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Current Oncology
Volume 31
Issue 1
10.3390/curroncol31010041
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Case Report

Primary Diffuse Leptomeningeal Melanomatosis in a Child with Extracranial Metastasis: Case Report

by
Shubin W. Shahab
1,2,3,4,*,
Prabhumallikarjun Patil
2,3,
Jason R. Fangusaro
1,2,3,4,
Brooke Patteson
1,2,
Adam Goldman-Yassen
2,5,
Bree R. Eaton
2,4,6,
William Boydston
2,7,
Matthew Schniederjan
2,8 and
Dolly Aguilera
1,2,3
1
Aflac Cancer and Blood Disorders Center, Atlanta, GA 30342, USA
2
Children’s Healthcare of Atlanta, Atlanta, GA 30322, USA
3
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA 30322, USA
4
Winship Cancer Institute, Atlanta, GA 30322, USA
5
Department of Radiology, Emory University School of Medicine, Atlanta, GA 30322, USA
6
Department of Radiation Oncology, Emory University School of Medicine, Atlanta, GA 30322, USA
7
Department of Neurosurgery, Emory University School of Medicine, Atlanta, GA 30322, USA
8
Department of Pathology, Emory University School of Medicine, Atlanta, GA 30322, USA
*
Author to whom correspondence should be addressed.
Curr. Oncol. 2024, 31(1), 579-587; https://doi.org/10.3390/curroncol31010041
Submission received: 20 December 2023 / Revised: 14 January 2024 / Accepted: 19 January 2024 / Published: 20 January 2024
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Abstract

Primary meningeal melanomatosis is an extremely rare tumor with very few documented responses to treatment. A 3-year-old male with a complex past medical history, including prematurity and shunted hydrocephalus, was diagnosed with primary meningeal melanomatosis with peritoneal implants. Molecular testing revealed an NRAS Q61R mutation. The patient received proton craniospinal radiation followed by immunotherapy with nivolumab (1 mg/kg) and ipilimumab (3 mg/kg) IV every 3 weeks and, upon progression, he was switched to a higher dose of nivolumab (3 mg/kg IV every 2 weeks) and binimetinib (24 mg/m2/dose, twice a day). The patient had significant improvement of CNS disease with radiation therapy and initial immunotherapy but progression of extracranial metastatic peritoneal and abdominal disease. Radiation was not administered to the whole abdomen. After two cycles of nivolumab and treatment with the MEK inhibitor binimetinib, he had radiographic and clinical improvement in abdominal metastasis and ascitis. He ultimately died from RSV infection, Klebsiella sepsis, and subdural hemorrhage without evidence of tumor progression. This is the first report of a child with primary meningeal melanomatosis with extracranial metastatic disease with response to a combination of radiation, immunotherapy and MEK inhibitor therapy.
Keywords: primary CNS melanoma; meningeal melanomatosis; diffuse leptomeningeal melanoma; immunotherapy; MEK inhibitor primary CNS melanoma; meningeal melanomatosis; diffuse leptomeningeal melanoma; immunotherapy; MEK inhibitor

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MDPI and ACS Style

Shahab, S.W.; Patil, P.; Fangusaro, J.R.; Patteson, B.; Goldman-Yassen, A.; Eaton, B.R.; Boydston, W.; Schniederjan, M.; Aguilera, D. Primary Diffuse Leptomeningeal Melanomatosis in a Child with Extracranial Metastasis: Case Report. Curr. Oncol. 2024, 31, 579-587. https://doi.org/10.3390/curroncol31010041

AMA Style

Shahab SW, Patil P, Fangusaro JR, Patteson B, Goldman-Yassen A, Eaton BR, Boydston W, Schniederjan M, Aguilera D. Primary Diffuse Leptomeningeal Melanomatosis in a Child with Extracranial Metastasis: Case Report. Current Oncology. 2024; 31(1):579-587. https://doi.org/10.3390/curroncol31010041

Chicago/Turabian Style

Shahab, Shubin W., Prabhumallikarjun Patil, Jason R. Fangusaro, Brooke Patteson, Adam Goldman-Yassen, Bree R. Eaton, William Boydston, Matthew Schniederjan, and Dolly Aguilera. 2024. "Primary Diffuse Leptomeningeal Melanomatosis in a Child with Extracranial Metastasis: Case Report" Current Oncology 31, no. 1: 579-587. https://doi.org/10.3390/curroncol31010041

APA Style

Shahab, S. W., Patil, P., Fangusaro, J. R., Patteson, B., Goldman-Yassen, A., Eaton, B. R., Boydston, W., Schniederjan, M., & Aguilera, D. (2024). Primary Diffuse Leptomeningeal Melanomatosis in a Child with Extracranial Metastasis: Case Report. Current Oncology, 31(1), 579-587. https://doi.org/10.3390/curroncol31010041

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