2015
DOI: 10.3340/jkns.2015.58.6.554
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Primary Intracranial Leptomeningeal Melanomatosis

Abstract: Primary intracranial malignant melanoma is a very rare and highly aggressive tumor with poor prognosis. A 66-year-old female patient presented a headache that had been slowly progressing for several months. A large benign pigmented skin lesion was found on her back. A brain MRI showed multiple linear signal changes with branching pattern and strong enhancement in the temporal lobe. The cytological and immunohiostochemical cerebrospinal fluid examination confirmed malignant melanoma. A biopsy confirmed that the… Show more

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Cited by 16 publications
(10 citation statements)
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“…Although diagnosis of primary CNS melanomas without biopsy is challenging, aggressive subarachnoid space invasion in meningeal melanomatosis may raise the odds of the presence of malignant cells in the CSF. In some human cases, as well as in our case, malignant melanocytes were observed in the CSF [3,9,15]. Although the probability of detecting malignant melanocytes in the CSF remains unclear in human literature, repeating CSF tap may be beneficial, since the first tap could only reveal elevated protein and pleocytosis without neoplastic cells, even when lesions were found on MRI [3,9].…”
supporting
confidence: 49%
“…Although diagnosis of primary CNS melanomas without biopsy is challenging, aggressive subarachnoid space invasion in meningeal melanomatosis may raise the odds of the presence of malignant cells in the CSF. In some human cases, as well as in our case, malignant melanocytes were observed in the CSF [3,9,15]. Although the probability of detecting malignant melanocytes in the CSF remains unclear in human literature, repeating CSF tap may be beneficial, since the first tap could only reveal elevated protein and pleocytosis without neoplastic cells, even when lesions were found on MRI [3,9].…”
supporting
confidence: 49%
“…Primary diffuse leptomeningeal melanomatosis (melanocytosis) is an extremely rare tumour, with the related literature primarily consisting of individual case reports. [2][3][4][5] Melanocytic lesions involving the CNS include both benign (leptomeningeal melanocytosis, melanocytoma) and malignant (leptomeningeal melanomatosis, melanoma) pathologies. 6 The signs and symptoms of these pathologies depend on the site of anatomical involvement (cerebral hemispheres, cranial nerves, spinal cord and nerve roots).…”
Section: Discussionmentioning
confidence: 99%
“…12 Most authors quote a median survival of less than one year. 3,4,11 Vemurafenib is a BRAF inhibitor that has demonstrated dramatic activity in BRAF V600E-mutated melanoma. There is a report of a patient who had melanoma with leptomeningeal melanomatosis, describing that the patient had remarkable clinical and radiological improvement after the initiation of vemurafenib.…”
Section: Discussionmentioning
confidence: 99%
“…In 1976, Hayward proposed this classification: (1) primary brain malignant melanoma, (2) secondary brain malignant melanoma, and (3) other brain tumors with melanin [ 17 ]. He described the primary CNS melanoma when there are (1) no malignant melanoma outside CNS, (2) absences of this lesion in other part of CNS, and (3) histological confirmations of melanoma [ 8 ]. Since then, no other criteria have been developed [ 18 ] probably because the low incidences of primary CNS melanoma.…”
Section: Case Presentationmentioning
confidence: 99%
“…Total excision of brain melanoma is the mainstay of the treatment in most cases [ 4 6 ]. Skin melanomas have an aggressive behavior and tend to metastasize to other organs [ 7 ]; nevertheless, extracerebral metastasis from primary brain melanoma is extremely rare [ 8 ].…”
Section: Introductionmentioning
confidence: 99%