Minimum Reporting Elements for a Quality Pathology Report by the Task Force of the American Society of Dermatopathology 黑色素瘤的最低病理报告要素:美国皮肤病理学会工作组对报告指南和高质量病理报告最低报告要素的建议的回顾。
IF 1.1 4区 医学 Q3 DERMATOLOGY Pub Date : 2025-07-31 DOI: 10.1111/cup.14848
Klaus J. Busam, Lyn M. Duncan, Pedram Gerami, Lori Lowe, Hina Sheikh, Michael Tetzlaff

Guidelines have been proposed for the pathology reporting of melanoma to ensure inclusion of data elements important for patient care. Compliance with guidelines has been made a yardstick for quality performance. However, there is controversy about how comprehensive a report must be, which is why the American Society of Dermatopathology has formed a task force with the goal of defining minimum data elements that should be included in a pathology report of a primary cutaneous melanoma. Importantly, additional information can or at times should be documented if a pathologist believes it is valuable to the clinical care team of a particular patient. The proposed minimum reporting guidelines outlined herein largely reflect core reporting elements by various professional organizations. Data elements must be included if they are needed for pathologic staging. Excisions require a margin status, but detailed margin metrics are not required for most cases. Furthermore, histopathologic subtyping of melanoma in situ is not routinely needed. Whether or not invasive melanoma should be subclassified depends on clinical relevance and whether the available evidence permits a definitive melanoma subclassification. When the minimum data elements are included, a pathology report should be considered compliant with quality reporting guidelines.

已经提出了黑色素瘤病理报告的指南,以确保包括对患者护理重要的数据元素。遵守指导方针已成为质量绩效的标准。然而,关于报告的全面程度有多大存在争议,这就是为什么美国皮肤病理学会(American Society of Dermatopathology)成立了一个工作组,目的是定义原发性皮肤黑色素瘤病理报告中应包含的最小数据元素。重要的是,如果病理学家认为附加信息对特定患者的临床护理团队有价值,则可以或有时应该将其记录下来。建议的最低限度报告准则在很大程度上反映了各专业组织的核心报告要素。如果需要病理分期,则必须包括数据元素。切除需要一个保证金状态,但详细的保证金指标不需要在大多数情况下。此外,通常不需要原位黑色素瘤的组织病理学分型。浸润性黑色素瘤是否应该进行亚分类取决于临床相关性和现有证据是否允许明确的黑色素瘤亚分类。当包含最低数据元素时,应认为病理报告符合质量报告指南。
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引用次数: 0
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Journal of Cutaneous Pathology
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