Primary Central Nervous System Melanoma Research Articles

Clinicopathological features, current status, and progress of primary central nervous system melanoma diagnosis and treatment.

Primary central nervous system (CNS) melanoma is an extremely rare condition, with an incidence rate of 0.01 per 100,000 individuals per year. Despite its rarity, the etiology and pathogenesis of this disease are not yet fully understood. Primary CNS melanoma exhibits highly aggressive biological behavior and presents clinically in a distinct manner from other types of melanomas. It can develop at any age, predominantly affecting the meninges as the primary site, with clinical symptoms varying depending on the neoplasm's location. Due to the lack of specificity in its presentation and the challenging nature of imaging diagnosis, distinguishing primary CNS melanoma from other CNS diseases. The combination of challenges in early detection, heightened tumor aggressiveness, and the obscured location of its origin contribute to an unfavorable prognostic outcome. Furthermore, there has been currently no consensus on a standardized treatment approach for primary CNS melanoma. Despite recent advancements in targeted therapy and immunotherapy for CNS melanoma, patients with primary CNS melanoma have limited treatment options due to their inadequate response to these therapies. Here, we provided a comprehensive summary of the epidemiology, clinical features, molecular pathological manifestations, and available diagnostic and therapeutic approaches of primary CNS melanoma. Additionally, we proposed potential therapeutic strategies for it.

Primary Intracranial Malignant Melanoma Mimicking Meningioma—Report of Two Cases

AbstractPrimary intracranial melanoma is uncommon and accounts for only approximately 1% of all cases of melanoma. This is interesting to neuro-oncologists and neurosurgeons because the clinical and radiological patterns of these tumors can mimic the presence of meningioma. Primary central nervous system melanomas have rarely been reported with less than 25 cases reported till date. We report two cases of the primary intracranial melanoma that even though very rare should be kept as a differential diagnosis when meningioma is suspected.

Primary Malignant Melanoma of the Brain: A Population-Based Study

Primary melanocytic neoplasms of the central nervous system (CNS) are rare and account for 1%of all melanomas. This study used the Surveillance, Epidemiology, and End Results (SEER) database to evaluate the epidemiology of primary CNS melanoma and further characterize their treatment. Data from the National Cancer Institute SEER program, collected from 1973-2015, were retrospectively analyzed. A total of 86 records of malignant melanoma cases with CNS as the primary site were identified, and 54 patients were studied based on the inclusion criteria. Demographic, tumor, and treatment regimen effectiveness were studied. A total of 54 patients were included in this study. Tumors were distributed evenly in size and localized primarily to the cerebral meninges and spinal cord. A total of 13% of patients underwent biopsy, 40.7% gross total resection (GTR), 7.4% subtotal resection (STR), 46.3% radiation therapy (RT), and 27.3% chemotherapy (CT) in a variety of treatment combinations. GTR alone and STR+RT resulted in increased disease-specific survival compared to biopsy alone, but no survival benefit was found with biopsy with RT and/or CT as well as STR alone. To our knowledge, this is the largest single database study completed for primary malignant melanoma of the CNS. The study identified the need for tumor resection for the proper treatment of these lesions, particularly GTR. GTR could be paired with adjuvant RT or RT+ CT providing survival benefit as well. In cases when GTR is unable to be completed, STR+ RT provides significant improvement in survival compared to biopsy alone.

Incidence, Outcomes and Predictors of Primary Central Nervous System Melanoma: A SEER-Based Study

Primary central nervous system (pCNS) melanoma is an extremely rare malignant tumor. We explored the incidence, outcomes, and predictors of pCNS melanoma. We queried the Surveillance, Epidemiology, and End Results database to identify all patients diagnosed with pCNS melanoma during 1973-2015. Overall survival (OS) was obtained by using the Kaplan-Meier curves. Log-rank test was used to compare survival across groups of age, sex, race, tumor location, size, surgical resection, radiotherapy, chemotherapy and year of diagnosis. Cox regression was used for univariate and multivariate analysis of survival. A total of 84 pCNS melanomas were identified with a 5-year OS of 37.7%. The overall age-adjusted incidence rate was 0.52 per 10,000,000 person-years. Age ≤19 years (vs. age 20-59 years, hazard ratio [HR]= 2.37, 95% confidence interval [CI]: 1.11-5.07, P= 0.03) and intracranial location (vs. intraspinal, HR= 1.98, 95% CI: 1.04-3.77, P= 0.04) were associated with decreased survival rate. Gross total resection surgery (vs. partial resection, HR= 0.31, 95% CI: 0.15-0.66, P= 0.002) was associated with improved survival rate. There was no significant association between other demographic characteristics, tumor size, therapy methods, year of diagnosis, and OS. The overall age-adjusted incidence rate of pCNS melanoma is 0.52 per 10,000,000 person-years. Age ≤19 years and intracranial tumor location are independent risk factors of low survival rate, whereas gross total resection is associated with better survival rate.

Neurocutaneous melanosis with pulmonary metastasis: looking beyond the skin

Neurocutaneous melanosis are neuroectodermal disorders characterized by giant congenital melanocytic nevus or multiple (≥3) nevus’s associated with melanocytic leptomeningeal tumour. Central nervous system tumour can be benign or malignant and usually presents at an early age. The neurological manifestation includes raised intracranial pressure, seizures and spinal cord compression. The dissemination of malignant nevomelanocytes to the peritoneal cavity has been observed with ventriculoperitoneal shunt placement in neurocutaneous melanosis. We report an infant of neurocutaneous melanosis with primary central nervous system melanoma and its metastasis to the lung in view of rarity.

Pigmented Lesions of the Nervous System and the Neural Crest: Lessons From Embryology.

Neurosurgeons encounter a number of pigmented tumors of the central nervous system in a variety of locations, including primary central nervous system melanoma, blue nevus of the spinal cord, and melanotic schwannoma. When examined through the lens of embryology, pigmented lesions share a unifying connection: They occur in structures that are neural crest cell derivatives. Here, we review the important progress made in the embryology of neural crest cells, present 3 cases of pigmented tumors of the nervous system, and discuss these clinical entities in the context of the development of melanoblasts. Pigmented lesions of the nervous system arise along neural crest cell migration routes and from neural crest-derived precursors. Awareness of the evolutionary clues of vertebrate pigmentation by the neurosurgical and neuro-oncological community at large is valuable for identifying pathogenic or therapeutic targets and for designing future research on nervous system pigmented lesions. When encountering such a lesion, clinicians should be aware of the embryological basis to direct additional evaluation, including genetic testing, and to work with the scientific community in better understanding these lesions and their relationship to neural crest developmental biology.

Primary melanoma of the cochlea with cerebellopontine extension and leptomeningeal spread

Less than 1 per cent of tumours occurring in the region encompassing the internal auditory canal and the cerebellopontine angle are malignant. Primary central nervous system melanomas arising from this region are exceptionally rare and are often initially misdiagnosed as acoustic neuromas. We present a 71-year-old man with acute vestibular disturbance and unilateral hearing loss. Magnetic resonance imaging demonstrated a mass, thought to be a cochlear nerve schwannoma, involving the cochlea and the internal auditory canal. At surgery, a pigmented mass adherent to the facial nerve was visualised, and the observed histopathology was consistent with a malignant melanoma. No extracranial site for the primary tumour was found, suggestive of a primary central nervous system melanoma. Despite surgical resection and adjuvant radiotherapy, the patient re-presented with extensive leptomeningeal disease 16 months later. Malignant tumours in the internal auditory canal and cerebellopontine angle region are rare. Early diagnosis and management are aided by recognition of characteristic factors such as a history of prior malignancy, atypical magnetic resonance imaging findings and accelerated audiovestibular symptoms. Despite the presented patient's outcome, total surgical resection with post-operative radiotherapy remains the recommended treatment.

The Risk of Melanoma and Neurocutaneous Melanosis Associated with Congenital Melanocytic Nevi

Congenital melanocytic nevi are commonly encountered in clinical practice. Although the development of malignant melanoma arising in small and intermediate congenital melanocytic nevi is rare, there is a significant risk of malignant degeneration associated with large congenital melanocytic nevi, in particular those that arise on the torso in the so-called "bathing trunk" distribution, where the risk is estimated to be about 2.5% to 5%. The risk of malignant melanoma arising within a large congenital melanocytic nevus is highest in the first 5 to 10 years of life and carries a significant mortality. Large congenital melanocytic nevi, in particular those overlying the posterior axis and occurring in the context of multiple satellite melanocytic nevi, are also associated with the development of neurocutaneous melanosis, which may result in neurologic and neurodevelopmental sequelae and is associated with a significant risk of primary central nervous system melanoma and death.

The diagnostic dilemma of primary central nervous system melanoma

Melanomas are malignant neoplasms of melanocytes developing predominantly in the skin, but occasionally arising from eyes, mucous membranes, and the central nervous system (CNS). The CNS can be affected by a spectrum of melanocytic lesions ranging from diffuse neurocutaneous melanosis, to a focal and benign neoplasm (melanocytoma), and to an overtly malignant tumor (melanoma). Primary melanocytic lesions involving the CNS are typically concentrated in the perimedullary and high cervical region. Primary CNS melanoma cannot be reliably distinguished from metastatic melanoma on neuroimaging and histopathological characteristics alone: its diagnosis is established only after exclusion of secondary metastatic disease from a cutaneous, mucosal or retinal primary. We present two patients with primary CNS melanoma and discuss relevant issues, available treatment options, and expected outcomes. Awareness of disease spectrum and clinico-biological differences may be used to guide therapeutic decision-making for a patient with a proven or suspected primary CNS melanoma.

Primary melanoma of the central nervous system. Clinical-pathological report of a case, with survey and discussion of the literature.

Primary melanoma of the central nervous system (CNS) was not known until 1859, when Virchow 1 described the first case of diffuse melanosarcomatosis of the central nervous system. In a thorough search through the American and European literature we found only 45 cases of primary involvement of the CNS (Table 1). None of these cases had been diagnosed without surgical intervention or autopsy because there are no characteristic signs or symptoms. It seems justified, therefore, to report one more case, that of a primary intramedullary melanoma of the cord with the unique finding of metastases to the brain. The original diagnosis was acute transverse myelitis. The histogenesis of primary CNS melanoma is an interesting, as-yet-unsolved problem. Various theories have been proposed and will be discussed briefly. Report of Case The patient, a woman 50 years old, was admitted to the neurological service of Lenox Hill Hospital on May 20, 1959,