acral

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  • 文章类型: Case Reports
    转移性手部肿瘤并不常见,但在手部肿块的鉴别诊断中很重要。在这项研究中,我们报告了1例患者最初出现手部疼痛和肿胀,除了广泛的吸烟史外,没有其他相关病史.随后的活检和检查显示转移性肺癌。作为原发性肿瘤的第一表现的手端转移是一种罕见但令人衰弱的疾病,预后不良。手外科医师必须继续意识到在没有已知恶性肿瘤的患者中转移性手肿瘤的可能性,并倡导迅速开始多学科护理和治疗,以最大程度地提高患者的预后。
    Metastatic hand tumors are uncommon but important to include in the differential diagnosis for hand masses. In this study, we report the case of a patient presenting initially with hand pain and swelling with no other pertinent medical history except for an extensive smoking history. Subsequent mass biopsy and work-up revealed metastatic lung cancer. Acral metastases to the hand as the first manifestation of a primary tumor are a rare but debilitating condition with a poor prognosis. Hand surgeons must remain aware of the potential for metastatic hand tumors in patients without known malignancy and advocate for the prompt initiation of multidisciplinary care and treatment to maximize patient outcomes.
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  • 文章类型: Journal Article
    背景:辅助治疗改善了术后黑色素瘤患者的临床预后。然而,在之前的试验中,该疗法对黑色素瘤肢端和粘膜亚型的长期疗效尚未得到充分评估.这项研究评估了黑色素瘤患者的3年无复发生存率和总生存率。包括肢端和粘膜亚型,用抗PD-1抗体(Ab)或BRAF和MEK抑制剂dabrafenib和trametinib的组合治疗。
    方法:我们回顾性分析了120例抗PD-1抗体(Ab)治疗患者的3年复发时间(TTR)和总生存期(OS),或与dabrafenib和trametinib的组合。
    结果:总TTR中位数为18.4个月,范围为0.69到36个月。肢端和粘膜类型的3年TTR分别为28.1%和38.5%,分别。在亚组分析中,基线肿瘤厚度(TT)和肢端类型与TTR相关。此外,在多项分析中,我们将104例肢端和非肢端皮肤患者分为抗PD-1Abs或dabrafenib+trametinib联合治疗队列.肢端亚型和TT是重要的预后因素。在3年操作系统中,在单变量和多重分析中,只有肿瘤溃疡与OS相关.粘膜类型的基线或治疗相关因素没有显着差异(p>0.05)。
    结论:这项研究表明,在3年TTR终点时,非肢端皮肤黑色素瘤的辅助治疗比肢端或粘膜类型更有效。
    BACKGROUND: Adjuvant therapy has improved the clinical prognosis for postoperative melanoma patients. However, the long-term efficacy of this therapy on the melanoma acral and mucosal subtypes has not been fully evaluated in previous trials. This study assessed the 3-year recurrence-free survival and overall survival of patients with melanoma, including the acral and mucosal subtypes, treated with anti-PD-1 antibody (Ab) or with the combination of the BRAF and MEK inhibitors dabrafenib and trametinib.
    METHODS: We retrospectively analyzed both the 3-year time to relapse (TTR) and overall survival (OS) of 120 patients treated with anti-PD-1 antibody (Ab), or with the combination of dabrafenib and trametinib.
    RESULTS: The overall median TTR was 18.4 months, with a range of 0.69 to 36 months. The 3-year TTR of the acral and mucosal types was 28.1% and 38.5%, respectively. Baseline tumor thickness (TT) and acral type were associated with the TTR in subgroup analysis. Moreover, we classified 104 acral and non-acral cutaneous patients into the anti-PD-1 Abs or dabrafenib plus trametinib combined therapies cohort in multiple analyses. The acral subtype and TT were detected as important prognostic factors. In the 3-year OS, only tumor ulceration was associated with the OS in both univariate and multiple analyses. There was no significant difference in baseline or treatment-related factors of the mucosal type (p > 0.05).
    CONCLUSIONS: This study suggests that adjuvant therapy is more effective with non-acral cutaneous melanoma than either the acral or mucosal types at the 3-year TTR endpoint.
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  • 文章类型: Journal Article
    背景:手突光化性角化病(AK)病变被认为难以治疗,关于这些病变的光动力疗法(PDT)的公开数据有限。因此,我们评估了持续的疗效,安全,PDT后对手上的AK的满意度。
    方法:我们分析了随机分组的手部治疗数据,双盲,个体内部III期研究。所有参与者先前都经历了多达两个具有10%5-氨基乙酰丙酸纳米乳液凝胶(BF-200ALA)的场定向红光PDT。评估包括PDT期间的疼痛,清除率和复发率,和满意度。
    结果:包括24名在手上治疗的参与者;分析了21名参与者。BF-200ALA的完全清除率为90.9%(基于病变)和76.2%(每个参与者侧),两者都明显高于车辆。BF-200ALA的病灶复发率为29.0%。不良事件反映了行动模式。在11分数字评定量表上,平均疼痛强度为4.8±3.8(BF-200ALA)和0.8±2.1(媒介物)。大多数参与者(81.0%)对BF-200ALA的满意度评价为非常好或良好。
    结论:此亚组分析表明,使用BF-200ALA的PDT为手部AK病变提供了合适的治疗方法。
    BACKGROUND: Acral actinic keratosis (AK) lesions are considered difficult to treat, and published data for photodynamic therapy (PDT) on these lesions is limited. Thus, we evaluated sustained efficacy, safety, and satisfaction after PDT for AK on the hands.
    METHODS: We analysed subgroup data for treatment on the hands from a randomised, double-blind, intra-individual phase III study. All participants previously underwent up to two field-directed red light PDTs with 10 % 5-aminolevulinic acid nanoemulsion gel (BF-200 ALA). Assessments included pain during PDT, clearance and recurrence rates, and satisfaction.
    RESULTS: 24 participants treated on the hands were included; 21 participants were analysed. Complete clearance rates with BF-200 ALA were 90.9 % (lesion-based) and 76.2 % (per participant\'s side), both markedly higher than with vehicle. The lesion recurrence rate with BF-200 ALA was 29.0 %. Adverse events reflected the mode of action. Mean pain intensities were 4.8 ± 3.8 (BF-200 ALA) and 0.8 ± 2.1 (vehicle) on an 11-point numeric rating scale. Most participants (81.0 %) rated their satisfaction with BF-200 ALA as very good or good.
    CONCLUSIONS: This subgroup analysis indicates that PDT with BF-200 ALA provides a suitable treatment for AK lesions on the hands.
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  • 文章类型: Journal Article
    尽管在治疗皮肤黑色素瘤方面取得了进展,肢端和粘膜(A/M)黑色素瘤患者的治疗选择仍然有限,预后较差.我们分析了156例黑色素瘤(101例皮肤,28肢,和27粘膜)使用FoundationOne癌症基因特异性临床测试平台,并确定了新的,A/M黑色素瘤特定解剖部位的潜在靶向基因组改变(GA)。使用新的A/M黑色素瘤临床前模型,我们证明了与皮肤黑素瘤相关的几种GA和相应的致癌途径在A/M黑素瘤中具有相似的靶向性。其他改动,包括MYC和CRKL扩增,是A/M黑色素瘤特有的,并且对使用BRD4抑制剂JQ1或Src/ABL抑制剂达沙替尼的间接靶向敏感,分别。我们进一步确定了新的,可操作的A/M特定更改,包括体内对达沙替尼反应的粘膜黑色素瘤中NF2融合失活。我们的研究强调了皮肤和A/M黑色素瘤之间新的分子差异,在A/M内的不同解剖部位,这可能会改变这些罕见黑素瘤的临床试验和治疗模式。
    Despite advancements in treating cutaneous melanoma, patients with acral and mucosal (A/M) melanomas still have limited therapeutic options and poor prognoses. We analyzed 156 melanomas (101 cutaneous, 28 acral, and 27 mucosal) using the Foundation One cancer-gene specific clinical testing platform and identified new, potentially targetable genomic alterations (GAs) in specific anatomic sites of A/M melanomas. Using novel pre-clinical models of A/M melanoma, we demonstrate that several GAs and corresponding oncogenic pathways associated with cutaneous melanomas are similarly targetable in A/M melanomas. Other alterations, including MYC and CRKL amplifications, were unique to A/M melanomas and susceptible to indirect targeting using the BRD4 inhibitor JQ1 or Src/ABL inhibitor dasatinib, respectively. We further identified new, actionable A/M-specific alterations, including an inactivating NF2 fusion in a mucosal melanoma responsive to dasatinib in vivo. Our study highlights new molecular differences between cutaneous and A/M melanomas, and across different anatomic sites within A/M, which may change clinical testing and treatment paradigms for these rare melanomas.
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  • 文章类型: Journal Article
    足肢端黑色素瘤(AMF)的临床特征和发病机理尚未得到充分阐明。足部的临床或亚临床持续性炎症是由足部皮肤癣菌病(DPF)引起的。持续性炎症可能与肿瘤发生有关。此外,据报道,糖尿病与皮肤癣菌病和癌症的发展有关。本研究旨在阐明DPF和AMF之间的临床关联。考虑到糖尿病。回顾性检查了114名日本患者的病历,并将其分为AMF组(n=30)和由黑色素瘤以外的足部疾病患者组成的对照组(n=84)。对所有报告足部症状的患者进行显微镜DPF筛查,有或没有AMF。患者接受显微镜检查以检测皮肤癣菌的存在,并根据阳性结果做出DPF的诊断。在AMF组中,18例(60.0%)和8例(26.7%)患者患有DPF和糖尿病,分别。4例患者(13.3%)同时患有DPF和糖尿病。在对照组中,25例(29.8%)和11例(13.1%)患者患有DPF和糖尿病,分别。5例患者(6.0%)同时患有DPF和糖尿病。单变量分析显示,AMF组中DPF的患病率明显高于对照组(比值比,3.540;p=0.003,Pearsonχ2检验)。此外,性别的多变量分析,身体质量指数,DPF,糖尿病显示DPF是与AMF相关的重要因素(比值比,4.285;p=0.002,逻辑回归分析)。在AMF患者中观察到的DPF过度角化类型比在对照组患者中更常见(优势比,11.083;p<0.001,Pearsonχ2检验)。总之,本研究发现DPF的患病率明显更高,尤其是它的过度角化型,AMF患者。DPF可能与AMF发病有关。
    The clinical characteristics and pathogenesis of acral melanoma of the foot (AMF) have not been sufficiently elucidated. Clinical or subclinical persistent inflammation of the feet is caused by dermatophytosis of the feet (DPF). Persistent inflammation is potentially associated with oncogenesis. Moreover, diabetes has been reported to be associated with the development of dermatophytosis and cancer. The present study aimed to elucidate the clinical association between DPF and AMF, with consideration of diabetes. The medical records of 114 Japanese patients were retrospectively examined and divided into an AMF group (n = 30) and a control group consisting of patients with foot diseases other than melanoma (n = 84). Microscopic DPF screening was performed on all patients who reported symptoms in the foot, with or without AMF. Patients underwent a microscopic test to detect the presence of dermatophytes, and the diagnosis of DPF was made based on a positive result. In the AMF group, 18 (60.0%) and eight (26.7%) patients had DPF and diabetes, respectively. Four patients (13.3%) had both DPF and diabetes. In the control group, 25 (29.8%) and 11 (13.1%) patients had DPF and diabetes, respectively. Five patients (6.0%) had both DPF and diabetes. Univariate analyses showed a significantly higher prevalence of DPF in the AMF group than in the control group (odds ratio, 3.540; p = 0.003, Pearson χ2 test). Furthermore, multivariate analyses of sex, body mass index, DPF, and diabetes revealed DPF as a significant factor associated with AMF (odds ratio, 4.285; p = 0.002, logistic regression analysis). The hyperkeratotic type of DPF was more frequently observed in patients with AMF than in control patients (odds ratio, 11.083; p < 0.001, Pearson χ2 test). In conclusion, the present study found a significantly higher prevalence of DPF, especially its hyperkeratotic type, in patients with AMF. DPF may be associated with AMF pathogenesis.
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  • 文章类型: Journal Article
    在这项研究中,我们描述了1例原发性皮肤肢端CD8阳性淋巴增殖性疾病位于非肢端区的患者.一名65岁的男性表现为不明确的橡胶稠度病变,右大腿的最大直径为2.5cm。组织学上,它由弥漫性真皮浸润的中型非典型淋巴细胞组成,这些淋巴细胞表达CD3,CD8和TIA-1.此外,观察到CD68的特征性核旁阳性.在后续行动中,患者腹部疾病复发,病灶形态和表型相似.据我们所知,英语文献中已经描述了<20例原发性皮肤肢端CD8阳性淋巴增生性疾病的非肢端表现。虽然罕见,它的鉴定对于将其与表达CD8和细胞毒性标志物的其他T细胞淋巴瘤区分开来至关重要,他们的临床课程非常积极。
    In this study, we describe a patient of primary cutaneous acral CD8-positive lymphoproliferative disorder located in a nonacral region. A 65-year-old male presented with an ill-defined lesion of rubbery consistency and a maximum diameter of 2.5 cm localized in the right thigh. Histologically, it was composed of a diffuse dermal infiltration of medium-sized atypical lymphocytes that expressed CD3, CD8, and TIA-1. In addition, a characteristic paranuclear positivity with CD68 was observed. During the follow-up, the patient had a recurrence of the disease in the abdomen with a lesion showing similar morphology and phenotype. To our knowledge, < 20 patients of primary cutaneous acral CD8-positive lymphoproliferative disorder with a nonacral presentation have been described in English literature. Although rare, its identification is essential to differentiate it from other T-cell lymphoma that express CD8 and cytotoxic markers, and whose clinical courses are very aggressive.
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  • 文章类型: Case Reports
    COVID-19是一场始于2020年的全球大流行。大流行期间,患者出现COVID-19感染的各种皮肤表现。目前,COVID-19感染在全球范围内仍然存在,虽然程度较小。该病例报告描述了一名21岁的男性患者,他的手和脚踝外侧出现丘疹囊泡三天。该患者还出现了8天的呼吸道症状,并检测出COVID-19阳性。重要的是要了解与COVID-19相关的皮肤表现,因为这可以帮助诊断和理解病情。
    COVID-19 is a global pandemic that started in 2020. During the pandemic, patients have presented with various cutaneous manifestations of COVID-19 infections. Currently, COVID-19 infections are still present worldwide, although to a lesser extent. This case report describes a 21-year-old male patient who presented with papulovesicles on his hands and lateral aspects of his ankles for three days. The patient also experienced respiratory symptoms for eight days and tested positive for COVID-19. It is important to have an understanding of the skin manifestations associated with COVID-19, as this can aid in the diagnosis and comprehension of the condition.
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  • 文章类型: Multicenter Study
    粘膜(MM)和肢端黑素瘤(AM)是临床需求未满足的罕见黑素瘤亚型;15%-20%有可能被小分子抑制剂靶向的KIT突变,但还没有被批准用于黑色素瘤.这个多中心,单臂II期试验(NICAM)研究尼洛替尼在KIT突变的转移性MM和AM中的安全性和活性。在39/219名筛查患者(18%)中发现了KIT突变;在29/39接受治疗的患者中,26可用于初步分析。6例患者在6个月时存活且无进展(当地放射学回顾,25%);5/26(19%)在12周时有客观反应;中位OS为7.7个月;ddPCR检测正确识别了16/17患者循环肿瘤DNA(ctDNA)的KIT改变。尼洛替尼在KIT突变AM和MM中具有活性,与其他KIT抑制剂相比,在非热点KIT突变中具有明显的活性,支持AM和MM中KIT评估的扩展。我们的结果支持尼洛替尼治疗KIT突变黑色素瘤的进一步研究。该临床试验已在ISRCTN(ISRCTN39058880)和EudraCT(2009-012945-49)注册。
    Mucosal (MM) and acral melanomas (AM) are rare melanoma subtypes of unmet clinical need; 15%-20% harbor KIT mutations potentially targeted by small-molecule inhibitors, but none yet approved in melanoma. This multicenter, single-arm Phase II trial (NICAM) investigates nilotinib safety and activity in KIT mutated metastatic MM and AM. KIT mutations are identified in 39/219 screened patients (18%); of 29/39 treated, 26 are evaluable for primary analysis. Six patients were alive and progression free at 6 months (local radiology review, 25%); 5/26 (19%) had objective response at 12 weeks; median OS was 7.7 months; ddPCR assay correctly identifies KIT alterations in circulating tumor DNA (ctDNA) in 16/17 patients. Nilotinib is active in KIT-mutant AM and MM, comparable to other KIT inhibitors, with demonstrable activity in nonhotspot KIT mutations, supporting broadening of KIT evaluation in AM and MM. Our results endorse further investigations of nilotinib for the treatment of KIT-mutated melanoma. This clinical trial was registered with ISRCTN (ISRCTN39058880) and EudraCT (2009-012945-49).
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  • 文章类型: Review
    钙化性软骨样间充质肿瘤是最近公认的骨和软组织实体,主要在四肢和颞下颌关节中发现。这种肿瘤通常由FN1基因与激酶的融合驱动。在这个案例报告中,我们提供了位于左侧大脚趾的罕见浅表钙化软骨样间充质肿瘤的详细说明,其特征在于FN1::FGFR2融合。肿瘤表现出外周结扎,由大型皮内组织细胞样上皮样细胞组成,没有有丝分裂活性。这些细胞表现出良好的染色质和丰富的苍白嗜酸性细胞浆,形成漩涡状的合胞体。它们散布着玻璃状软骨粘液基质的局部区域,其中包含随机矿化的钙化物质和分离的破骨细胞样巨细胞。RNA测序证实了FN1(外显子29)::FGFR2(外显子7)基因融合物的存在。我们的报告强调皮肤病理学家在评估显示间充质的浅表病变时考虑这个实体的重要性,软骨样,和钙化属性。
    Calcified chondroid mesenchymal neoplasm is a recently recognized bone and soft tissue entity primarily found in the extremities and the temporomandibular joint. This neoplasm is typically driven by the fusion of the FN1 gene with a kinase. In this case report, we provide a detailed account of a rare superficial calcified chondroid mesenchymal neoplasm located on the left big toe, characterized by an FN1::FGFR2 fusion. The tumor exhibited a peripheral collarette and consisted of large intradermal histiocytoid to epithelioid cells with no mitotic activity. These cells displayed fine chromatin and abundant pale eosinophilic cytoplasm, forming a swirling syncytium. They were interspersed with localized areas of glassy chondromyxoid matrix containing randomly mineralized calcific material and isolated osteoclast-like giant cells. RNA sequencing confirmed the presence of an FN1 (exon 29)::FGFR2 (exon 7) gene fusion. Our report emphasizes the importance for dermatopathologists to consider this entity when evaluating superficial lesions displaying mesenchymal, chondroid, and calcified attributes.
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  • 文章类型: Case Reports
    暂无摘要。
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