{"id":"d0de5015-7f72-4da6-9140-dfcca09bc3c2","slug":"primary-diffuse-leptomeningeal-melanomatosis-a-case-report-of-an-unusual-presentation-in-a-pediatric-patient","title":"Primary diffuse leptomeningeal melanomatosis: A case report of an unusual presentation in a pediatric patient","authors":["Yamila Basilotta Marquez","Agustin Ruiz Johnson","Romina Argañaraz","Beatriz Mantese"],"abstract":"Background: Primary diffuse leptomeningeal melanomatosis (PDLM) is a rare neoplasm characterized by malignant melanocyte-derived lesions that diffusely affect the leptomeninges without extracranial metastases. This condition is particularly uncommon in pediatric patients, with few cases reported in the literature. The clinical presentation is often nonspecific, leading to potential misdiagnosis. Summary: We present a case of a 14-year-old female diagnosed with PDLM, who exhibited symptoms including headache, vomiting, and limb paresthesia. Imaging studies revealed interhemispheric leptomeningeal inflammation, and a biopsy confirmed the diagnosis despite the absence of pigmentation in the lesion. The patient experienced complications, including hydrocephalus and seizures, ultimately leading to palliative care and death four months post-diagnosis. Conclusion: PDLM is a rare and aggressive neoplastic disease with a poor prognosis, particularly in pediatric patients. The absence of pigmentation in this case highlights the need for awareness of non-pigmented variants of PDLM. Early diagnosis and the identification of tumor markers such as GD2 are crucial for appropriate management and treatment. Keywords: leptomeningeal, melanomatosis, pediatrics, tumor markers, diagnosis, hydrocephalus, palliative care","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/d0de5015-7f72-4da6-9140-dfcca09bc3c2/featured/hero-1781559806023.png","publishDate":"2024-01-12T00:00:00.000Z","doi":"10.25259/SNI_641_2023","categories":["Neuro-oncology","Letter to the Editor"],"fullTextUrl":"https://surgicalneurologyint.com/api/articles/d0de5015-7f72-4da6-9140-dfcca09bc3c2/pdf"}