Primary diffuse meningeal melanomatosis – a rare form of meningeal melanoma: case report

Abstract

BackgroundMeningeal melanomatosis is a rare type of central nervous system neoplasm (with incidence ranging between 3 and 5%) that develops in the course of malignant melanoma. In a small percentage of cases, meningeal melanomatosis may develop without a primary focus. It affects the leptomeninx. The clinical activity of the disease is uncharacteristic, with a number of neurological symptoms developing over weeks or months.Case presentationA 45-year-old male patient presented with consciousness disturbance, cognitive dysfunctions, seizures and progressive paresis. None of the examinations performed, including cerebrospinal fluid examination, neuroimaging and biopsy of the leptomeninges, permitted us to establish a diagnosis during the patient’s hospital stay. The diagnosis of meningeal melanomatosis was established after an autopsy had been carried out.ConclusionsIn the absence of unequivocal test results, it is also worth taking into account the primary changes in the leptomeninx, including those caused by melanoma.

Diagnosis of meningeal melanomatosis in a dog using magnetic resonance imaging and cerebrospinal fluid findings

“…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]. In a case series, two out of three patients had malignant melanocytes in the CSF, but in one of them, neoplastic cells were only detected from the repeated CSF tap [3].…”

“…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 ].…”

“…In human medicine, meningeal melanomatosis is extremely rare and involves a high degree of malignancy. The incidence of this disease is 0.005 in 100,000 people [ 9 ]. The rapid clinical progression and diffuse involvement of the entire CNS in our case reflects the malignancy of the disease.…”

Sudden Death from Primary Cerebral Melanoma: Clinical Signs and Pathological Observations

“…In our case, two months after the first symptoms, the patient died. The patient had a history of drug addiction, presenting psychomotor agitation and aggressive behavior, as can be observed in many cerebral neoplasms [ 54 , 55 , 56 , 57 ]. Morais described a case of delirium in a patient with brain melanoma metastases [ 58 ] and other authors reported headache and diplopia in a 27-year-old man affected by primary meningeal melanocytoma in the anterior cranial fossa [ 59 ].…”

Uncommon Subtypes of Malignant Melanomas: A Review Based on Clinical and Molecular Perspectives

“…The World Health Organization (WHO) classification divides the melanocytic lesions of the central nervous system into three types: diffuse melanosis, meningeal melanocytoma (benign course), and melanoma [ 210 ]. Considering the latter, two forms of PIMM should be highlighted: solid tumors and diffuse meningeal melanomatosis [ 211 ].…”