毛母质瘤的临床、超声、皮肤镜特征

目的 探讨毛母质瘤的临床表现、超声特征、皮肤镜特征及组织病理特征，以提高该病术前正确诊断率，减少误诊及漏诊。方法 对2018年1月至2022年3月在复旦大学附属中山医院厦门医院经外科手术切除且术后病理诊断为毛母质瘤的75例患者的病例资料进行回顾性分析。结果 75例患者（39例女性和36例男性）中发现了77个毛母质瘤，患者年龄4～79岁，平均年龄（32.2±17.2）岁。最常见的临床表现为皮下硬结节；最好发的部位为头面部和颈部（62.3%）。73例（97.3%）患者表现为单发皮疹，仅2例患者为多发（2处）。术前临床诊断正确率为14.3%(11/77)。将34个病灶进行软组织超声检查，诊断准确率32.4%(11/34)。超声发现病灶多位于皮下脂肪层（33例，97.1%），呈椭圆形或扁圆形、边界清晰、不均质的低回声团块，可伴点状、斑片状或带状钙化或不伴钙化，大部分不存在低回声晕或后方声影，周边或内部可有彩色血流信号。将9个病灶进行皮肤镜检查，发现血管结构7例（77.8%），包括红色均质结构5例（55.6%）、不规则线状血管结构1例（11.1%）、点状血管结构2例（22.2%），白色均质结构2...     

Pigmented pilomatricoma: an underrecognized variant

The presence of melanin pigment and/or melanocytes in pilomatricoma has been rarely documented. In this study, we analyzed the incidence and clinicopathological features of pigmented pilomatricoma. Fifty-seven consecutive pilomatricoma cases from 53 Japanese patients were examined in this study. In fourteen cases (24.6%), pigmentation was observed in pilomatricoma. This variant equally affected in males and females, and the common locations were the upper arm and face. Proliferation of dendritic melanocytes was observed within basaloid cell nests in all cases, and melanin pigment was also present within the cytoplasm of the basaloid cells in 11 cases. Melanin pigment was also present in the shadow cells in 7 cases. The incidence of pigmented pilomatricoma as documented in previous reports is approximately 10%. However, our analysis revealed that pigmented pilomatricoma was found in 24.6% of Japanese cases of pilomatricoma, thus, this variant is not uncommon and may be under-recognized.     

Pilomatricoma: An Unusual Dermatologic Neoplasm

Pilomatricoma is typically an isolated benign tumor of the hair follicle matrix with very low incidence, recurrence, and initial diagnostic accuracy. This report presents a case of a pilomatricoma of the left chest that was initially ignored due to the limited extent of access to medical care in Palau, and subsequent treatment for cervical cancer. The paper helps to emphasize the importance of a vast differential diagnosis, especially in those patients from the Pacific Islands.     

Pilomatricoma: Experience of The Hospital for Sick Children

BACKGROUND:

Pilomatricoma (calcifying epithelioma of Malherbe) is a common skin neoplasm in the pediatric population that is often misdiagnosed as other skin conditions or tumours.

OBJECTIVES:

The objective of the present retrospective study was to review the clinical and histopathological presentation of this neoplasm in children.

METHODS:

The records of the pathology department at The Hospital for Sick Children, Toronto, Ontario, were searched for all cases of pilomatricoma between 2001 and 2006. The records of these patients were reviewed to determine sex, age, location and size of the tumour, pathological features and recurrence rate. All patients underwent surgical excision of the lesions.

RESULTS:

A total of 93 lesions in 85 patients were identified. The median age was 8.7 years. Of the 85 patients diagnosed with pilomatricoma, 44 (52%) were female. In all cases, the initial presentation was an asymptomatic, slow growing, superficial hard mass with bluish discolouration. The most common sites of occurrence were the face (48%), neck (21%) and upper limbs (18%). The size of the surgical specimens collected ranged from 0.1 cm to 2.6 cm. The diagnosis was confirmed by histopathological examination in all cases. Ghost cells and basaloid cells were described in most of the cases (83%). There were no recurrences in this series.

CONCLUSIONS:

This entity should be considered with other benign or malignant conditions in the clinical differential diagnosis of solitary firm skin nodules, especially those on the face, neck and upper limbs. The diagnosis can generally be made by clinical examination. The treatment of choice is surgical excision, and the recurrence rate is very low.     

32例头颈部毛母质瘤临床及病理资料研究

目的探讨毛母质瘤的临床和病理特点，为临床正确诊治提供参考。方法对1993年10月～2003年6月在解放军总医院诊治的32例头颈部毛母质瘤患者的临床及病理资料做统计分析；并利用回归方程等，阐述与易误诊疾病的差别。结果本资料显示毛母质瘤头面部与颈部例数比为25：7；腮腺咬肌区、颞部是其在头面部好发部位；它较易误诊为皮脂腺囊肿和表皮样囊肿；多发少见(3．1％)；有1例患病前此部位曾受外伤，1例嗜碱细胞核分裂象易见，未见复发及癌变。全部病理切片镜下均见影细胞和嗜碱性细胞；有完整包膜者占37，5％；随病程延长可见钙化；治疗方法是手术切除，服药无效。结论依照毛母质瘤的临床尤其是病理特点可对其进行鉴别、诊断；尽早切除，减少对颜面美观的影响。     

面颈部皮肤钙化上皮瘤临床及病理研究

目的：面颈部皮肤钙化上皮瘤为一类较少见的真性肿瘤,本文总结并讨论其临床及病理特点.方法：收集南京口腔医院颌面外科及病理科1991～2005年已经确诊并经手术治疗的钙化上皮瘤共9例临床病案进行回顾性分析.结果：本病患者以青少年俱多,其中～19岁组占42%,P＜0.05,性别趋向女性,男女之比为1∶2.本病临床上极易误诊,本文资料中9例其误诊率达66%.组织病理观察,表现为影细胞及嗜硷性细胞片状排列为特征.本病经外科手术切除后未见复发.结论：皮肤钙化上皮瘤好发于青少年,且女性占多数.钙化上皮瘤临床易发生误诊,利用影像学手段可提高诊断率.手术切除彻底可以预防复发.     

Pilomatricomas in children: imaging characteristics with pathologic correlation

Background Although pilomatricoma commonly occurs in children, there is still a poor understanding of the imaging characteristics of pilomatricoma and lack of agreement regarding its imaging findings and histopathologic features. Objectives To characterize the radiologic appearance of pilomatricomas on US, CT, and MR and to correlate the imaging findings with histopathologic features. Materials and methods The imaging findings of 47 pilomatricomas on US (n = 17), CT (n = 31), and MR (n = 5) were retrospectively evaluated. Pathologic specimens of all cases were reviewed and compared with imaging findings. Results All lesions were well-circumscribed, subcutaneous nodules with partial attachment to the overlying skin. On US, the lesions were mostly hyperechoic with posterior acoustic shadowing and hypoechoic rim. On CT, they appeared as enhancing soft-tissue masses with varying amounts of calcification. MR findings were internal reticulations and patchy areas on T2-weighted images and contrast-enhanced T1-weighted images, corresponding to edematous stroma on pathology. Peritumoral inflammatory changes and connective capsule on pathology were well correlated with imaging findings. Conclusion Pilomatricoma should be considered when US or CT shows a well-defined hyperechoic or calcific nodule in subcutaneous fat attached to the skin in children. MR images may be helpful in diagnosis. Pathologic findings are well correlated with imaging findings.     

毛母质瘤9例临床分析并文献复习

摘要：目的探讨毛母质瘤（pilomatricoma, PM）的临床特点、诊断与鉴别诊断及治疗并复习相关文献。方法选取2014年3月~2016年10月平煤神马医疗集团总医院耳鼻咽喉头颈外科收治的9例毛母质瘤,回顾性分析其临床表现,发病年龄、发病部位、组织病理学资料、手术及随访情况。结果9例患者平均发病年龄26.9岁;男女比例为1∶1.25;均为单发结节,平均直径为1.9 cm;光镜下可见嗜碱性细胞及影细胞。术后病理诊断与术前诊断有44.4%误诊率。术后随访半年以上,无一例复发,术后皮肤创面较为平整。结论毛母质瘤多发生在头颈部,影细胞是诊断毛母质瘤的特征性病理表现。首选治疗方法为手术切除,应用荷包缝合修复,圆形皮损瘢痕小,效果显著,值得推广。在临床工作中应提高对该病的认识,降低误诊率。     

Multiple pilomatricomas in twins with Rubinstein-Taybi syndrome

Pilomatricomas are benign tumors originating from the capillary matrix, which may present as solitary lesions or, less commonly, multiple. Myotonic dystrophy and familial adenomatous polyposis are the most frequently associated disorders with multiple pilomatricomas. There are few reports relating these tumors to other genetic syndromes. Rubinstein-Taybi syndrome is a rare autosomal dominant disorder characterized by intellectual disability and typical dysmorphic characteristics. There are five case reports relating to multiple pilomatricoma to Rubinstein-Taybi syndrome, an association that needs to be clarified. For this reason, we report the first case of multiple pilomatricoma in monozygotic twins with typical Rubinstein-Taybi syndrome.     

毛母质瘤MRI误诊的总结分析

＿目的：对比分析毛母质瘤的病理、超声表现及 MRI表现,总结 MRI误诊毛母质瘤的原因,提高 MRI对毛母质瘤的诊断符合率。方法：回顾性分析13例经病理证实的毛母质瘤的临床资料,分析其病理、超声与 MRI 表现对应关系。结果：MRI术前误诊的13例中,病灶均与皮肤关系密切,伴有钙化9例,不伴钙化4例,1例误诊为表皮样囊肿,3例误诊为皮脂腺囊肿,3例误诊为纤维腺瘤,6例考虑良性占位性病变。结论：MRI可清楚显示毛母质瘤生长部位及与皮肤关系,病灶与皮肤的关系及病灶内异常低信号灶对该病的诊断具有提示作用。