Spitz样肿瘤320例临床及组织病理分析

【摘要】 目的 总结Spitz样肿瘤的临床及组织病理特征。方法 回顾2005年1月至2020年1月西京皮肤医院确诊的320例Spitz样肿瘤患者的临床及病理资料。结果 320例患者中,男141例,女179例,年龄0 ~ 65（12.5 ± 11.7）岁,病程1个月至30年;其中,Spitz痣307例,不典型Spitz肿瘤（AST）8例,Spitz痣样黑素瘤（SM）5例。皮损多为单发,可见于头面部、躯干和四肢,边界均清楚。307例Spitz痣皮损以黑色（132例,43.0%）和红色（108例,35.1%）为主,多数色素均匀（262例,85.3%）且表面平滑（272例,88.6%）。Spitz痣存在特殊临床亚型,11例 （3.6%）发生在斑痣上,11例 （3.6%）呈簇发性,6例（2.0%）播散性,7例（2.3%）结节性,1例（0.3%）为瘢痕疙瘩样。Spitz痣特征性病理表现包括表皮内痣细胞呈Paget样扩散（123例,40.1%）,真表皮交界处出现Kamino小体（74例,24.1%）,痣细胞呈水平带状（177例,57.8%）及楔形分布（118例,38.4%）,痣细胞巢周围出现裂隙（177例,57.8%）,可见生理性核分裂象（117例,38.1%）,核染色质均细腻。根据特殊组织病理表现,Spitz痣又分为色素性上皮样Spitz痣（9例,2.9%）、结缔组织增生性Spitz痣（13例,4.2%）、血管瘤样Spitz痣（8例,2.6%）、疣状Spitz痣（12例,3.9%）、黏液样Spitz痣（10例,3.3%）、晕痣样Spitz痣（4例,1.3%） 等。4例AST皮损为黑色,7例色素均匀,3例皮损表面粗糙;特征病理表现包括细胞均有轻度至中度的异型性,均可见核分裂象（7例为2 ~ 6个/mm2）,5例核染色质粗糙。3例SM皮损呈红色,4例色素不均匀,3例表面粗糙;特征病理表现包括黑素细胞呈Paget样扩散（3例）,瘤细胞呈无极性浸润性生长且均未见成熟现象,均有明显异型性,并可见病理性核分裂象（3例, > 6个/mm2）,核染色质均粗糙且核膜明显着色。结论 Spitz样肿瘤的临床及组织病理表现具有特征性,Spitz痣的临床及病理亚型繁多,AST同时具有Spitz痣和黑素瘤的临床及组织学特征。     

Angiomatoid and desmoplastic Spitz nevus presenting as a keloidal nodule

Angiomatoid and desmoplastic Spitz nevi are rare histologic variants of Spitz nevi that present most frequently on the extremities of children and young adults. Although Spitz nevi are clinically heterogeneous, one presenting as a keloidal nodule has not been previously published. We present a case of an angiomatoid and desmoplastic Spitz nevus clinically akin to a keloid on an African‐American teenager and describe its unique histopathologic features.     

A case of multiple disseminated eruptive Spitz nevi

Spitz nevus is usually a solitary lesion. However, multiple Spitz nevi do arise and can be divided into two variants, agminated (grouped) or disseminated. We report an interesting case of multiple disseminated eruptive Spitz nevi with an unusual feature. The appearance of most of the nevi resembled that of melanocytic nevi, and clinically it was impossible to distinguish these multiple disseminated eruptive Spitz nevi from eruptive melanocytic one.     

Reed痣26例临床病理特征分析

【摘要】 目的 分析总结Reed痣的临床病理特点。方法 分析2015年1月至2019年6月于第四军医大学西京皮肤医院确诊的26例Reed痣患者的临床病理特征。结果 男15例,女11例,平均发病年龄17.35岁,中位发病年龄13.85岁。发病部位：下肢12例,面部6例,上肢5例,躯干3例。皮疹颜色均呈黑色,7例为斑疹,19例为扁平丘疹,22例皮疹形态类圆形,4例皮疹形态不规则。皮疹直径2 ~ 10 mm,23例直径 ≤ 5 mm。组织病理：15例为交界痣,11例为混合痣,26例黑素细胞形态均呈梭形,细胞色素明显,4例痣细胞巢与周围表皮融合,22例与周围表皮形成明显的裂隙,均未见明显细胞异型性及有丝分裂象,6例可见Kamino小体。皮损均予手术切除,随访1 ~ 5年无复发。结论 Reed痣皮疹形态可不规则,组织病理可出现呈Paget样分布的大小不一梭形细胞,需与黑素瘤鉴别。     

Distinctive eosinophilic cytoplasmic inclusion bodies in melanocytic nevi: an immunohistochemical and ultrastructural study

Background: We sought to further determine the histochemical, immunohistochemical and ultrastructural properties of eosinophilic cytoplasmic inclusion bodies in melanocytic nevi. Methods: Skin specimens from four patients with a known diagnosis of conventional melanocytic nevus (3) or Spitz nevus (1) and containing intracytoplasmic eosinophilic inclusion bodies were selected. In addition, melanomas (25), Spitz nevi (10) and blue nevi (4) were examined to determine the frequency of the inclusions. Results: Inclusions tended to be located in multinucleated melanocytes with abundant vacuolated cytoplasm. In conventional (hematoxylin and eosin‐stained) sections, the degree of density and eosinophilia of intracytoplasmic inclusions varied with size. Periodic acid‐Schiff, Fontana and Congo red stains showed no reactivity. All bodies were immunoreactive for ubiquitin but negative for tyrosinase, keratin and vimentin. Ultrastructurally, inclusion bodies were non‐membrane bound, ranged from 4 to 7 µm, and were comprised of radiating filamentous structures with or without an electron‐dense core. Electron probe x‐ray microanalysis revealed no significant peaks. None of additional melanomas, Spitz nevi and blue nevi that were evaluated showed similar inclusions. Conclusions: The inclusion bodies described herein bear no resemblance to other cytoplasmic inclusion bodies previously described in melanocytic lesions. There is no discernible relationship to melanosomes by ultrastructural analysis. We postulate a relationship with dysfunction of ubiquitin‐mediated protein degradation occurring in melanocytes. Shon W, Wada DA, Gibson LE, Flotte TJ, Scheithauer BW. Distinctive eosinophilic cytoplasmic inclusion bodies in melanocytic nevi: an immunohistochemical and ultrastructural study.     

Acral pigmented Spitz nevus in a child with transepidermal migration of melanocytes: Dermoscopic and reflectance confocal microscopic features

Acral pigmented Spitz nevi are seldom reported in the literature. We report a new case on the palm of a 4‐year‐old girl that demonstrated correlation between features observed on dermoscopy and reflectance confocal microscopy (RCM). Histopathology revealed a benign intraepidermal Spitz nevus with transepidermal elimination of melanocytes that showed on RCM as focal atypical bright cells concerning for malignancy. This case is one of few reports in the literature combining dermoscopy, reflectance confocal microscopy, and histology for an acral Spitz nevi, which are rarely evaluated by RCM given the thickness of the stratum corneum in acral sites.     

Linear arrangement of hypopigmented Spitz nevi on the ear after trauma

A 10‐year‐old boy had multiple papules after surgery on a primary lesion on his left ear. The papules were pink and showed a linear arrangement around the helix. Histopathological examination of two separate lesions showed proliferation of epithelioid cells in the dermis and some of them formed nests. No melanin was found in the epithelioid cells, and immunohistochemistry staining with S100, HMB45 and Melan‐A were positive in the neoplasm cells. According to the clinical and histopathological characteristics, it was diagnosed as hypopigmented Spitz nevi with linear arrangement after trauma.     

Acral Pigmented Spitz Nevus That Clinically Mimicked Acral Lentiginous Malignant Melanoma

Pigmented Spitz nevus is a benign melanocytic skin lesion with distinct clinical features and it is frequently found on the extremities. However, it rarely occurs on acral area of the body, and such a case has not yet been fully documented. We present a case of acral pigmented Spitz nevus occurring on the foot, and this mimicked acral lentiginous malignant melanoma. Clinicians should be well aware of this entity and its possible clinical presentations. Since acral pigmented Spitz nevus is benign in nature, making the correct diagnosis is important to avoid unnecessary mutilating or excessive surgery.     

Genomic analysis of a case of agminated Spitz nevi and congenital‐pattern nevi arising in extensive nevus spilus

Nevus spilus is a melanocytic neoplasm characterized by a tan macular background punctuated by multiple hyperpigmented macules or papules that represent various types of nevi. These include junctional and compound nevi, Spitz nevi, and rarely blue nevi. We report a unique case of widespread, multiple nevi spili giving rise to agminated Spitz nevi and congenital‐pattern compound nevi. We performed genetic analysis to further characterize the mutational profile of this rare entity.     

多发性先天性色素痣1例

报告先天性多发性色素痣1例,患者男,19岁,全身散在黑痔19年,皮肤科检查见头面部,躯干部,四肢密集黑色结节,大片状黑斑,皮肤增厚,质软,上有黑色毳毛生长,前后共3次手术,术后效果满意,皮损组织病理学检查显示为皮内痣。     

Nevo de Spitz y otros tumores spitzoides en la infancia. Parte 1: aspectos clínicos,histológicos e inmunohistoquímicos

A Spitz nevus is a melanocytic neoplasm of epithelioid and/or spindle cells that usually appears in childhood. These lesions are by nature benign, but their features can sometimes make them difficult to distinguish from melanomas. Spitzoid melanocytic lesions have been grouped into 3 types in recent decades: Spitz nevi, atypical Spitz tumors, and spitzoid melanomas. Atypical Spitz tumors are spitzoid melanocytic proliferations that have atypical histopathologic features that are insufficient to support a diagnosis of melanoma. The malignant potential of these lesions is at present uncertain. This review examines the clinical, dermoscopic, and histopathologic features of this group of lesions.     

Spitz nevus is relatively frequent in adults: a clinico-pathologic study of 247 cases related to patient's age

Spitz nevus is a clinico-pathologic entity that can cause diagnostic concern, particularly in adults. Many studies have been performed to establish reliable histologic criteria, in the attempt to differentiate this lesion from melanoma. A series of 247 Spitz nevi, 6 of which were formerly classified as melanomas, were reviewed for clinical and histopathological parameters. Patients older than 20 comprised 66% of cases, with a predominance of women. The lower extremity was more affected in females of any age, whereas the trunk was more frequently involved in men over 40. Histopathologic examination showed the following differences among Spitz nevi related to age: acanthosis, parakeratosis, pagetoid infiltration, and Kamino bodies were more frequent in young people, whereas multinucleated melanocytes were more frequent in adults. The latter also had lesions that were less pigmented, with less maturation and more desmoplasia. At a mean follow-up of 94 months (range 52-172), recurrence at the site of biopsy or metastases were absent. In our study, a greater proportion of Spitz nevi occurred in adults than in previous series. Moreover, the relative incidence of Spitz nevus compared with melanoma in our population was higher than in other studies. Histopathologic criteria elaborated to diagnose Spitz nevus, applied to our cases, appeared reliable, allowing a correct diagnosis, even in adults.     

Meyerson痣6例临床及病理分析

目的:探讨Meyerson痣临床及组织病理特征。方法:回顾性分析2015年1月至2020年1月第四军医大学西京皮肤医院确诊的6例Meyerson痣患者临床及病理资料。结果:6例患者中,男3例,女3例,年龄7个月至28岁,中位年龄10.5岁。3例皮损位于四肢,3例位于躯干。4例发生于先天性色素痣,2例发生于获得性色素痣。...     

Multiple skin metastases of malignant melanoma with unusual clinical and histopathologic features in an immunosuppressed patient

Immunosuppressive regimens may have significant impact on the number of pigmented lesions and the clinical appearance of nevi. Whether immunosuppression can also influence the clinical and histopathologic appearance of malignant melanocytic lesions is still a matter of debate. A patient was immunosuppressed because of heart and bone marrow transplantation. A clinically inconspicuous mole was removed from the left flank and was considered to be a papillomatous nevus. After 1 year, the patient developed multiple pigmented lesions over the entire body, which presented clinically as benign papillomatous nevi and histologically as atypical Spitz nevi. Three months later melanoma metastases were removed from the patient's left axilla, which finally resulted in the death of the patient. Thus, in retrospect, the eruptive pigmented lesions have to be considered as cutaneous melanoma metastases. The atypical clinical and histopathologic appearance of the melanocytic lesions as well as the course of disease may have been influenced by the immunosuppression.     

Differentiation of melanoma from benign mimics using the relative‐color method

Background: Previous studies have successfully classified 86% of malignant melanomas using a relative‐color segmentation method, by feature extraction from photographic images in the automatic identification of skin tumors. These studies were extended by applying the relative‐color method to dermoscopic images of melanoma grouped with melanoma in situ and clark nevus lesions in dermoscopic images allow more control over lighting variations, which contribute to lesion misclassification. Dermoscopic images then enable a more detailed examination of the structure of skin lesions, provide much more structural detail within lesions, and contain visual information that cannot be seen in photographic images. This present work extends the previous studies by applying relative‐color feature extraction to dermoscopic images to differentiate among melanoma, seborrheic keratoses and Reed/Spitz nevi. Objective: To develop a method for automatically differentiating among malignant melanoma, seborrheic keratoses and Reed/Spitz nevi, using digitized, color, dermoscopic images. Methods: Images underwent preprocessing, tumor segmentation, feature extraction and tumor classification. The relative‐color method was used in the segmentation stage. Classification was accomplished by taking the inner products of model tumor feature vectors with test‐image tumor vectors followed by the nearest‐neighbor classification method. Results: The classification rates of melanoma, seborrheic keratoses and Reed/Spitz nevi images mixed together, were 60%, 58.3% and 80%, respectively. Classification of melanoma and Reed/Spitz nevi mixed, were 70% and 90%, respectively. Classification rates were the best when melanoma was being differentiated from seborrheic keratoses. These rates were 100% and 88.9%, respectively. Conclusion: Dermoscopic rather than photographic images were preprocessed, using a hair‐removal technique. They were then converted to relative‐color images, which were segmented using the principal components transform and median split, followed by morphological filtering. After processing, the multi‐dimensional tumor feature space described herein was used to differentiate the tumors. The high success rates for differentiating seborrheic keratoses from melanoma show that the use of dermoscopic images has a strong promise in enabling prescreening, as well as automated assistance and significant improvement in tumor diagnosis in clinics.     

Melanocytic nevi with features of Spitz nevi and Clark's/dysplastic nevi ("Spark's" nevi)

Background: Nevi with cytologic characteristics of Spitz nevus and architectural features of Clark's/dysplastic nevus are not well recognized in the literature.
Methods: Twenty-seven nevi with characteristics of Spitz nevus and Clark's/dysplastic nevus are reviewed.
Results: The patients' mean age was 33 years, and 17/27(63%) patients were female. Lesions were most frequent on the trunk and lower extremities. Histopathologically, these nevi were composed of large, monomorphous spindled and/or epithelioid melanocytes. Spindle cells were often oriented parallel to the epidermis, with fused rete and lamellar fibroplasias. Lateral extension of the junctional component was a feature of compound lesions. An average of 10 years of clinical follow up in 12 patients revealed no recurrence or metastasis.
Conclusions: Recognition of this type of nevus is important to avoid confusion with malignant melanoma.     

Linear arrangement of multiple deep penetrating nevi: report of first case and review of literature

BACKGROUND: Deep penetrating nevus is a recently described variant of melanocytic nevi with clinical and histopathological features that may be confused with malignant melanoma, blue nevus, pigmented Spitz nevus, or congenital melanocytic nevus. We report a case with linear arrangement of multiple deep penetrating nevi. To our knowledge, such presentation has never been reported in the literature. OBSERVATIONS: We describe a patient with multiple darkly pigmented lesions in the right periauricular area, above and behind the ear. The histopathological features of these lesions were consistent with deep penetrating nevus. CONCLUSIONS: To our knowledge, this is the first report of linear arrangement of multiple deep penetrating nevi. We consider this case a unique presentation of deep penetrating nevus.     

Disseminated Eruptive Nevocellular Nevi

A 13-year-old boy suddenly developed about 2,000 dark brown to black colored papules on his face and neck and about 500 lesions on his trunk and upper extremities during a six month period. Histopathologic features were compatible with junctional nevus. The results of alpha melanocyte stimulating hormone (MSH), proliferating cell nuclear antigen (PCNA), and epidermal growth factor receptor (EGF/R) studies are presented. To the best of our knowledge, this report represents an outbreak of the highest number of nevocellular nevi in a short period without any malignant nature or evident triggering factor.     

The morphologic spectrum of germline‐mutated BAP1‐inactivated melanocytic tumors includes lesions with conventional nevic melanocytes: A case report and review of literature

Germline mutations in BRCA1‐associated protein 1 (BAP1) are associated with several neoplasms, including BAP1‐inactivated melanocytic tumors (BIMTs). BIMTs are classically described as biphenotypic melanocytic proliferations with BAP1‐deficient large epithelioid and rhabdoid melanocytes showing various degrees of cytologic atypia. This morphology has been traditionally classified as “spitzoid” despite the various differences between these lesions and the more classic Spitz nevi. Herein, we report a case of an otherwise healthy 11‐year‐old female patient with a family history of several malignancies who presented with multiple pink to brown papules. Histologic and immunohistochemical evaluation identified three lesions with loss of nuclear BAP1 staining. The histologic spectrum of these lesions included junctional spitzoid cells within a triphenotypic proliferation and a separate lesion composed entirely of dermal small to medium‐sized epithelioid melanocytes with maturation. BAP1 gene sequencing revealed a germline frameshift pathogenic BAP1 mutation, denoted c.1717delC. This case provides further evidence that not all BIMTs conform to classic morphological criteria and that the morphologic spectrum includes lesions resembling conventional nevi. As BIMTs can serve as an early marker of the BAP1 hereditary tumor predisposition syndrome, we believe a need exists for a more comprehensive combined clinical and pathological approach for BIMT identification.     

Morphologic changes of a pigmented Spitz nevus assessed by dermoscopy

Pigmented Spitz nevus may simulate cutaneous melanoma clinically and histopathologically. In an effort to characterize Spitz nevi using dermoscopy, we documented the dermoscopic features of a single pigmented Spitz nevus over a 6-month period. A 3-year-old boy had a brownish black papule, 3 mm in diameter, on the dorsum of the first finger of his left hand, clinically diagnosed as a Reed nevus. Two follow-up examinations were performed after 3 and 6 months, when the lesion finally was excised for histopathologic examination. Dermoscopically, a globular pattern was recognized during the initial examination, whereas a starburst pattern was identified 3 months later. After 6 months, a variation of the starburst pattern was still detectable. Based on our observation, the globular and the starburst patterns might be considered different morphologic expressions corresponding to the evolutionary phases of pigmented Spitz nevi.