Primary intracranial melanoma, amelanotic variant: Case report

Sanz, Julio Alberto Andrés; Ginés, Juan Antonio Ruiz; Iliev, Hristo; Valiente, J. Aguas

doi:10.1016/j.neucie.2022.02.005

Cited by 2 publications

(1 citation statement)

References 14 publications

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“…Meningeal melanoma is estimated to be extremely rare, with 0.005 cases per 100,000 [2] or about 1% of all melanoma cases [3]. Prognosis is generally poor, with an average life expectancy of approximately 22 months [4]. The outcome is worse for patients with diffuse leptomeningeal involvement, with life expectancy of approximately 4 months [5].…”

Section: Introductionmentioning

confidence: 99%

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

Shahab,

Patil,

Fangusaro

et al. 2024

Current Oncology

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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.

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Section: Introductionmentioning

confidence: 99%

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

Shahab,

Patil,

Fangusaro

et al. 2024

Current Oncology

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Primary Malignant Meningeal Melanoma Complicated by Cerebral Venous Sinus Thrombosis: An Illustrative Case With a Systematic Review of the Literature

Mann,

Bhatt,

Tso

2024

Cureus

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Melanocytic tumors of the central nervous system (CNS) such as meningeal melanoma are exceedingly rare tumours derived from leptomeningeal melanocytes. We report an illustrative case of a previously healthy 47year-old male who presented with tonic-clonic seizure. Magnetic resonance imaging (MRI) with contrast demonstrated a homogenously enhancing right temporal extra-axial lesion. The patient was stabilized on anti-epileptic medications and dexamethasone prior to proceeding with complete surgical resection of the lesion. Intraoperatively, the lesion was heavily pigmented with invasion of the surrounding dura and skull. Histopathology revealed a poorly differentiated neoplasm with nuclear atypia and melanin-containing cells with strong SOX10 and variable S100 positivity. Computed tomography (CT) of the chest, abdomen, and pelvis showed no metastatic disease, and molecular profiling was negative including absent BRAF mutation. He began checkpoint inhibitor therapy and subsequently developed cerebral venous sinus thrombosis managed with anticoagulation. Sixteen months post-operatively, he was neurologically intact, working fulltime, and had resumed immunotherapy.We systematically reviewed the literature on primary intracranial malignant melanoma (PIMM) with the goal of understanding the prognosis and best treatment options for this disease. Our systematic review produced 82 articles (118 unique cases) of PIMM. The average age at diagnosis was 45.9 years (95% CI:42.9-48.9), and headache (54.2%) was the most common initial presentation. Eighty-nine percent of patients had primary surgical resection, and 41.0% of these individuals experienced a recurrence with a mean time to recurrence of 19.6 months (95% CI:6.95-32.23). Adjuvant therapy was administered in 65.7% of surgically resected patients; including radiotherapy, chemotherapy, immunotherapy, or a combination. In summary, PIMM is a rare tumour that can appear radiographically similar to meningioma. The results of our systematic review demonstrate that surgical resection remains the mainstay of therapy for best long-term prognosis.

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Primary intracranial melanoma, amelanotic variant: Case report

Cited by 2 publications

References 14 publications

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

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

Primary Malignant Meningeal Melanoma Complicated by Cerebral Venous Sinus Thrombosis: An Illustrative Case With a Systematic Review of the Literature

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