Cerebellar amelanotic melanoma can mimic cerebellar abscess in a pediatric case of neurocutaneous melanosis

Mormina, Enricomaria; Vinci, Sergio Lucio; Coglitore, Alessandra; Visalli, Carmela; Tessitore, Agostino; Cavallaro, Marco; Galletta, Karol; Granata, Francesca

doi:10.1002/ccr3.3926

Cited by 2 publications

(2 citation statements)

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“…Overall survival of pediatric patients registered in the German Registry for Rare Pediatric Tumors (STEP registry) and literature with diagnosis of a leptomeningeal melanoma on the basis of a neurocutaneous melanocytosis (26 patients with exact survival time) 6–21 …”

Section: Resultsmentioning

confidence: 99%

“…The characteristics of these patients are displayed in Table2. Treatment consisted primarily of medical therapy (n = 20), most commonly based on trametinib (n = 9), temozolomide (n = 4), Overall survival of pediatric patients registered in the German Registry for Rare Pediatric Tumors (STEP registry) and literature with diagnosis of a leptomeningeal melanoma on the basis of a neurocutaneous melanocytosis (26 patients with exact survival time) [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. or vinca-alkaloids/dacarbazine (n = 3) with various other cytostatic agents, and PD-1 and/or CTLA-4 inhibition (n = 3).…”

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confidence: 99%

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Melanoma of the central nervous system based on neurocutaneous melanocytosis in childhood: A rare but fatal condition

Abele,

Forchhammer,

Eigentler

et al. 2024

Pediatric Blood & Cancer

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BackgroundMelanomas of the central nervous system (CNS) based on neurocutaneous melanocytosis (NCM) are exceptionally rare in childhood and have been described only sporadically. Rapidly progressive disease may represent a major challenge for treating physicians, especially given the limited knowledge about this condition. This analysis aimed to increase knowledge about the occurrence and treatment of these malignancies.ProcedureData on diagnosis, treatment, and outcome of patients aged 0–18 years with CNS melanoma based on NCM recorded in the German Registry for Rare Pediatric Tumors (STEP registry) were analyzed. Additionally, published case reports on this condition were analyzed.ResultsIn STEP, five patients with leptomeningeal melanoma based on NCM were identified, with a median age at melanoma diagnosis of 3.7 years. Various multimodal treatments were performed: (partial) resection (n = 4), irradiation (n = 2), trametinib (n = 3), different cytostatics (n = 2), and anti‐GD2 immunotherapy (n = 1). All patients died between 0.3 and 0.8 years after diagnosis. Including published case reports, 27 patients were identified with a median age of 2.8 years at melanoma diagnosis (range: 0.2–16.6). Fourteen of 16 cases with reported data had a NRAS alteration (88%), particularly NRAS p.Q61K (85%). In the expanded cohort, no patient survived longer than 1 year after diagnosis despite multimodal therapy (including trametinib; n = 9), with a median survival of 0.4 years (range 0.1–0.9).ConclusionsCNS melanomas based on NCM in childhood are aggressive malignancies without curative treatment to date. Therapeutic approaches must be individualized. Genetic tumor sequencing is essential to improve understanding of tumorigenesis and potentially identify new therapeutic targets.

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Section: Resultsmentioning

confidence: 99%