Spitz Nevi and Other Spitzoid Neoplasms in Children: Overview of Incidence Data and Diagnostic Criteria

Abstract

Spitz nevi are benign melanocytic neoplasms characterized by epithelioid or spindle melanocytes or both. In some rare cases their presentation overlaps with the clinical and histopathologic features of malignant melanoma, so a differential diagnosis can be difficult to make. Intermediate forms between Spitz nevi and malignant melanoma, with unpredictable behavior, have been called atypical Spitz tumors. A literature search was performed to review the clinical, dermoscopic, genetic, and histopathologic aspects of spitzoid tumors. Spitz nevi mainly occur in children, with no predilection for sex, and in young women. Common sites are the head and lower arms, where Spitz nevi present as pink nodules or hyperpigmented plaques. Spitzoid lesions may have diverse dermoscopic patterns: vascular, starburst, globular, atypical, reticular, negative homogeneous, or targetoid. The management of spitzoid lesions can be invasive or conservative; surgical excision is usually reserved for those with doubtful features, whereas clinical and dermoscopic follow-up is preferred for typical pediatric Spitz nevi. The role of sentinel lymph node biopsy in atypical Spitz tumors is debated. Immunohistochemistry and new molecular techniques such as comparative genomic hybridization, polymerase chain reaction, and fluorescence in situ hybridization offer new diagnostic perspectives, investigating genetic alterations that are specific for malignant melanoma or for Spitz nevi.

Characteristics of Spitz nevi, Atypical Spitz nevi and Melanomas in patients younger than 20 years

“…Editor The spectrum of Spitzoid tumours and early onset melanomas has been extensively studied during the last few years. 1,2 Although significant progress has been made in the histopathological and clinical description of these lesions, the biologic potential of the most atypical lesions of the spectrum still remains to be elucidated.…”

“…It is estimated that the incidence of SN ranges from 1.4 to 7 new cases in 100 000 persons/year. 1 Paediatric melanomas are extremely rare and are believed to comprise 1 to 3% of all malignancies during childhood. 2 In our study, the prognosis for patients with SN and ASN was found to be excellent.…”

Evolution of Spitz Nevi

“…Atypical Spitz tumors do not appear dermoscopically as a typical spitzoid, but rather exhibit a multicomponent or unspecific pattern. Although spitzoid melanomas can also dermoscopically exhibit spitzoid features, the characteristic symmetry of Spitz nevi is not observed in spitzoid melanoma .…”

“…Forms intermediate between Spitz nevi and malignant melanoma have also been described and referred to as atypical Spitz tumors. Atypical Spitz tumors have been characterized as melanocytic proliferations with intermediate histopathologic features between Spitz nevi and spitzoid melanoma with unpredictable malignant potential . Local excision is the suggested treatment for atypical Spitz tumors given the lack of clarity about their clinical and histopathologic morphologic overlap with spitzoid melanoma.…”

Atypical Spitz Tumors are Biohistologically Categorizable as Melanocytic Tumors of Uncertain Malignant Potential

“…We read with great interest the article by Dika et al about Spitz nevi and other spitzoid neoplasms in children. The authors discuss the so‐called atypical Spitz tumor, also correctly referred to as spitzoid tumor of uncertain malignant potential, which has an asymmetric and poorly circumscribed shape, deeper extension, ulceration, absence of Kamino bodies, presence of mitoses (>6 mitoses/mm 2 ) and single melanocytes instead of typical nests . Atypical Spitz tumors may be difficult to distinguish from melanoma; for this reason, Spatz et al proposed a scoring system based on reproducible parameters (age, diameter, fat involvement, ulceration, mitotic activity/mm 2 ).…”