Spitz nevus

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OBJECTIVE: The aim of this study is to evaluate the demographic characteristics of patients diagnosed with Spitz nevus and to investigate potential distinctions in clinicopathological findings of Spitz nevi in relation to age and location of the lesion. MATERIALS AND METHODS: Clinical and histopathological findings of 32 patients who were diagnosed with Spitz nevus from our archives between 2010 and 2020 were obtained and evaluated retrospectively. RESULTS: A total of 32 patients were included, of whom 19 (59...

Spitz tumors are a subset of melanocytic neoplasms characterized by epithelioid or spindled melanocytes(1). The benign nature of the "Spitz nevus" has since been clarified, but the debate regarding Spitzoidtumors (STs) is still ongoing. Spitzoid tumors encompass a wide spectrum of cutaneous lesions ranging from benign Spitz nevus (SN) to Spitzoid melanoma (SM), the latter displaying capacity for widespread metastasis and a potentially lethal outcome (2). The term atypical Spitz tumors (ASTs) refers to melanocytic tumors exhibiting the morphological features of SN, as well as some features associated with malignancy, but not sufficient to classify them as SMs...

Over the last 75 years, our understanding of Spitz lesions has undergone substantial evolution. Initially considered a specific type of melanoma, the perception has shifted towards recognizing Spitz lesions as a spectrum comprising Spitz nevi, Spitz melanocytomas, and Spitz melanomas. Spitz lesions are known for posing a significant diagnostic challenge regarding the distinction between benign neoplasms displaying atypical traits and melanomas. A comprehensive understanding of their molecular basis and genomic aberrations has significantly improved precision in classifying and diagnosing these challenging lesions...

Background: Agmination and/or satellitosis in pigmented blue lesions is a phenomenon rarely mentioned in the literature and not well known. This phenomenon can be expressed by several benign and malignant pigmented blue lesions, such as blue nevi, Spitz nevi, melanocytoma and melanoma. On this spectrum, dermoscopy, reflectance confocal microscopy (RCM) and dynamic Optical coherence tomography (D-OCT) represent non-invasive imaging technologies, which may help clinicians in the diagnosis of melanoma and non-melanoma skin cancers in daily clinical practice...

Balloon cell melanoma (BCM) is an exceptionally uncommon histological variant, making up <1% of all malignant melanomas. Diagnosing balloon cell melanoma on cytological specimens can be challenging due to its scarcity and its similarity to other medical conditions. A comprehensive clinical assessment and histological analysis, coupled with immunohistochemical staining, play a crucial role in distinguishing balloon cell melanoma from various benign and malignant skin conditions. The differential diagnoses encompass spitz nevus, balloon cell nevus, clear cell sarcoma of tendons and aponeuroses (melanoma of soft tissues), metastatic clear cell renal cell carcinoma, sebaceous carcinoma, and benign adnexal tumours like clear cell hidradenoma...

After 25 years, "Ackerman's conundrum", namely, the distinction of benign from malignant Spitz neoplasms, remains challenging. Genomic studies have shown that most Spitz tumors harbor tyrosine and serine/threonine kinase fusions, including ALK , ROS1 , NTRK1 , NTRK2 , NTRK3 , BRAF and MAP3K8 , or some mutations, such as HRAS and MAP3K8 . These chromosomal abnormalities act as drivers, initiating the oncogenetic process and conferring basic bio-morphological features. Most Spitz tumors show no additional genomic alterations or few ones; others harbor a variable number of mutations, capable of conferring characteristics related to clinical behavior, including CDKN2A deletion and TERT -p mutation...

Atypical and malignant cutaneous tumors are understudied in the pediatric population, with limited data on long-term follow-up. This study examines pediatric (0-18 years) atypical melanocytic proliferations over a twenty-year period (January 2002-December2022) using the EPIC SlicerDicer at our institution. Over a twenty-year period, there were 55 cases of pediatric melanoma (53 patients). The median follow-up time was 8 years, 11 months. A proportion of 96% were treated with wide local excision (WLE), and 47% had a sentinel lymph node biopsy (SLNB) (35% positive rate)...

INTRODUCTION: The Spitz nevus (SN) is an acquired melanocytic neoplasm composed of epithelioid and/or spindle cells, which tends to develop in childhood. In pediatric patients, it is usually located on the face and neck. Unusual locations have been found in the literature, such as the penis, mouth, and tongue, as well as 2 cases of ungual SN. CASE REPORT: A 15-year-old male evaluated for dark brown-black longitudinal melanonychia that covered 40% of the nail, with pseudo-Hutchinson's sign, of 1 year of evolution...

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Spitz tumors are melanocytic neoplasms morphologically characterized by spindled and/or epithelioid cells and specific stromal and epidermal changes associated with mutually exclusive fusion kinases involving ALK, ROS1, NTRK1, NTRK2, NTRK3, MET and RET, BRAF and MAP3K8 genes or, less commonly, HRAS mutation. RAF1 fusions have been recently detected in cutaneous melanocytic neoplasms, including conventional melanoma, congenital nevus and BAP-1 inactivated tumors. We report herewith three Spitz neoplasms with a RAF1 fusion, including a previously reported CTDSPL::RAF1 fusion and two novel PPAP2B::RAF1 and ATP2B4::RAF1 fusions...

Activating mutations in MAP2K1 can be seen in benign and intermediate-grade melanocytic neoplasms with spitzoid morphology. We analyzed the clinical, histopathologic, and genetic features for 16 cases of benign and intermediate-grade melanocytic tumors harboring activating MAP2K1 mutations. We compared them to Spitz neoplasms with characteristic Spitz fusions or HRAS mutation. We also compared the mutational pattern of benign and intermediate-grade MAP2K1-mutated neoplasms and melanomas with activating MAP2K1 mutations...

Spitzoid lesions are challenging melanocytic lesions comprising benign, intermediate, and malignant lesions. In this study, we aimed to analyze the diagnostic accuracy of clinical and dermatoscopical evaluations of pediatric spitzoid ear lesions. We collected and analyzed, clinically, dermatoscopically, and histologically, pediatric spitzoid ear lesions. We also conducted a systematic review of the literature. At the Pediatric Hospital Gaslini, excision and histopathological evaluation were performed on eight cases: 87...

In addition to morphologic analysis, molecular diagnostic work up of Spitz tumours is often of great value for their accurate diagnosis/classification. Nowadays, next-generation sequencing (NGS) is the predominant screening method in molecular diagnostics. Up to 80% of these melanocytic neoplasms comprise gene fusions as genetic anomalies for which the driver codes for a protein harbouring a kinase domain. However, because of the variety of fusion partners the use of PCR-based targeted enrichment NGS methods is not recommended...

Eccrine duct fibroadenomatosis is a reactive histological phenomenon observed in a variety of inflammatory and neoplastic pathologies. We report a case of a ROS1 fusion angiomatoid Spitz nevus with associated acrosyringeal hyperplasia and a syringoma-like ductal proliferation in a young female patient.

BACKGROUND: Melanocytic tumors driven by MAP2K1 in-frame deletions are among the most recently described class of melanocytic neoplasms. The reported range of diagnoses and associated genomic aberrations in these neoplasms is wide and includes melanomas, deep penetrating melanocytomas, and pigmented epithelioid melanocytoma. However, little is known about the characteristics of these tumors, especially in the absence of well-known second molecular "hits." Moreover, despite their frequent spitzoid cytomorphology, their potential categorization among the Spitz tumors is debatable...

INTRODUCTION: Spitz nevi (SN) include a wide range of benign melanocytic nevi, which are controversial due to their morphologic resemblance to melanoma (MM). AIM: To describe dermoscopic and reflectance confocal microscopic (RCM) features of SN compared to MM and assess the RCM utility in the differential diagnosis. MATERIAL AND METHODS: We performed a multicentre retrospective analysis of MM and SN evaluated with dermoscopy and reflectance confocal microscopy...

INTRODUCTION: Spitzoid lesions are a wide tumour class comprising Spitz nevus (SN), atypical Spitz tumour (AST) and Spitz melanoma (SM). MATERIALS AND METHODS: We conducted a single-centre-based retrospective survey on all histologically diagnosed spitzoid lesions of paediatric patients (1-18 years) of the last 10 years (2012-2022). Histopathological reports and electronic records of patients were used to retrieve relevant data regarding patients' features, clinical and dermatoscopical aspects of lesions when recorded, and FISH tests when present...

INTRODUCTION: Phacomatosis pigmentokeratotica (PPK), an epidermal nevus syndrome, is characterized by the coexistence of nevus spilus and nevus sebaceus. Within the nevus spilus, an extensive range of atypical nevi of different morphologies may manifest. Pigmented lesions may fulfill the ABCDE criteria for melanoma, which may prompt a physician to perform a full-thickness biopsy. MOTIVATION: Excisions result in pain, mental distress, and physical disfigurement. For patients with a significant number of nevi with morphologic atypia, it may not be physically feasible to biopsy a large number of lesions...

BACKGROUND: The indication for surgical management and histological diagnosis of melanocytic nevi in children is a major challenge in clinical routine. In consultations with children and parents, the exclusion of malignant findings, on the one hand, and the risk of complications, on the other hand, are important. PATIENTS AND METHODOLOGY: Included were 946 children under the age of 10 years who underwent surgery with a suspected diagnosis of melanocytic nevus at the University Department of Dermatology, Tübingen, Germany, between 2008 and 2018...