Clinicopathologic Features, Management, and Outcomes Of Pediatric CRTC1::TRIM11 and MED15::ATF1 Tumors With Spitzoid Morphology: A Case Series
- PMID: 40390195
- DOI: 10.1002/pbc.31814
Clinicopathologic Features, Management, and Outcomes Of Pediatric CRTC1::TRIM11 and MED15::ATF1 Tumors With Spitzoid Morphology: A Case Series
Abstract
Limited information is available on the clinicopathologic features and management of pediatric CRTC1::TRIM11 (CTRTs) and MED15::ATF1-rearranged tumors (MARTs). In this series of pediatric CTRTs (n = 5) and MARTs (n = 2), all tumors exhibited spitzoid morphology and expressed melanocytic markers. Metastatic progression occurred in one CTRT and two MARTs: all three received immunotherapy, resulting in disease control in two patients and one death due to progressive disease. A fourth individual had lymph node micrometastasis that remained stable without progression. This case series highlights the potentially aggressive behavior of these tumors and suggests that immunotherapy may be an effective treatment option for advanced disease.
Keywords: CRTC1::TRIM11 fusion; MED15::ATF1 fusion; Spitz; immunotherapy; melanoma; spitzoid.
© 2025 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA.
References
-
- D. E. Elder, B. C. Bastian, I. A. Cree, D. Massi, and R. A. Scolyer, “The 2018 World Health Organization Classification of Cutaneous, Mucosal, and Uveal Melanoma: Detailed Analysis of 9 Distinct Subtypes Defined by Their Evolutionary Pathway,” Archives of Pathology & Laboratory Medicine 144, no. 4 (2020): 500–522.
-
- M. R. Sargen, R. L. Barnhill, D. E. Elder, et al., “Evaluation and Surgical Management of Pediatric Cutaneous Melanoma and Atypical Spitz and Non‐Spitz Melanocytic Tumors (Melanocytomas): A Report from Children's Oncology Group,” Journal of Clinical Oncology 43, no. 9 (2025): 1157–1167.
-
- S. Lee, R. L. Barnhill, R. Dummer, et al., “TERT Promoter Mutations Are Predictive of Aggressive Clinical Behavior in Patients With Spitzoid Melanocytic Neoplasms,” Scientific Reports 5 (2015): 11200.
-
- B. E. Vest, C. L. Harview, V. Liu, et al., “Cutaneous Melanocytic Tumor With CRTC1::TRIM11 Fusion and Prominent Epidermal Involvement: A Case Report,” Journal of Cutaneous Pathology 49, no. 12 (2022): 1025–1030.
-
- B. E. Vest, K. G. Berrebi, M. S. Stone, M. R. Sargen, and P. Gerami, “CRTC1::TRIM11 cutaneous Tumor: An Update With Local Recurrence and Lung Metastasis in a Pediatric Patient,” Journal of Cutaneous Pathology 51, no. 3 (2024): 181–183.
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