CNS Melanoma Research Articles

Diagnosis of Primary CNS Melanoma With Neuroimaging

Diagnosis of Primary CNS Melanoma With Neuroimaging

Black brain: transformation of a melanocytoma with diffuse melanocytosis into a primary cerebral melanoma

We describe here a rare case of the transformation of a supratentorial melanocytoma and diffuse melanocytosis into a primary malignant, CNS melanoma. A 71-year-old woman was admitted after suffering from bulbar speech and dysfunction of fine motor skills for three years. Cerebral imaging revealed an inhomogeneous tumor mass in the right frontal lobe. After craniotomy and opening of the dura, a partially black brain surface was exposed. The solid tumor mass was completely resected. Histopathological workup of the surgical specimen (solid tumor) revealed a melanocytoma with transformation into a malignant melanoma. Three months after surgery, an MR scan was carried out, revealing local tumor recurrence and intracranial spreading. At this time the patient agreed to undergo whole brain radiation therapy, which was carried out. At this date patient has survived 18months since primary diagnosis with progressive neurological symptoms.

Primary spinal melanoma with bilateral papilledema

A case of primary leptomeningeal malignant melanoma localized in the cervical region in a 41-year-old woman is presented. The only clinical finding was intracranial hypertension with papilledema. A diagnosis of primary CNS melanoma was made after dermatological and ophthalmological consultations, ruled out a metastatic lesion. Primary leptomeningeal melanoma is an extremely rare spinal tumor. Its clinical presentation with signs of increased intracranial pressure but without cord symptoms is unusual. Clinical features of this case including the radiological and histologic findings are described. Diagnosis as well as management is discussed.

Neurocutaneous melanosis with associated Dandy-Walker complex

The authors report the case of a child with neurocutaneous melanosis associated with a Dandy-Walker complex. Magnetic resonance (MR) images showed shortened T1-weighted images in areas involving the amygdala, mesencephalon, rostral brain stem, and superior cerebellar surface compatible with melanin deposits. There was also partial agenesis of the cerebellar vermis with an enlarged fourth ventricle cyst along with a high-lying torcular and ventricular enlargement. Endoscopic fenestration and biopsy of the cyst wall was performed without evidence of abnormal melanin deposits in the meninges. The patient eventually required ventriculoperitoneal shunting and at 1-year follow-up did not develop evidence of primary CNS melanoma. ILLUSTRATION: Computed tomography and MR images consistent with neurocutaneous melanosis and the Dandy-Walker complex are presented along with photographs of the cutaneous nevi. The major clinical and radiological features of this rare association, with only 11 previously reported cases, are discussed in detail.

Neurocutaneous melanosis.

Neurocutaneous melanosis (NCM) is a rare congenital disorder characterized by the presence of large or multiple congenital melanocytic nevi in association with benign or malignant proliferation of melanocytes in the leptomeninges. NCM is believed to occur as a consequence of an error in morphogenesis in neural ectoderm in the developing embryo. Animal models suggest that aberrant expression of the hepatocyte growth factor/scatter factor (HGF/SF) may be involved in the pathogenesis in the NCM. While the majority of patients with NCM have large congenital melanocytic nevi in a posterior axial distribution, a significant proportion of patients present with multiple smaller nevi in the absence of a single larger lesion. Neurologic manifestations generally occur within the first two years of life, and are often related to increased intracranial pressure. Associated structural anomalies of the CNS have been reported in NCM, particularly the Dandy-Walker complex. The long-term clinical significance of characteristic magnetic resonance findings in neurologically asymptomatic patients is unclear. Approximately half of NCM patients develop CNS melanoma. The prognosis of symptomatic patients remains poor.

Primitive cerebral melanoma: case report and review of the literature

BACKGROUND Central nervous system primary malignant melanoma accounts for approximately 1% of all the cases of melanoma; reports in the literature are relatively rare. CASE DESCRIPTION A 74-year-old man was hospitalized because of an episode of aphasia. The neuroradiologic examinations demonstrated a round homogeneous lesion extending near the left sylvian fissure. He had no extracranial abnormalities. The patient underwent a neurosurgical procedure and the tumor was macroscopically totally excised. Pathological examination of the surgical specimen revealed a histological appearance similar to that of melanoma. A diagnosis of primary CNS melanoma was made after careful dermatologic and ophthalmologic examination, which ruled out presence of cutaneous or choroidal melanoma. The patient did not receive any further treatment and he is free of disease 2 years after diagnosis. CONCLUSIONS We report a case of primary cerebral melanoma of the left temporal lobe; clinical, neuroradiological, and histological findings are discussed with review of the literature. Primary melanoma of the CNS may present either with localized intra/extra-axial mass lesions or with meningeal spread, which carries a worse prognosis. The prognosis of cerebral primitive melanoma is variable, although it is common opinion that primitive cerebral melanoma has a better prognosis than cutaneous melanoma, with two cases in the literature surviving 9 and 12 years.