pilomatrixoma

毛心房瘤
  • 文章类型: Case Reports
    Pilomatrixoma,也被称为Malherbe钙化上皮瘤,是一种起源于毛囊基质细胞的罕见良性皮肤肿瘤。它通常作为一家公司,无痛皮下结节,最常见于头部,脖子,和上肢。Pilomatrixoma偶尔会出现在不典型的位置,由于其非特异性临床表现,构成了诊断挑战。一名43岁的女性出现了无痛,她左脚踝外侧的肿块慢慢扩大,已经存在了大约一年。体检显示,界限清楚的皮下结节,直径约2厘米,正常覆盖皮肤。左脚踝的MRI显示界限清楚,具有不同信号强度的皮下肿块,与钙化一致,提示毛结肠瘤.细针穿刺活检证实了基底细胞的存在,阴影细胞,和钙化区域。手术切除了病灶,和组织病理学检查证实了毛心房瘤的诊断。病人术后进展顺利,在6个月的随访中没有复发。该病例强调了在皮下结节的鉴别诊断中考虑毛房瘤的重要性,即使在不寻常的地方。全面的诊断方法,包括临床评估,成像,和组织病理学检查,对准确诊断至关重要。切缘清晰的手术切除是首选治疗方法,确保低复发率和良好的患者预后。本报告增强了对毛心房瘤的理解,并强调了有效管理这种罕见疾病的多模式诊断策略的必要性。
    Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a rare benign skin tumor originating from hair follicle matrix cells. It typically presents as a firm, painless subcutaneous nodule, most commonly found in the head, neck, and upper extremities. Pilomatrixoma can occasionally appear in atypical locations, posing a diagnostic challenge due to its nonspecific clinical presentation. A 43-year-old female presented with a painless, slowly enlarging mass on the lateral side of her left ankle, which had been present for approximately one year. Physical examination revealed a firm, well-circumscribed subcutaneous nodule measuring about 2 cm in diameter with normal overlying skin. An MRI of the left ankle demonstrated a well-circumscribed, subcutaneous mass with heterogeneous signal intensity, consistent with calcifications, suggesting pilomatrixoma. A fine-needle aspiration biopsy confirmed the presence of basaloid cells, shadow cells, and areas of calcification. The lesion was surgically excised, and histopathological examination validated the diagnosis of pilomatrixoma. The patient had an uneventful postoperative course, with no recurrence at the six-month follow-up. This case underscores the importance of considering pilomatrixoma in the differential diagnosis of subcutaneous nodules, even in unusual locations. A comprehensive diagnostic approach, including clinical evaluation, imaging, and histopathological examination, is essential for an accurate diagnosis. Surgical excision with clear margins is the treatment of choice, ensuring low recurrence rates and excellent patient outcomes. This report enhances the understanding of pilomatrixoma and highlights the necessity for a multimodal diagnostic strategy in managing this rare condition effectively.
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  • 文章类型: Journal Article
    背景:Pilomatricoma(PM)是一种源自毛发基质的皮肤良性肿瘤。它在临床上表现为覆盖正常表皮的孤立而坚固的结节,通常在早期不容易被注意到。然而,当特殊的大疱性病变在短时间内发生甚至溃疡时,皮肤科医生的术前诊断通常具有挑战性,尤其是当儿科患者拒绝活检时。
    方法:我们介绍了6例大疱性PM病例,特别是对皮肤镜检查和组织病理学检测数据进行了相关性分析。基本信息,病史,还提供了患者的症状和病变形态结果。我们发现女性大疱性PM的发病率高于男性,大多数患者是青少年,好发部位似乎与疫苗注射部位一致。报告的大疱性PM的皮肤镜特征是下面的发光黄色结构,具有灰蓝色均匀区域和分支毛细管。组织学特征与PM一致,肿瘤上方有明显的表皮大疱,真皮上部淋巴结异常扩张。本研究中描述的患者是汉族人群中的中国患者,包括4名女性和2名男性,巧合的是,他们几乎是青少年,分别为5,11,17,19,21,22岁。
    结论:本研究报告并分析了大疱性PM的皮肤镜检查和临床特征,皮肤镜检查可以作为一种快速可靠的大疱性PM诊断技术。
    BACKGROUND: Pilomatricoma (PM) is a cutaneous benign neoplasm derived from the hair matrix. It clinically presents as a solitary and firm nodule overlying normal epidermis and is usually not easy to be noticed at early stage. Nevertheless, when special bullous lesion occurs in a short time or even ulcerates, preoperative diagnosis by a dermatologist is often challenging especially when the pediatric patients refuse biopsy.
    METHODS: We present six bullous PM cases and particularly conduct correlation analysis on the dermotoscopy and histopathology detection data. The basic information, medical history, symptoms and lesion morphology results of the patients were also provided. We found that the incidence of bullous PM was higher in females than in males, and most patients were adolescents and the predilection location seem to be consistent in the vaccine injection site. The dermatoscopic features of bullous PM reported were luminous yellow structure below, with gray-blue homogeneous areas and branched capillary. The histological features were consistent with PM, and evident epidermis bullae were above the tumor with extraordinary dilation of lymphangion in the upper dermis. The patients described in this study were Chinese patients in Han population included 4 females and 2 males, coincidentally, they are almost teen-age, respectively are 5,11,17,19,21,22 year-old.
    CONCLUSIONS: This study reported and analyzed the dermotoscopy and clinical characteristics of bullous PM, dermotoscopy may guide as a rapid and reliable technique in bullous PM diagnosis.
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  • 文章类型: Case Reports
    一名55岁的男性患者在左腹股沟区出现肿块,并伴有左下肢肿胀,3个月前因疼痛未缓解而首次去当地医院就诊。MRI扫描提示左侧耻骨上支和左侧髋臼骨破坏,左髂骨前缘髂腰肌的软组织信号异常,左髂窝和左腹股沟区淋巴结肿大。患者随后接受了左盆腔病变开放活检和腹股沟淋巴结切除活检。根据病理报告,左侧腹股沟肿块被认为是皮肤附件起源的恶性肿瘤(毛发癌),伴有广泛的玻璃体改变。耻骨上支肿块被认为是骨转移的毛发肿瘤癌。免疫组织化学(IHC)显示PDL1组合阳性评分(CPS)为8。DNA下一代测序(NGS)显示CDKN2AL65Rfs*53突变。患者接受了三个周期的吉西他滨和奈达铂。然而,病变进展。
    化疗对治疗毛囊癌无效。PDL1抗体和CDK4/6抑制剂可能是治疗毛发上皮癌的选择。
    UNASSIGNED: A 55-year-old male patient developed a mass in the left inguinal area with left lower limb swelling and first visited a local hospital 3 months earlier because of unrelieved pain. An MRI scan suggested left suprapubic branch and left acetabular bone destruction, abnormal soft tissue signals within the iliopsoas muscle of the anterior edge of the left iliac bone, and enlarged lymph nodes in the left iliac fossa and left inguinal region. The patient subsequently underwent left pelvic lesion open biopsy and inguinal lymph node resection biopsy. According to pathological reports, the left inguinal mass was considered to be a malignant tumor of cutaneous accessory origin (pilomatrix carcinoma) with extensive vitreous changes. The suprapupubis branch mass was considered to be a bone metastatic pilomatrix carcinoma. Immunohistochemistry (IHC) revealed a PDL1 combined positive score (CPS) of 8. DNA next-generation sequencing (NGS) showed CDKN2A L65Rfs*53 mutation. The patient received three cycles of gemcitabine and nedaplatin. However, the lesion progressed.
    UNASSIGNED: Chemotherapy is not effective for treating pilomatrix carcinoma. PDL1 antibodies and CDK4/6 inhibitors might be treatment options for pilomatrix carcinoma.
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  • 文章类型: Case Reports
    罕见的研究报道了双胞胎中的绒毛瘤,极为罕见的病例显示出身体位置相同部位的病变。我们报道了在右上肢皮肤同一位置的单卵双胞胎伴毛囊瘤的病例。
    7岁的同卵双胞胎被送到我们部门,无痛,和右上肢皮下组织中的实性肿块。在双胞胎右上肢的前外侧发现了一个直径1.5厘米的结节。节点形状不规则,在触诊时,患者报告没有明显的压痛。局部麻醉后,这对双胞胎接受了手术切除固体块。最后,根据临床和组织病理学特征,他们被诊断为毛囊瘤。进行完整的手术切除,然后进行初次闭合。在三年的随访期间,这对双胞胎没有复发。
    我们报道了一例在右上臂皮肤上相同位置的单卵双生子患有毛囊瘤。我们的发现强调了在罕见疾病的诊断和治疗中考虑遗传因素的要求。
    UNASSIGNED: Rare studies have reported pilomatricoma in twins, and extremely rare cases showed lesions in the same part of the body position. We reported a case of monozygotic twins with pilomatricoma in the same location on the skin of the right upper extremity.
    UNASSIGNED: Seven-year-old monozygotic twins presented to our department with a palpable, painless, and solid mass in the subcutaneous tissue of the right upper limb. A 1.5-cm diameter nodule was seen on the anterolateral aspect of the right upper extremity of the twins. The node was irregular in shape, and upon palpation, the patients reported no noticeable tenderness. Following the administration of local anesthesia, the twins underwent surgical procedure to excise the solid mass. Finally, they were diagnosed with pilomatricoma based on the clinical and histopathological features. Complete surgical resection followed by primary closure was performed. During a follow-up period of three years, there has been no recurrence observed in the twins.
    UNASSIGNED: We reported a case of monozygotic twins with pilomatricoma in the same location on the skin of the right upper arm. Our findings underscore the requirement of considering genetic factors in the diagnosis and treatment of the rare conditions.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    经常在小型哺乳动物宠物中观察到皮肤肿瘤疾病,比如狗,不管他们的性别。
    这项工作的一个重要目标是全面介绍临床,病态,和几种狗皮肤肿瘤的免疫组织化学特征。
    这项研究是医院诊所的一个案例系列,兽医学院,扎加齐格大学,埃及。在2022年3月至2023年10月期间,对25只狗(14只雄性和11只雌性)进行了临床检查。从受影响的动物收集皮肤肿胀,然后进行详细的组织病理学研究,以记录不同的总体和微观发现,并通过免疫组织化学确认诊断。
    狗的皮肤瘤形成暴露于各种临床症状,狗的年龄在3到11岁之间。关于肿瘤特征,大多数肿瘤的恶性(65.52%)高于良性(34.48%)。研究发现29例犬存在瘤形成,患病率不同,包括鳞状细胞癌(13.79%),肥大细胞瘤(6.89%),基底细胞肿瘤(10.34%),组织细胞瘤(6.89%),毛发上皮瘤(10.34%),传染性性病瘤(10.34%),三毛鞘瘤(3.44%),头皮副神经节瘤(3.44%),毛囊瘤(10.34%),恶性黑色素瘤(17.24%),和其他病例为脂肪坏死(6.89%),在具有不同组织病理学病变和全细胞角蛋白(CK)的免疫组织化学表达的雄性和雌性犬中,黑素细胞分化抗原(S100蛋白),和突触素。
    恶性黑素瘤(17.24%)是本研究中最常见的皮肤肿瘤。同时,良性肿瘤如毛癣瘤,毛发上皮瘤,pilomatricoma,副神经节瘤在狗中的发病率较低。
    UNASSIGNED: Cutaneous neoplastic disorders are often observed in small mammal pets, such as dogs, regardless of their gender.
    UNASSIGNED: An important objective of this work was to give a full account of the clinical, pathological, and immune-histochemical features of several skin tumors in dogs.
    UNASSIGNED: This study was a case series in the hospital clinic, Faculty of Veterinary Medicine, Zagazig University, Egypt. Twenty-five dogs (14 males and 11 females) were examined clinically during the period from March 2022 to October 2023. The skin swelling was collected from affected animals and then subjected to a detailed histopathological study to record the different gross and microscopic findings and confirm the diagnosis by immunohistochemistry.
    UNASSIGNED: Skin neoplasia in dogs was exposed to various clinical signs, and the dogs\' ages ranged between 3 and 11 years. Concerning tumor features, the majority of neoplasms were malignant (65.52%) more than benign (34.48%). The study revealed the presence of 29 cases of dogs showed neoplasia with different prevalence rates including squamous cell carcinoma (13.79%), mast cell tumor (6.89%), basal cell tumors (10.34%), histiocytoma (6.89%), trichoepithelioma (10.34%), transmissible venereal tumor (10.34%), trichilemmoma (3.44%), scalp paraganglioma (3.44%), pilomatricoma (10.34%), malignant melanomas (17.24%), and miscellaneous cases as fat necrosis (6.89%), in males and females dogs with different histopathological lesions and immunohistochemistry expressions for pan-cytokeratin (CK), melanocyte-differentiation antigens (S100 protein), and synaptophysin.
    UNASSIGNED: Malignant melanomas (17.24%) are the extremely common cutaneous tumors diagnosed in this study. Meanwhile, benign tumors such as trichilemmoma, trichoepithelioma, pilomatricoma, and paraganglioma are less frequent in dogs.
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  • 文章类型: Case Reports
    Pilomatrixoma,也被称为马尔赫贝上皮瘤,是来源于毛囊基质细胞的良性肿瘤。它通常表现为头部和颈部区域的孤立肿块,在儿童和年轻人中更常见,女性,和高加索人口。等于或大于5厘米的病变被归类为巨大的毛心房瘤。我们介绍了一个75岁女性的案例,没有已知的病史,在街上摔倒后被带到急诊室(ED)。她有一个巨大的软组织肿瘤,严重贫血,由于病灶内慢性小出血和叶酸和钴胺缺乏,和华丽的演讲。肿瘤活检的解剖学病理学结果显示为毛囊结肠瘤。然后病人接受了整形手术,完全切除肿瘤.手术后,她被调到精神科,他认为演讲是在精神分裂症的背景下进行的。她在入院四个月后出院。
    Pilomatrixoma, also called epithelioma of Malherbe, is a benign neoplasm derived from hair follicle matrix cells. It usually presents as a solitary mass in the head and neck region and is more frequent in children and young adults, females, and the Caucasian population. Lesions equal to or greater than 5 cm are categorized as giant pilomatrixomas. We present a case of a 75-year-old female, with no known medical history, who was brought to the emergency department (ED) after falling on the street. She had a giant soft head tissue tumor, severe anemia due to intralesional chronic small hemorrhages and folates and cobalamin deficiencies, and delirant speech. The anatomopathological result of the biopsy of the tumor revealed to be a pilomatrixoma. The patient was then referred to plastic surgery, with complete excision of the tumor. After surgery, she was transferred to the psychiatric team, who assumed the delirant speech to be in the context of schizophrenia. She was discharged four months after admission.
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  • 文章类型: Journal Article
    背景:大疱性绒毛瘤是一种罕见的绒毛瘤变种。正如它已经在零星病例报告中公布的那样,对其临床病理特征的有限了解限制了其有效的诊断和治疗。
    目的:本研究旨在分析大疱性毛囊瘤的临床病理和免疫组织化学特征,以更好地了解毛囊瘤的大疱性转化。
    方法:作者对12例大疱性绒毛瘤患者进行了回顾性研究,并比较了他们的临床,组织病理学,以及普通绒毛瘤患者的免疫组织化学数据。
    结果:大疱性绒毛瘤没有性别偏好,平均发病年龄为31.2岁。常见部位为上肢和躯干。大疱性毛囊瘤的病程较短,更大的直径,与普通的毛囊瘤相比,尺寸增加的趋势更大。组织病理学,大疱性毛囊瘤的持续时间较短,钙化较少,更多的有丝分裂图,与普通的毛囊瘤有明显的真皮特征。免疫组织化学,基质金属蛋白酶(MMP)-2、MMP-9、血管内皮生长因子受体-3(VEGFR-3)的表达,VEGF-C升高。
    结论:这项研究是回顾性的,样本量很小。
    结论:大疱性毛囊瘤的独特特征可能是由与血管生成因子和蛋白水解酶释放相关的真皮变化引起的。这种综合分析为大疱性毛囊瘤的临床特征和发病机理提供了新的见解。
    BACKGROUND: Bullous pilomatricoma is a rare variant of pilomatricoma. As it has been published in sporadic case reports, a limited understanding of its clinicopathological characteristics restricts its effective diagnosis and treatment.
    OBJECTIVE: This study aimed to analyze the clinicopathological and immunohistochemical characteristics of bullous pilomatricoma to better understand the bullous transformation of pilomatricoma.
    METHODS: The authors conducted a retrospective study of 12 patients with bullous pilomatricoma and compared their clinical, histopathological, and immunohistochemical data with those of patients with ordinary pilomatricoma.
    RESULTS: Bullous pilomatricoma showed no sex preference, with a mean onset age of 31.2 years. The common sites were the upper extremities and trunk. Bullous pilomatricoma had a shorter disease duration, a larger diameter, and a greater tendency to increase in size than those of ordinary pilomatricoma. Histopathologically, bullous pilomatricoma had a shorter duration, lesser calcification, more mitotic figures, and distinct dermal features from those of ordinary pilomatricoma. Immunohistochemically, the expression of Matrix Metalloprotease (MMP)-2, MMP-9, vascular endothelial growth factor receptor-3 (VEGFR-3), and VEGF-C was elevated.
    CONCLUSIONS: The study was retrospective, and the sample size was small.
    CONCLUSIONS: The distinctive features of bullous pilomatricoma potentially result from dermal changes associated with the release of angiogenic factors and proteolytic enzymes. This comprehensive analysis provides novel insights into the clinical features and pathogenesis of bullous pilomatricoma.
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  • 文章类型: Case Reports
    毛囊瘤(PM)是由毛囊基质细胞引起的良性肿瘤。它们是最常切除的小儿皮肤肿块之一。典型的临床表现是生长缓慢,无痛,公司,儿童头部和颈部的浅表肿块。我们提出了一个罕见的PM介绍。一名学龄前女孩在该地区有轻微钝器外伤病史后,头皮肿块增大。两个月前,该儿童持续了地面跌倒,随后在头皮受伤部位出现了无痛的“颠簸”。“凸起”最初是“蚊子叮咬”的大小,并扩大到“乒乓球”的大小。鉴于创伤的快速发展和历史,有一个广泛的鉴别诊断。包括超声检查在内的广泛检查,计算机断层扫描,MRI,进行活检。最终病理诊断为毛囊瘤。儿童PM的非典型表现可以模仿多种病理。我们的病例显示出快速扩大和先前创伤的异常特征。随着超声检查在浅表病变评估中的广泛应用,放射科医生可能是第一个怀疑PM的诊断。了解PM中的典型成像发现可能是有价值的,尤其是在更不寻常的情况下。
    Pilomatricomas (PM) are benign neoplasms that arise from hair follicle matrix cells. They are one of the most frequently excised pediatric skin masses. A typical clinical presentation is a slow growing, painless, firm, superficial mass in the head and neck of a child. We present a rare presentation of PM. A preschool aged girl presented with an enlarging scalp mass following a history of minor blunt trauma to the area. Two months prior, the child sustained a ground level fall and subsequently developed a painless \"bump\" at the site of injury on the scalp. The ``bump\" was initially the size of a \"mosquito bite\" and enlarged to the size of a \"ping pong ball\". Given the rapid progression and history of trauma, there was a broad differential diagnosis. An extensive workup including sonography, computed tomography, MRI, and biopsy were performed. The final pathologic diagnosis was confirmed as pilomatricoma. An atypical presentation of PM in a child can mimic a wide variety of pathology. Our case demonstrates unusual features of rapid enlargement and preceding trauma. With the widespread use of sonography for evaluation of superficial lesions, the radiologist may be the first to suspect the diagnosis of PM. Knowledge of the typical imaging findings in PM can be valuable, especially in more unusual cases.
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  • 文章类型: Journal Article
    背景:Pilomatricoma在彩色多普勒超声上有多种表现,鉴别诊断具有挑战性。这项研究的目的是探讨彩色多普勒超声检查的皮肤病变特征对区分表皮样囊肿和隆突性皮肤纤维肉瘤有效。
    方法:毛囊瘤患者的记录(n=63),表皮样囊肿(n=76),和接受彩色多普勒超声评估和手术切除的隆突性皮肤纤维肉瘤(n=19)进行了回顾。分析这些病变的解剖分布及彩色多普勒超声特征。根据其彩色多普勒超声特征将63例毛囊瘤分为五种类型,研究了这5种类型在上述疾病鉴别诊断中的作用。
    结果:Pilomatricoma,表皮样囊肿,隆突性皮肤纤维肉瘤表现出一些相似的特征。明显的高回声或高回声外观,后声阴影,血管的存在是毛囊瘤的主要特征。毛囊瘤可以分为五种类型,II型诊断灵敏度为65.08%,特异性98.95%,受试者工作特征曲线下面积(AUC)为0.743,阳性预测值为97.62%,诊断上述皮肤病的阴性预测值为81.03%。
    结论:明显的高回声或高回声外观的组合,后声阴影,血管的存在对毛囊瘤的鉴别诊断表现出更高的诊断性能,表皮样囊肿,和隆突性皮肤纤维肉瘤.
    BACKGROUND: Pilomatricoma has various manifestations on color Doppler ultrasound, and a differential diagnosis is challenging. The objective of this study was to investigate which characteristics of skin lesions on color Doppler ultrasound are effective in distinguishing pilomatricoma from epidermoid cyst and dermatofibrosarcoma protuberans.
    METHODS: Records of patients with pilomatricomas (n = 63), epidermoid cysts (n = 76), and dermatofibrosarcoma protuberans (n = 19) who underwent color Doppler ultrasound evaluation and surgical excision were reviewed. The anatomical distribution and color Doppler ultrasound characteristics of these lesions were analyzed. The 63 pilomatricomas were categorized into five types based on their color Doppler ultrasound characteristics, and the roles of these five types in the differential diagnosis of the aforementioned diseases were studied.
    RESULTS: Pilomatricomas, epidermoid cysts, and dermatofibrosarcoma protuberans exhibited some similar characteristics. Dominantly markedly hyperechoic or hyperechoic appearance, posterior acoustic shadowing, and the presence of vascularity were the major characteristics of pilomatricomas. The pilomatricomas could be categorized into five types, with type II having a diagnostic performance of sensitivity of 65.08%, specificity of 98.95%, area under the receiver operating characteristic curve (AUC) of 0.743, positive predictive value of 97.62%, and negative predictive value of 81.03% for the diagnosis of the aforementioned skin diseases.
    CONCLUSIONS: A combination of dominantly markedly hyperechoic or hyperechoic appearance, posterior acoustic shadowing, and the presence of vascularity exhibits higher diagnostic performance for the differential diagnosis of pilomatricomas, epidermoid cysts, and dermatofibrosarcoma protuberans.
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