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    Parallel ridge dermoscopic pattern in plantar atypical Spitz nevus

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    Clinicopathological classification, diagnosis and management of spitzoid melanocytic lesion presents one of the most intriguing issues in dermatopathology (1,2). No single clinicopathological feature can offer reliable differentiation of Spitz nevus and melanoma. Although not all the experts agree with the concept of Spitzoid neoplasms as a morpho-biological spectrum, there is a four-tiered classification system proposed by Da Forno et al. and encompassing: 1) Spitz nevus; 2) atypical Spitz nevus; 3) (atypical) Spitz tumor; 4) Spitzoid melanoma (1,3). It is commonly said that Spitz nevus can show all the "local" dermoscopic features of melanoma, but in a more or less tidy fashion (4). The occurrence of melanoma-like dermoscopic pattern in Spitz nevus is also possible (4). The relationship between dermoscopic and histopathologic atypia is not absolute, in as much as dermoscopically atypical lesions are not necessarily histopathologically atypical as well. This article is protected by copyright. All rights reserved

    Atypical Spitz tumors in patients younger than 18 years

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    BACKGROUND: Diagnosis and proper management of atypical Spitz tumors in pediatric age are still controversial. OBJECTIVE: We sought to investigate the clinicopathological and molecular features of atypical Spitz tumors in patients aged 18 years or younger. METHODS: We performed a retrospective clinicopathological and fluorescence in situ hybridization study on 50 pediatric atypical Spitz tumors. RESULTS: Parameters that were significantly correlated with a diagnosis of atypical Spitz tumors over Spitz nevus included asymmetry, level IV/V, lack of maturation, solid growth, nuclear pleomorphism, high nuclear-cytoplasmic ratio, atypical and deep mitoses, and more than 6 mitoses/mm(2). In the atypical Spitz tumors group, a significantly higher mitotic rate was observed in prepuberal age (P = .04). The 4-probe fluorescence in situ hybridization melanoma assay did not discriminate atypical Spitz tumors from Spitz nevi. Heterozygous 9p21 loss was found in 3 of 37 cases and homozygous 9p21 loss in 2 of 37 cases. Only 1 child experienced a fatal outcome, showing genetic abnormalities by melanoma fluorescence in situ hybridization probe and a heterozygous 9p21 deletion. LIMITATIONS: The limited number of adverse outcomes did not allow the prognostic analysis of single morphologic features. CONCLUSION: Pediatric atypical Spitz tumors are associated with minimal lethal potential. Atypical Spitz tumors require complete excision and careful follow-up while our data do not support any clinical benefit for the sentinel lymph node biopsy procedure and completion lymphadenectomy

    Spitz nevus and its variants

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    The eponymic designation Spitz nevus refers to a benign melanocytic proliferation, which was first described in 1948 by Sophie Spitz as melanoma of the childhood [1]. Along with this original description, we presently consider as classical Spitz nevus a rapidly growing, pink or flesh-colored papule or nodule of the lower extremities or the face in childhood or early adulthood [2-6]. Its histopathological hallmark is the presence of large spindle and/or epithelioid cells, usually in the paucity or absence of melanin. © 2007 Springer-Verlag

    Ntrk gene fusion detection in atypical spitz tumors

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    Atypical Spitz tumors (AST) deviate from stereotypical Spitz nevi for one or more atypical features and are now regarded as an intermediate category of melanocytic tumors with uncertain malignant potential. Activating NTRK1/NTRK3 fusions elicit oncogenic events in Spitz lesions and are targetable with kinase inhibitors. However, their prevalence among ASTs and the optimal approach for their detection is yet to be determined. A series of 180 ASTs were screened with pan-TRK immunohistochemistry and the presence of NTRK fusions was confirmed using FISH, two different RNA-based NGS panels for solid tumors, and a specific real time RT-PCR panel. Overall, 26 ASTs showed pan-TRK immunostaining. NTRK1 fusions were detected in 15 of these cases showing cytoplasmic immunoreaction, whereas NTRK3 was detected in one case showing nuclear immunoreaction. Molecular tests resulted all positive in only two ASTs (included the NTRK3 translocated), RNA-based NGS and real time RT-PCR were both positive in three cases, and FISH and real time RT-PCR in another two cases. In seven ASTs NTRK1 fusions were detected only by FISH and in two cases only by real time RT-PCR. The frequency of NTRK fusions in ASTs is 9%, with a clear prevalence of NTRK1 compared to NTRK3 alterations. Pan-TRK immunohistochemistry is an excellent screening test. Confirmation of NTRK fusions may require the use of different molecular techniques

    Atypical Spitz tumors in patients younger than 18 years

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    Background Diagnosis and proper management of atypical Spitz tumors in pediatric age are still controversial. Objective We sought to investigate the clinicopathological and molecular features of atypical Spitz tumors in patients aged 18 years or younger.Methods We performed a retrospective clinicopathological and fluorescence in situ hybridization study on 50 pediatric atypical Spitz tumors.Results Parameters that were significantly correlated with a diagnosis of atypical Spitz tumors over Spitz nevus included asymmetry, level IV/V, lack of maturation, solid growth, nuclear pleomorphism, high nuclear-cytoplasmic ratio, atypical and deep mitoses, and more than 6 mitoses/mm2. In the atypical Spitz tumors group, a significantly higher mitotic rate was observed in prepuberal age (P =.04). The 4-probe fluorescence in situ hybridization melanoma assay did not discriminate atypical Spitz tumors from Spitz nevi. Heterozygous 9p21 loss was found in 3 of 37 cases and homozygous 9p21 loss in 2 of 37 cases. Only 1 child experienced a fatal outcome, showing genetic abnormalities by melanoma fluorescence in situ hybridization probe and a heterozygous 9p21 deletion.Limitations The limited number of adverse outcomes did not allow the prognostic analysis of single morphologic features. Conclusion Pediatric atypical Spitz tumors are associated with minimal lethal potential. Atypical Spitz tumors require complete excision and careful follow-up while our data do not support any clinical benefit for the sentinel lymph node biopsy procedure and completion lymphadenectom

    Clinical review of 247 case records of Spitz nevus (epithelioid cell and/or spindle cell nevus)

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    Background: Spitz nevus has clinically been described as a dome-shaped usually nonpigmented papular or nodular lesion variable in color from pink to red. Objectives: To give an exhaustive description of the clinical features of the Spitz nevus from a large series of 247 patients. Methods: A retrospective analysis of the clinical features of 247 Spitz nevi excised from 1974 to 1993 has been performed. We evaluated the following features: age, sex, anatomical location, clinical and histopathologic features; descriptive statistics were calculated and relationships among the above variables were assessed. Results: Most lesions were pigmented (71.7%), located on the lower extremities (43.3%), more frequent in the first decade (55.8%) and in females (57.9%), The nonpigmented type was more frequent in the head or neck region, whereas the pigmented types were more frequent on the lower extremities. Besides, these types showed different histopathologic features: the spindle cells usually predominated in the flat pigmented type, whereas dome-shaped types were usually composed of both spindle and epithelioid cells. Conclusions: In our patients, the pigmented Spitz nevi were more common than the nonpigmented ones; furthermore pigmented and nonpigmented Spitz nevi showed different anatomical locations and different histopathologic features

    Update on dermoscopy of Spitz/Reed naevi and management guidelines by the International Dermoscopy Society

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    Spitzoid lesions represent a challenging and controversial group of tumours, in terms of clinical recognition, biologic behavior and management strategies. Although Spitz naevi are considered benign tumours, their clinical and dermoscopic morphologic overlap with spitzoid melanoma renders the management of spitzoid lesions particularly difficult. The controversy deepens because of the existence of tumours that cannot be safely histopathologically diagnosed as naevi or melanomas (atypical Spitz tumours). The dual objective of the present study was to provide an updated classification on dermoscopy of Spitz naevi, and management recommendations of spitzoid looking lesions based on a consensus among experts in the field. After a detailed search of the literature for eligible studies, a data synthesis was performed from 15 studies on dermoscopy of Spitz naevi. Dermoscopically, Spitz naevi are typified by 3 main patterns: starburst pattern (50.6%), a pattern of regularly distributed dotted vessels (19.3%) and globular pattern with reticular depigmentation (17.0%). A consensus-based algorithm for the management of spitzoid lesions is proposed. According to it, dermoscopically asymmetric lesions with spitzoid features (both flat/raised and nodular) should be excised to rule out melanoma. Dermoscopically symmetric spitzoid nodules should also be excised or closely monitored, irrespectively of the age, to rule out atypical Spitz tumours. Dermoscopically symmetric flat spitzoid lesions should be managed according to the age of the patient. Finally, the histopathologic diagnosis of atypical Spitz tumour should warrant wide excision but not a sentinel lymph node biopsy. This article is protected by copyright. All rights reserved

    Agminated intradermal Spitz nevi arising on an unusual speckled lentiginous nevus with localized lentiginosis: a continuum?

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    We report an 18-year-old boy with a congenital pigmented lesion measuring 2 x 6 cm on his right thigh. About a third of the lesion was composed of numerous lentiginous macules superimposed on histologically normal and clinically nontan skin; in the remainder of the lesion, several macules and papules with histologic features of junctional and compound nevi were superimposed on clinically normal skin, which had a lentiginous pattern histologically. Some years later, eruptive intradermal Spitz nevi developed at one corner of the lesion. The combined clinical and histological features of the lesion fulfill descriptions for both segmental lentiginosis and an unusual variant of speckled lentiginous nevus. Our case points out the limitations of using strict diagnostic criteria to define speckled lentiginous nevus and offers an opportunity to consider the natural history of the lesion as a continuum from lentigines to melanocytic nevi. Moreover, the presence of eruptive intradermal Spitz nevi arising within the area of speckled lentiginous nevus lacking a distinct tan background, suggests the possibility that the entire area of the lesion per se constitutes an environment where development of nevi is enhanced

    Clinical and dermoscopic features of atypical Spitz tumors: A multicenter, retrospective, case-control study

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    Background Few studies have described the clinical and dermoscopic features of atypical Spitz tumors. Objective We sought to describe the clinical and dermoscopic features of a series of atypical Spitz tumors as compared with those of conventional Spitz nevi. Methods This was a multicenter, retrospective, case-control study, analyzing the clinical and dermoscopic characteristics of 55 atypical Spitz tumors and 110 Spitz nevi that were excised and diagnosed histopathologically. Results The majority of atypical Spitz tumors presented clinically as a plaque or nodule, dermoscopically typified by a multicomponent or nonspecific pattern. A proportion of lesions (16.4%) exhibited the typical nonpigmented Spitzoid pattern of dotted vessels and white lines under dermoscopy. Nodularity, ulceration, linear vessels, polymorphic vessels, white lines, and blue-white veil were associated with atypical Spitz tumors by univariate analysis, but only nodularity and white lines remained significant after multivariate analysis. In contrast, a pigmented typical Spitzoid pattern was a potent predictor of Spitz nevi, associated with 6.5-fold increased probability. Limitations Differentiation from Spitzoid melanoma and other nonmelanocytic lesions was not investigated. Conclusion Atypical Spitz tumors are polymorphic melanocytic proliferations with a nodular clinical appearance. Dermoscopically they demonstrate a multicomponent and nonspecific pattern. A typical nonpigmented Spitzoid pattern on dermoscopy (with dotted vessels and white lines) does not exclude atypical Spitz tumors
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