Conceptual Evolution and Current Approach to Spitz Tumors

Urso, Carmelo; Giorgi, Vincenzo De; Massi, Daniela

doi:10.3390/dermatopathology9020017

Cited by 2 publications

(1 citation statement)

References 41 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…As a matter of fact, large spindle and epithelioid neoplasms are highly controversial skin melanocytic tumors [2,3]. Because of their histological atypia, they were originally regarded as melanomas with a favorable prognosis, occurring in childhood (juvenile melanomas) [4].…”

Section: Introductionmentioning

confidence: 99%

Spitz Tumors and Melanoma in the Genomic Age: A Retrospective Look at Ackerman’s Conundrum

Urso

2023

Cancers

Self Cite

View full text Add to dashboard Cite

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. Since the accumulation of mutations is gradual and progressive, tumors appear to form a bio-morphologic spectrum, in which they show a progressive increase of clinical risk and histological atypia. In this context, a binary classification Spitz nevus-melanoma appears as no longer adequate, not corresponding to the real genomic substrate of lesions. A ternary classification Spitz nevus-Spitz melanocytoma-Spitz melanoma is more adherent to the real neoplastic pathway, but some cases with intermediate ambiguous features remain difficult to diagnose. A prognostic stratification of Spitz tumors, based on the morphologic and genomic characteristics, as a complement to the diagnosis, may contribute to better treatment plans for patients.

show abstract

Section: Introductionmentioning

confidence: 99%