Reference | Makin, 1999 | Nicolaides, 1995 | Selcuk, 2008 | Demir, 2010 | Kosker, 2014 | Erdogan, 2014 | Our patient |
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Diagnosis | Primitive leptomeningeal melanoma | Primitive leptomeningeal melanoma | Atypical Teratoid Rhabdoid Tumor | Spinal low-grade neoplasm | Primary diffuse leptomeningeal gliomatosis | Primary spinal leptomeningeal gliomatosis | Primitive leptomeningeal melanoma |
Age and Sex | 5,5Â years, Male | 5Â years, Male | 6Â years, Female | 8Â years, Female | 3Â years, male | 3Â years, male | 2Â years, Female |
Onset signs and symptoms | 13-week history of headaches, vomiting, and weight loss followed by acute deterioration of conscious level | 3-month history of vomiting, anorexia, and weight loss, 1-month history of headaches and pyrexia, acute deterioration of conscious level | 2-months history of confusion, headache, vomiting, aphasia, and right hemiparesis | History of headache, nausea, fever, and vomiting, followed by double vision | 3-month history of strabismus and 1-week history of headache and restlessness | Deviation of left eye, weakness, lack of appetite, headache and behavioral change | 1-week history of vomiting |
Imaging at onset | CT: diffuse meningeal enhancement | CT: diffuse meningeal enhancement | MRI: marked leptomeningeal involvement and basal meningitis | MRI: communicating hydrocephalus, diffuse leptomeningeal enhancement at basal cisterns | MRI: leptomeningeal infiltration, prominent around the Sylvian fissure and at the level of the basal cisterns | MRI: diffuse leptomeningeal enhancement, predominantly involving the basal cisterns and hydrocephalus | MRI: enhancement of the cervical and basal meninges and cranial nerves, in addition to a small focal enhancement anterior to the pons |
CSF analysis at onset | - Protein 1.5 g/dL - Glucose 0.7 mmol/L - No cells | - Protein 1.5 g/L - Glucose 0.5 mmol/L (serum glucose 5 mmol/L) - Leukocytes 36/mm3 | - Protein 40.8 mg/dL - Glucose 36 mg/dL (serum glucose 136 mg/dL) - Lymphocytes 350/mm3 | - Protein 242 mg/dL - Glucose 74 mg/dL (serum glucose 116 mg/dL) - 10 × 5 cells (60 % lymphocyte, 40 % PMNL) | - Protein 9.2 mg/dL - Glucose 102 mg/dL (serum glucose 136 mg/dL) - Leukocytes 470/mm3 (90 % lymphocyte, 10 % PMNL) | - Protein elevated - Glucose normal | - Protein 62 mg/dL - Glucose 83 mg/dL (serum glucose 133 mg/dL) - Leukocytes 2/mm3 |
CSF cyto-morphological examination | ND | - 1st sample: negative - 2nd sample: positive for malignant cells | Negative | ND | Negative | Negative | - 1st sample: negative - 2rd sample: positive for malignant cells |
Time delay between onset of symptoms and definitive diagnosis | 3Â months | 3Â months | UNK | 4Â months | 10Â months | 4Â months | 10Â weeks |
Chemotherapy | Vincristine, carboplatin, and etoposide | Chemotherapy according to local protocol (not specified) | Not done (parent’s refusal) | Cisplatin and etoposide; radiotherapy | Vincristine, carboplatin, and etoposide; (parents refused radiotherapy) | Vincristine and carboplatin | Temozolomide, cis-platinum, vindesina and peginterferon alfa-2b; radiotherapy |
Outcome | Dead 6Â months after diagnosis | Unknown | Dead 3Â months after onset | Alive after 19Â months follow-up | Alive after 18Â months follow-up | Unknown | Dead 11Â months after diagnosis |