Primary CNS Melanoma Research Articles

Nevus of Ota associated with intracranial melanoma: Case report and review of the literature

<p>There is a known association between nevus of Ota and melanomas involving the brain parenchyma and/or the meninges. We present the unusual case of a 32-year-old African-American female with a nevus of Ota and a contralateral parenchymal, primary CNS melanoma. We discuss the unique features of this case and provide a brief review of the literature regarding nevi of Ota and associated CNS melanoma. Our patient is a 32 year-old, African-American female with a left-sided nevus of Ota who presented with a three month history of headaches and paresthesias involving her left face and arm. An MRI of the brain revealed a hemorrhagic mass in the right temporal lobe, which, after craniotomy, was determined to be a melanoma. Extensive imaging, ophthamologic examination and full-body skin examination revealed no other foci of melanoma. To our knowlege, this is the only case of a nevus of Ota associated with contralateral parenchymal melanoma in an African-American patient. The association of contralateral parenchymal primary CNS melanoma with nevus of Ota is extremely unusual. Futhermore, despite the association of nevus of Ota with CNS melanoma, the literature does not support routine screening of patients with nevus of Ota for CNS melanoma with imaging modalities. </p>

Primary CNS Melanoma: A Case Report with Review of the Literature

Primary CNS Melanoma: A Case Report with Review of the Literature

Nevus of Ota associated with intracranial melanoma: Case report and review of the literature

<p>There is a known association between nevus of Ota and melanomas involving the brain parenchyma and/or the meninges. We present the unusual case of a 32-year-old African-American female with a nevus of Ota and a contralateral parenchymal, primary CNS melanoma. We discuss the unique features of this case and provide a brief review of the literature regarding nevi of Ota and associated CNS melanoma. Our patient is a 32 year-old, African-American female with a left-sided nevus of Ota who presented with a three month history of headaches and paresthesias involving her left face and arm. An MRI of the brain revealed a hemorrhagic mass in the right temporal lobe, which, after craniotomy, was determined to be a melanoma. Extensive imaging, ophthamologic examination and full-body skin examination revealed no other foci of melanoma. To our knowlege, this is the only case of a nevus of Ota associated with contralateral parenchymal melanoma in an African-American patient. The association of contralateral parenchymal primary CNS melanoma with nevus of Ota is extremely unusual. Futhermore, despite the association of nevus of Ota with CNS melanoma, the literature does not support routine screening of patients with nevus of Ota for CNS melanoma with imaging modalities. </p>

MEK inhibition appears to improve symptom control in primary NRAS-driven CNS melanoma in children

Background:Primary melanoma of the CNS in children is extremely rare, and usually linked to congenital melanocytic naevus syndrome, caused by mosaicism for oncogenic NRAS mutations. Outcome is fatal in all cases. Data from murine and in vitro studies suggest that MEK inhibition is a possible therapeutic option.Methods:Four children with NRAS-mutated CNS melanoma were treated with Trametinib on a compassionate basis.Results:All four had an improvement in symptoms and objectively in signs. These varied from mild improvement for 1 month, to a sustained symptom-free period of 9 months in one case. In all cases there was eventual disease progression through treatment, followed by rapid death after discontinuation. There were no clinically-significant side effects.Conclusions:Trametinib is the first therapy to show any objective or measurable effect in NRAS-mutated primary CNS melanoma, with few side effects in this small series. The role of this therapy should be explored further in this rare paediatric tumour.

Primary CNS Malignant Melanoma Associated With a Contralateral Nevus of Ota in an African-American Female: Unique Case Report

Background: Primary CNS melanoma is a rare neoplasm of the brain. Nevus of Ota is an area of macular, melanocytic pigmentation of the sclera and skin around the eye. Association between intracranial melanocytic neoplasms and Nevus of Ota has previously been described in case reports. The two lesions tend to occur ipsilaterally and in Caucasian patients. We present the first documented case of a primary malignant CNS melanoma contralateral to a Nevus of Ota in an African-American patient. Case Discussion: A 32-year-old female presented with intermittent numbness involving the left side of her face, arm, and leg. General physical exam revealed a large area of hyperpigmentation involving the left side of her face, especially around the eye and upper cheek, consistent with Nevus of Ota. Neuroimaging revealed a hemorrhagic intraparenchymal mass in the right frontotemporal region. Surgical resection of the lesion was performed, and pathology was consistent with primary CNS malignant melanoma. Conclusion: Primary CNS melanocytic neoplasms and Nevus of Ota share a similar embryological origin. Thus, the associated occurrence of the two is not surprising. We present a unique case of primary CNS malignant melanoma associated with a contralateral Nevus of Ota in an African-American patient. Primary CNS melanoma should be considered in the differential diagnosis of any patient with a Nevus of Ota who is found to have an intracranial mass.

MS-30 * MENINGEAL MELANOCYTOMA WITH MULTIFOCALITY, RAPID CNS SEEDING, AND SYSTEMIC METASTASES

OBJECTIVE: Meningeal melanocytomas are rare entities that range from benign and well-differentiated lesions with a favorable prognosis to higher-grade lesions that may undergo malignant transformation with subsequent CNS dissemination. Transition from a meningeal melanocytoma to primary CNS melanoma is particularly rare with only a handful of cases reported. Systemic spread of a transformed meningeal melanocytoma, an exquisitely rare occurrence, is, to the best of our knowledge, reported in only three cases. Here we report the case of a woman who initially presented with multifocal meningeal melanocytomas with subsequent rapid transformation, diffuse CNS seeding, and systemic metastases. CLINICAL PRESENTATION AND MANAGEMENT: A 43 year-old woman presented with headaches, nausea, and vomiting for two months with negative GI work-up. She experienced a syncopal episode with CT and MRI brain scans showing 3 dural-based lesions involving the foramen magnum, fourth ventricle, and right CPA. The patient underwent STR of the lesions; the anterior lesion was inaccessible. Pathology reported a melanocytoma with malignant features. A negative dermatology and ophthalmology work-up were performed. The patient received post-operative radiotherapy (50.4 Gy) to the posterior fossa. Six months following surgery she presented with new neurological symptoms, and MRI of the neuroaxis showed new leptomeningeal enhancement within the cerebellum, thoracic spine, and supratentorially. With neuro-oncology input, she was started on Temodar and underwent palliative spinal RT. Unfortunately she continued to experience pain, and follow-up MRI showed new intrathecal C-spine nodules. Abdominal imaging revealed multiple lung and liver metastases, with biopsy of a 7-cm liver lesion revealing malignant melanoma. The patient unfortunately continued to deteriorate and succumbed to her disease approximately 9 months following diagnosis. CONCLUSION: Meningeal melanocytomas are rare tumors that are not only challenging to diagnose, but challenging to clinically predict and manage. This case illustrates these challenges and the multidisciplinary response needed to optimize outcomes.

English

Primary intracranial melanoma is a rare and uncommon lesion. Association of primary CNS melanoma in an albino has not been reported in literature searched till now. We are presenting a rare case of primary CNS melanoma in a 52years old male with occulocutaneous albinism. The patient presented with repeated episodes of generalized headache, vomiting and ataxia for duration of 5months. MRI examination showed a tumor in the posterior fossa that was diagnosed as Ependymoma radiologically. Surgical treatment with total removal of tumor was done. Intra- operative squash cytology and frozen section followed by histopathology confirmed the diagnosis of Melanoma. A thorough investigation of the patient was performed including chest radiography, ocular examination, ultrasonography of abdomen and barium enema to rule out any other site of primary melanoma in the body. Thus a final diagnosis of primary CNS melanoma was given.

A Model for Primary Melanoma of the CNS Implicates NRAS

In this issue of Cancer Discovery, Pedersen and colleagues present the first mouse model of primary CNS melanoma, which arises when oncogenic NRAS is expressed from the endogenous Nras promoter in melanocytes during embryogenesis. In support of this model, two pediatric cases of NRAS-mutant primary melanoma of the CNS are identified.

Diagnosis of Primary CNS Melanoma With Neuroimaging

Diagnosis of Primary CNS Melanoma With Neuroimaging

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.

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.