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Primary leptomeningeal melanoma: an unusually aggressive tumor in childhood
D Allcutt1, S Michowiz, S Weitzman
1Division of Neurosurgery, Hospital for Sick Children, University of Toronto, Ontario, Canada.
Insights
Primary leptomeningeal melanoma in children is rare and aggressive. Early diagnosis and combined treatments like chemotherapy and radiation may improve survival for this challenging central nervous system tumor.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Dermatology
Background:
- Primary leptomeningeal melanoma is an exceptionally rare and aggressive central nervous system tumor in pediatric patients.
- This study reviews eight cases diagnosed between 1964 and 1990, highlighting diagnostic challenges and treatment outcomes.
Observation:
- Patients presented with symptoms of increased intracranial pressure due to hydrocephalus, often with delayed diagnosis.
- Associated findings included hairy nevi in some cases.
- Cerebrospinal fluid (CSF) analysis revealed elevated pressure, low glucose, and high protein, with malignant melanoma cells detected in three patients.
Findings:
- Diagnosis was confirmed via pathological examination of surgical or post-mortem tissues, or through CSF cytology.
- Hydrocephalus was managed with shunts, and two patients underwent subtotal tumor resection.
- Treatment regimens included radiation therapy and chemotherapy, with promising survival in two patients receiving cisplatinum, dimethyltriazenoimidazole carboxamide, and craniospinal irradiation.
Implications:
- The rarity and aggressive nature of pediatric leptomeningeal melanoma necessitate prompt diagnosis and multimodal treatment strategies.
- Further research into effective therapeutic approaches is crucial for improving outcomes in affected children.
- Understanding the clinical presentation and diagnostic markers is vital for timely intervention.
Abstract:
Primary malignant melanoma of the leptomeninges of the central nervous system is a rare and aggressive tumor in children. We report our experience from 1964 to 1990 with this tumor in eight children. The mean age at diagnosis was 4.9 years (range, 1.3 to 13 yr). Five children presented with signs and symptoms of raised intracranial pressure from hydrocephalus secondary to tumoral obliteration of the basal cisterns, but the time from the initial symptomatology to diagnosis was frequently delayed. Three patients in this series had hairy nevi in association with their leptomeningeal melanoma. Cerebrospinal fluid (CSF) analysis typically showed raised opening pressures, decreased glucose, and increased protein concentrations. Malignant melanoma cells were found in the CSF in three patients. Confirmatory radiographic examinations included air encephalography, myelography, and computed tomographic and magnetic resonance scanning. Four patients were treated with lumboperitoneal shunts, and one patient was treated with a ventriculoperitoneal shunt for hydrocephalus. Two patients underwent craniotomies and subtotal excisions of their tumors. In seven patients, a definitive diagnosis of leptomeningeal melanoma was made by pathological examination of tissues sent at surgery or at post mortem. In one case, the diagnosis was established by a detailed cytological analysis of the CSF. Four children died of fulminant disease and tumor spread before treatment could be instituted. The four children who received treatment had a combination of radiation therapy and chemotherapy. One child received intrathecal methotrexate. The two children with the longest survivals (2 and 3 yr, respectively) received cisplatinum and dimethyltriazenoimidazole carboxamide in addition to craniospinal irradiation.(ABSTRACT TRUNCATED AT 250 WORDS)