Dermatology: Practical and Conceptual Letter | Dermatol Pract Concept 2019;9(3):19 237 Dermatology Practical & Conceptual An 8-year-old boy presented with a well-demarcated pig- mented lesion localized 5 cm above his right knee. His parents reported that it had appeared approximately 5 months before as a small pink-red papule initially diagnosed as a wart. Gradually, the lesion grew in size and developed a darker area in the center (Figure 1). Dermoscopically, the lesion showed a multicomponent pattern with blue to black dots and globules, blue-white veil, and pinkish color, especially visible at the periphery (Figure 2). Histological evaluation revealed a well-circumscribed dermoepidermal pigmented atypical melanocytic prolifera- tion composed of epithelioid spindle cells (spitzoid cytology). A Strange Atypical Spitz Tumor Andrea Bassi1, Vincenzo Piccolo2, Maurizio de Martino1, Teresa Oranges1, Cesare Filippeschi1, Giuseppe Argenziano2 1 Department of Health Sciences, University of Florence & Department of Paediatric Medicine, Anna Meyer Children’s University Hospital, Florence, Italy 2 Dermatology Unit, University of Campania Luigi Vanvitelli, Naples, Italy Key words: dermoscopy, Spitz tumor, histopathology, atypical Spitz tumor Citation: Bassi A, Piccolo V, de Martino M, Oranges T, Filippeschi C, Argenziano G. A strange atypical Spitz tumor. Dermatol Pract Concept. 2019;9(3):237-238. DOI: https://doi.org/10.5826/dpc.0903a19 Accepted: February 12, 2019; Published: July 31, 2019 Copyright: ©2019 Bassi et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. Authorship: All authors have contributed significantly to this publication. Corresponding author: Vincenzo Piccolo, MD, c/o II Policlinico, Edificio 9, Primo piano, Via Pansini 5 - 80131 Napoli, Italy. Email: piccolo.vincenzo@gmail.com Figure 1. Small pink-red papule on the right knee (0.8  × 1 cm) with a cross shape distribution of the pig- ment. [Copyright: ©2019 Bassi et al.] Figure 2. Multicomponent pattern with blue to black dots and globules, blue-white veil, and pinkish color at the pe- riphery of the lesion. [Copyright: ©2019 Bassi et al.] 238 Letter | Dermatol Pract Concept 2019;9(3):19 Melanocytes were mostly arranged in confluent fascicles, with retraction artifacts. There was prominent central and lateral pagetoid spread (Figure 3). A final diagnosis of atypical Spitz tumor (AST), with free margins, was made. ASTs have been regarded as “borderline” melanocytic lesions with overlapping features with Spitz nevus and spit- zoid melanoma, but their true biological significance is still poorly understood. Pediatric ASTs seem to be associated with minimal lethal potential. In our case the clinical diagnosis was not straightforward; the site and the patient’s age led us to suspect initially a Spitz with an atypical presentation [1]. The atypical dermoscopy presentation and the atypical melanocytic proliferation of spitzoid cells led us to the final diagnosis. Given the complexities in interpretation, great care should be given when dealing with spitzoid lesions that show any degree of deviation from the classic features of Spitz nevus. Reference 1. Moscarella E, Piccolo V, Argenziano G, et al. Problematic lesions in children. Dermatol Clin. 2013;31(4):535-547. Figure 3. (A) Histopathological examination shows a raised, well-circumscribed, slightly asymmetric dermoepidermal atypical melanocytic proliferation associated with marked hyperkeratosis and irregular hyperplasia (H&E, original magnification ×2.5). (B) There is prominent central and lateral pagetoid spread of melanocytes and no epidermal ulceration (H&E, original magnification ×10). (C) Pigmented melano- cytes display an epithelioid spindle (spitzoid) morphology and are mostly arranged in confluent fascicles with peripheral retraction artifacts (H&E, original magnification ×20). (D) Melanocytes show mild pleomorphism and are intermingled with numerous melanophages (H&E, original magnification ×40). H&E = hematoxylin and eosin. [Copyright: ©2019 Bassi et al.] A C B D