Sebaceous Gland Neoplasms

皮脂腺肿瘤
  • 文章类型: Journal Article
    目的:报告以铂类为基础的新辅助化疗(NACT)治疗眼睑和眼周皮脂腺癌(eSGC)的疗效。
    方法:对25例患者进行回顾性研究。
    结果:出现eSGC的平均年龄为59岁。平均肿瘤基底直径为46mm。按AJCC第8版分类,肿瘤属于T2(n=2,8%),T3(n=6,24%),和T4(n=17,68%);N1(n=12,48%);和M1(n=1,4%)。NACT与5-氟尿嘧啶(5-FU)和顺铂/卡铂在21(84%)/4(16%)患者中,分别。每位患者新辅助全身化疗的平均周期数为2(中位数,3).新辅助化疗后肿瘤基础体积的平均减少百分比为65%(中位数,60%)。NACT之后,12例(48%)患者接受手术治疗,6例(12%)患者行EBRT,4例(8%)接受辅助化疗。共有11例(44%)患者在治疗过程中失访,其中3人死于转移性疾病。16例患者随访时间≥3个月,11例(69%)患者肿瘤完全控制,14例(88%)局部肿瘤控制,在平均25个月的随访中,全球抢救人数为7人(44%)(中位数,7个月;范围,3至110个月)。在任何情况下都没有看到肿瘤复发。注意到一个(4%)严重的心脏毒性不良事件。
    结论:基于铂的NACT是具有晚期肿瘤和局部转移的eSGC的合适选择。不良事件很少见,在符合治疗的患者中,基于NACT的联合治疗提供全球挽救和全身肿瘤控制。
    OBJECTIVE: To report the outcomes of platinum-based neoadjuvant chemotherapy (NACT) for eyelid and periocular sebaceous gland carcinoma (eSGC).
    METHODS: Retrospective study of 25 patients.
    RESULTS: The mean age at presentation of eSGC was 59 years. The mean tumor basal diameter was 46 mm. By the 8th edition of AJCC classification, tumors belonged T2 (n = 2, 8%), T3 (n = 6, 24%), and T4 (n = 17, 68%); N1 (n = 12,48%); and M1 (n = 1, 4%). NACT with 5-fluorouracil (5-FU) and cisplatin/carboplatin was administered in 21 (84%)/4 (16%) patients, respectively. The mean number of cycles of neoadjuvant systemic chemotherapy per patient was 2 (median, 3). The mean percentage reduction of tumor basal volume after neoadjuvant chemotherapy was 65% (median, 60%). After NACT, 12 (48%) patients underwent surgical treatment, 6 (12%) patients underwent EBRT, and 4 (8%) underwent adjuvant chemotherapy. A total of 11 (44%) patients were lost to follow-up during the course of treatment, of whom 3 died from metastatic disease. In 16 patients followed up for ≥ 3 months, complete tumor control was achieved in 11 (69%) patients, local tumor control in 14 (88%), and globe salvage in 7 (44%) at a mean follow-up of 25 months (median, 7 months; range, 3 to 110 months). No tumor recurrence was seen in any case. One (4%) serious adverse event of cardiotoxicity was noted.
    CONCLUSIONS: Platinum-based NACT is a suitable option for eSGC with advanced tumors and locoregional metastasis. Adverse events are rare and in patients compliant with treatment, NACT-based combination therapy offers globe salvage and systemic tumor control.
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  • 文章类型: Journal Article
    局部复发预示眼睑皮脂腺癌(SC)预后不良。复发预测因素因研究而异。准确的复发估计对于眼睑SC的个体化治疗至关重要。本研究旨在确定复发预测因子,并开发用于眼睑SC个性化预测的列线图。
    我们进行了一项多中心回顾性队列研究。对418例连续的眼睑SC患者进行了图表回顾。所有患者均在初次手术后进行随访。采用多因素Cox回归分析探讨复发的独立预测因子。开发了用于复发预测的列线图,并通过Bootstrap重采样进行了验证。将预测准确性和辨别能力与肿瘤进行比较,节点,转移(TNM)分期系统。
    超过60个月的中位随访时间,167例(40%)患者局部复发。从诊断到复发的中位时间为14个月。1年累积复发率为18%。诊断延迟(危险比[HR]=1.01,95%置信区间[CI]=1.00-1.01,P=0.001),眼眶受累(HR=4.47,95%CI=3.04-6.58,P<0.001),Ki67(HR=1.01,95%CI=1.00-1.02,P=0.008)和术中冰冻切片控制的Mohs显微手术初始手术(HR=0.53,95%CI=0.35-0.80,P=0.003)是复发的独立影响因素。将这四个因素与pagetoid扩散相结合的列线图显示出令人满意的辨别能力(C指数=0.80-0.83;曲线下面积[AUC]=0.82-0.84),比较优于TNM分期(均P<0.05)。
    眼睑SC的复发率很高。建议早期发现和Mohs显微手术切除以控制复发。眼眶受累的患者,Ki67高表达,和pagetoid传播可能需要辅助措施。这个列线图提供了更准确的复发估计,帮助做出治疗决策。
    UNASSIGNED: Local recurrence predicts dismal prognosis in eyelid sebaceous carcinoma (SC). Recurrence predictors vary across studies. Accurate recurrence estimation is essential for individualized therapy in eyelid SC. This study aims to identify recurrence predictors and develop a nomogram for personalized prediction in eyelid SC.
    UNASSIGNED: We conducted a multicenter retrospective cohort study. Chart reviews were performed in 418 consecutive patients with eyelid SC. All patients were followed up after their initial surgery. Multivariate Cox regression was used to explore the independent predictors of recurrence. A nomogram for recurrence prediction was developed and validated with bootstrap resampling. The predictive accuracy and discriminative ability were compared with the Tumor, Node, Metastasis (TNM) staging system.
    UNASSIGNED: Over a median of 60-month follow-up, 167 patients (40%) had local recurrence. The median time from diagnosis to recurrence was 14 months. The 1-year cumulative recurrence rate was 18%. Diagnostic delay (hazard ratio [HR] = 1.01, 95% confidence interval [CI] = 1.00-1.01, P = 0.001), orbital involvement (HR = 4.47, 95% CI = 3.04-6.58, P < 0.001), Ki67 (HR = 1.01, 95% CI = 1.00-1.02, P = 0.008) and initial surgery of Mohs micrographic surgery with intraoperative frozen section control (HR = 0.53, 95% CI = 0.35-0.80, P = 0.003) were independent influencing factors of recurrence. A nomogram integrating these four factors combined with pagetoid spread displayed satisfactory discriminative ability (C-index = 0.80-0.83; area under the curve [AUC] = 0.82-0.84), which compared favorably than TNM staging (all P < 0.05).
    UNASSIGNED: The recurrence rate is high in eyelid SC. Early detection and primary resection with Mohs micrographic surgery are recommended in controlling recurrence. Patients with orbital involvement, high Ki67 expression, and pagetoid spread may require adjuvant measures. This nomogram offers more accurate recurrence estimates, aiding in therapeutic decision making.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    一名77岁的日本男子因右上臂和腋下的皮下肿瘤来到我们医院。活检显示皮肤附件肿瘤,显示顶腺分泌分化,腋窝淋巴结转移。放化疗缩小肿瘤后,两个病灶均被切除.切除的手臂肿瘤标本显示出多样化的组织学:(1)具有类器官模式和皮脂腺细胞的经典皮脂腺瘤;(2)具有细胞异型性的皮脂腺肿瘤;(3)乳头状肾小管肿瘤,显示出苍白的嗜酸性细胞的双相模式,具有腺体分泌分化和基底样细胞;(4)具有微乳头状结构的浸润性腺癌,让人想起乳腺浸润性微乳头状癌。腋窝肿瘤消退。据我们所知,这是第一例报道的皮肤附件肿瘤,皮脂腺肿瘤中出现侵袭性微乳头状结构。
    UNASSIGNED: A 77-year-old Japanese man presented to our hospital with subcutaneous tumors of the right upper arm and axilla. A biopsy revealed a cutaneous adnexal tumor, showing apocrine differentiation, and axillary lymph node metastasis. After chemoradiotherapy to shrink the tumors, both lesions were resected. A resected specimen of the arm tumor showed a variegated histology: (1) a classic sebaceoma with an organoid pattern and sebocytes; (2) a sebaceous tumor with cellular atypia; (3) a papillotubular tumor showing a biphasic pattern of pale eosinophilic cells with apocrine differentiation and basaloid cells; and (4) an invasive adenocarcinoma with a micropapillary structure, reminiscent of an invasive micropapillary carcinoma of the breast. The axillary tumor was regressed. To our knowledge, this is the first reported case of an adnexal tumor of the skin with an invasive micropapillary structure arising in a sebaceous tumor.
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  • 文章类型: Journal Article
    大约5-15%的所有皮肤恶性肿瘤表现在上下眼睑。原发类型包括基底细胞癌,鳞状细胞癌,皮脂腺细胞癌,默克尔细胞癌和黑色素瘤紧随其后。基底细胞癌主要影响下眼睑,还有其他各种癌症,黑色素瘤,转移,不同来源的肿瘤可能出现在上下眼睑。危险因素,如高龄,吸烟,尤其是,暴露于UV光显着有助于这些眼睑病变的发展。尽管发病率越来越高,对皮肤性眼睑恶性肿瘤的研究仍然有限。然而,鉴于许多全身性肿瘤恶性肿瘤最初表现为转移性眼睑病变,因此这项研究势在必行。本文对眼睑解剖进行了深入的探索,临床表现,诊断,和治疗管理。要点:眼睑转移占所有眼睑癌的不到百分之一,然而,它们通常作为潜在的系统性恶性肿瘤的初始指征。早期发现和治疗对于改善患者预后和生活质量至关重要。治疗方案包括一系列模式,以Mohs手术为眼部肿瘤切除的金标准。其他治疗选择包括局部切除以及非手术干预,如放疗,冷冻疗法,免疫疗法,和局部用药。
    Approximately 5-15% of all dermatologic malignancies manifest in the upper and lower eyelids. The primary types include basal cell carcinoma, squamous cell carcinoma, and sebaceous cell carcinoma, with Merkel cell carcinoma and melanoma following closely behind. Basal cell carcinoma predominantly affects the lower eyelid, yet various other carcinomas, melanomas, metastases, and neoplasms of diverse origins can arise on both upper and lower eyelids. Risk factors such as advanced age, smoking, and notably, exposure to UV light significantly contribute to the development of these eyelid lesions. Despite the increasing incidence, research on dermatologic eyelid malignancies remains limited. However, such study is imperative given that many systemic oncologic malignancies initially present as metastatic eyelid lesions. This paper provides an in-depth exploration of eyelid anatomy, clinical presentation, diagnosis, and treatment management.Key Points: Eyelid metastases represent less than one percent of all eyelid cancers, yet they often serve as the initial indication of an underlying systemic malignancy. Early detection and treatment is crucial in improving prognosis and quality of life for patients. Treatment options encompass a range of modalities, with Mohs surgery as the gold standard for the removal of ocular tumors. Additional treatment options include local excision as well as non-surgical interventions such as radiotherapy, cryotherapy, immunotherapy, and topical medications.
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  • 文章类型: Journal Article
    背景:眼睑皮脂腺癌(SGC)是一种侵袭性肿瘤,具有转移能力和增加的发病率。关于这种恶性眼睑肿瘤的流行病学的争议在科学文献中很普遍。西方报道反复将眼睑SGC描述为一种罕见的肿瘤,占所有眼睑肿瘤的1%-3%,然而,来自亚洲的研究发现眼睑SGC的频率更高,包括日本54%的眼睑肿瘤,印度占43%-56%。我们希望检索眼睑SGC患病率与总眼睑肿瘤成比例的观察数据,从世界各地发表的病理学研究中解决了这一争议。
    方法:我们将搜索OvidMedline,EMBASE,Cochrane中央控制试验登记册,Scopus和谷歌学者确定已发表的关于眼睑SGC患病率比例的报告,旨在澄清肿瘤的发病率。我们将包括观察性临床病理研究,报告患病率并确认组织病理学。对出版日期或语言没有限制。来自个体研究和研究质量的数据将由两名个体审阅者提取。研究质量将使用JBI关键评估仪器评估研究报告患病率数据。将使用用于荟萃分析的随机效应模型来转换和汇集原始比例。并根据地理位置进行亚组分析。如果数据被认为不适合进行荟萃分析,将呈现叙事综合。我们将判断证据的确定性,并提出这是否对结果有总体影响。结果可能会揭示科学文献的长期学术差异。
    背景:本系统评价不需要道德批准。这项拟议审查的结果将成为眼科或病理专业内国际同行评审期刊上出版物的主题。
    CRD42023487141。
    BACKGROUND: Sebaceous gland carcinoma (SGC) of the eyelid is an aggressive tumour with the ability to metastasise and an increased morbidity. Controversies regarding the epidemiology of this malignant eyelid tumour is widespread in the scientific literature. Western reports repeatedly describes eyelid SGC as a rare occurring tumour in general, accounting for 1%-3% of all eyelid tumours, however studies from Asia have uncovered a higher frequency of eyelid SGC including 54% of all eyelid tumours in Japan, and 43%-56% in India. We wish to retrieve observational data of eyelid SGC prevalence in proportion to total eyelid tumours, from pathological studies published worldwide to resolve this controversy.
    METHODS: We will search Ovid Medline, EMBASE, Cochrane Central Register of Controlled Trials, Scopus and Google Scholar to identify published reports on eyelid SGC prevalence proportions, aiming to clarify the incidence of the tumour. We will include observational clinicopathological studies reporting prevalence with confirmed histopathology. No limitations on publication date or language will be applied. Data from the individual studies and study quality will be extracted by two individual reviewers. Study quality will be assessed using the JBI Critical Appraisal Instrument for Studies Reporting Prevalence Data. Raw proportions will be transformed and pooled using a random effects model for meta-analysis. And subgroup analysis according to geography will be performed. If data are deemed unsuitable for a meta-analysis, a narrative synthesis will be presented. We will judge the certainty of evidence and present whether this has an overall effect on the results. The results may shed light on a long-standing academic disparity of the scientific literature.
    BACKGROUND: This systematic review does not require ethical approval. The results of this proposed review will be the subject to a publication in an international peer-reviewed journal within the ophthalmic or pathological specialty.
    UNASSIGNED: CRD42023487141.
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  • 文章类型: Journal Article
    脑型皮脂腺痣(CSN)是一种罕见的形态皮脂腺痣变种,由于其平坦,在产前诊断具有挑战性,光滑和蜡质的外观,与皮外表现缺乏关联。妊娠24周时,多胎患者被转诊到我们的三级产科单元,以评估胎儿耳廓病变。我们能够借助三维(3D)技术通过连续产科超声成像进一步表征病变。尽管产前的准确诊断尚不确定,使用3D技术可以重建胎儿皮肤病变,进行多学科评估,以促进新生儿管理计划的制定。CSN的诊断是在出生后进行活检。
    Cerebriform sebaceous naevus (CSN) is a rare morphological sebaceous naevus variant and challenging to diagnose prenatally due to its flat, smooth and waxy appearance and lack of association with extracutaneous manifestations.A multigravida was referred to our tertiary obstetric unit at 24 weeks of gestation for evaluation of fetal auricular lesions. We were able to further characterise the lesions via serial obstetric ultrasound imaging with the aid of three-dimensional (3D) technology. Although the precise diagnosis prenatally was uncertain, the use of 3D technology allowed the reconstruction of the fetal cutaneous lesions for multidisciplinary assessment to facilitate the development of a neonatal management plan. The diagnosis of CSN was made postnatally on biopsy.
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  • 文章类型: Case Reports
    患有Muir-Torre综合征的患者可能患有全身性恶性肿瘤和皮脂腺肿瘤,例如腺瘤,上皮瘤,和/或癌。该综合征通常由一个或多个错配修复基因中的种系突变引起。医源性或获得性免疫抑制可以促进皮脂腺肿瘤的出现,既可以作为孤立事件,也可以作为Muir-Torre综合征的一个特征,并且可以揭开遗传易患该综合征的个体的面纱。描述了两名具有Muir-Torre综合征特征的医源性免疫抑制男性。类似于这些免疫功能低下的男性,Muir-Torre综合征相关皮脂腺肿瘤发生于实体器官移植受者,人类免疫缺陷病毒感染的个体,以及接受免疫抑制剂治疗的慢性病患者。Muir-Torre综合征相关皮脂腺肿瘤在肾脏受者中更频繁和更早发生,接受更多移植后免疫抑制剂的人,而不是肝脏接受者。通过用西罗莫司或依维莫司代替环孢菌素或他克莫司减少皮脂腺肿瘤的发展。特异性抗癌疫苗或检查点阻断免疫疗法可能值得探索Muir-Torre综合征相关皮脂腺肿瘤和综合征相关内脏癌症的免疫拦截。我们建议,对于所有患有Muir-Torre综合征相关皮脂腺肿瘤的患者,无论是免疫活性的还是免疫抑制的患者,都应常规进行错配修复基因基因组畸变的种系测试。
    Patients with Muir-Torre syndrome may have a systemic malignancy and a sebaceous neoplasm such as an adenoma, epithelioma, and/or carcinoma. The syndrome usually results from a germline mutation in one or more mismatch repair genes. Iatrogenic or acquired immunosuppression can promote the appearance of sebaceous tumors, either as an isolated event or as a feature of Muir-Torre syndrome and may unmask individuals genetically predisposed to the syndrome. Two iatrogenically immunosuppressed men with Muir-Torre syndrome features are described. Similar to these immunocompromised men, Muir-Torre syndrome-associated sebaceous neoplasms have occurred in solid organ transplant recipients, human immunodeficiency virus-infected individuals, and patients with chronic diseases who are treated with immunosuppressive agents. Muir-Torre syndrome-associated sebaceous neoplasms occur more frequently and earlier in kidney recipients, who are receiving more post-transplant immunosuppressive agents, than in liver recipients. The development of sebaceous neoplasms is decreased by replacing cyclosporine or tacrolimus with sirolimus or everolimus. Specific anti-cancer vaccines or checkpoint blockade immunotherapy may merit exploration for immune-interception of Muir-Torre syndrome-associated sebaceous neoplasms and syndrome-related visceral cancers. We suggest germline testing for genomic aberrations of mismatch repair genes should routinely be performed in all patients-both immunocompetent and immunosuppressed-who develop a Muir-Torre syndrome-associated sebaceous neoplasm.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    皮脂腺癌是罕见的恶性皮肤附件肿瘤,伴有皮脂腺分化。典型的好发区域是头颈部,皮脂腺癌是最常见的皮肤附件恶性肿瘤。根据它们的定位,眼周皮脂腺癌和眼外皮脂腺癌之间有区别。如果怀疑Muir-Torre综合征(MTS),应始终排除。在预后方面,皮脂腺癌是潜在的侵袭性肿瘤,具有明显的复发和转移趋势。只有完全切除的小眼外皮脂腺癌的预后非常好。皮脂腺癌最常通过淋巴途径转移到区域或远处淋巴结;器官转移发生频率较低。眼周皮脂腺癌的转移率(高达15%)高于眼外皮脂腺癌(高达2%)。原发肿瘤的完全显微控制手术(MCS)是首选的治疗方法,无论眼周或眼外定位。可以考虑辅助或治疗性放疗。目前还没有确定的晚期标准疗法,无法操作,或转移性皮脂腺癌。可以考虑局部程序和全身疗法,例如化疗或免疫疗法。该程序应由跨学科肿瘤委员会单独确定。建议对这些潜在的侵袭性癌进行密切随访。
    Sebaceous gland carcinomas are rare malignant cutaneous adnexal tumors with sebocytic differentiation. The typical predilection area is the head and neck region, where sebaceous gland carcinomas are the most common malignant adnexal tumors of the skin. According to their localization a distinction is made between periocular and extraocular sebaceous gland carcinomas. Muir-Torre syndrome (MTS) should always be ruled out if it is suspected. In terms of prognosis, sebaceous gland carcinomas are potentially aggressive tumors with a clear tendency to recur and metastasize. Only small extraocular sebaceous gland carcinomas that have been completely resected have a very good prognosis. Sebaceous gland carcinomas most frequently metastasize lymphogenously to regional or distant lymph nodes; organ metastasis occurs less frequently. Periocular sebaceous gland carcinomas have a higher metastasis rate (up to 15%) than extraocular sebaceous gland carcinomas (up to 2%). Complete micrographically controlled surgery (MCS) of the primary tumor is the therapy of first choice, regardless of periocular or extraocular localization. Adjuvant or therapeutic radiotherapy may be considered. There is currently no established standard therapy for advanced, inoperable, or metastatic sebaceous gland carcinomas. Local procedures and systemic therapies such as chemotherapy or immunotherapy can be considered. The procedure should be determined individually by an interdisciplinary tumor board. Close follow-up care is recommended for these potentially aggressive carcinomas.
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