Recurrent spinal meningeal melanocytoma at lumbar spine level: a case report.

INTRODUCTION: Spinal meningeal melanocytoma is an extremely rare tumour with an estimated annual incidence of 1 per 10 million people. It usually arises from the intradural extramedullary compartment at the cervical levels. Although these tumours are histologically benign, they may behave aggressively. Local recurrence could occur even after total tumour excision. CASE REPORT: We report a case of a 33-year-old Asian male who developed progressive weakness and numbness of the bilateral lower extremities as well as urinary retention five years after complete tumour resection of lumbar spinal meningeal melanocytoma. Magnetic resonance imaging of the lumbar spine revealed a mass with thecal sac compression which was hypointense on T2-weighted images and hyperintense on T1-weighted images. The patient underwent total tumour removal. Histologic examination was compatible with recurrent meningeal melanocytoma. After a 4-week inpatient rehabilitation programme, he was able to ambulate without assistance and to do clean intermittent catheterisation for micturition on a regular basis. DISCUSSION: This is the first reported case of intradural extramedullary meningeal melanocytoma located at the lumbar region. Clinicians should consider the possibility of these rare tumours at any level of the spine, and be aware of sphincter dysfunction in addition to motor and sensory deficits of extremities.

A National Cancer Database analysis of the patterns of care for meningeal melanocytoma.

Aim: To evaluate demographics, treatment patterns, radiotherapy utilization and patient outcomes in meningeal melanocytomas. Materials & methods: The National Cancer Database was queried for meningeal melanocytomas diagnosed in 2002-2016. The effects of demographic, clinical and treatment variables were determined via Kaplan-Meier log-rank and Cox regression analyses. Results: The median and 5-year overall survival were 57.46 months and 48%, respectively. Patients earning ≥$48K showed improved survival (p = 0.0319). Radiotherapy and chemotherapy were utilized in 37.7 and 9% of patients, respectively. Conclusion: Income significantly affected survival. Surgery remains the mainstay approach. Radiotherapy was delivered in more than one-third of patients but did not impact survival. However, further analyses were limited by poor treatment modality information in the database.

Lay abstract Aim: To assess the impact of demographic, clinical and treatment factors on survival in patients with meningeal melanocytomas. Materials & methods: Biostatistical analyses were conducted on patients diagnosed with meningeal melanocytomas between 2002 and 2016 using data from the National Cancer Database. Results: The median and 5-year survival rate were 57.46 months and 48%, respectively. Patients with incomes ≥$48K showed improved survival (p = 0.0319). Radiotherapy and chemotherapy were administered in 37.7 and 9% of patients, respectively. Conclusion: Income was a critical factor in increasing patient survival. Surgery serves as the primary means for treatment, while radiotherapy and chemotherapy remain underutilized.

[Meningeal melanoma arising from a preexisting meningeal melanocytoma: A clinical, pathological and cytogenetic study about one case]. / Transformation d'un mélanocytome méningé en mélanome : étude clinique, histopathologique et cytogénétique.

Meningeal melanocytic tumors are rare. We report an exceptional case of transformation of a meningeal melanocytoma in a malignant melanoma. The course of the disease extents from 61-years to 85-years and ends with the death of the patient. Besides histopathological and immunohistochemical data, we also report the array CGH study of the melanocytoma and melanoma components suggesting the malignant transformation from whole chromosome gains in the melanocytoma to additional segmental aberrations in the malignant melanoma. Beyond the rarity of this tumor subtype, this case report highlights the potential interest of molecular analyses for diagnostic and prognostic purposes in the field of meningeal melanocytic tumors.

The Role of Radiotherapy for Meningeal Melanocytomas - A 20 Year Update.

BACKGROUND/AIM: Meningeal melanocytomas are rare tumors of the central nervous system and optimal treatment needs further clarification. This study compared subtotal resection (STR), STR plus radiation therapy (RT), gross total resection (GTR), and GTR+RT to better define the role of postoperative RT. PATIENTS AND METHODS: All cases reported in the literature were reviewed. Patients (n=184) with complete data were analyzed for local control (LC) and overall survival (OS). RESULTS: On univariate analysis, GTR (vs. STR) was associated with improved LC (p=0.016). When comparing the treatment regimens, best and worst results were found after GTR+RT and STR alone, respectively (p<0.001). On univariate analysis, GTR resulted in better OS than STR (p=0.041). Moreover, the treatment regimen had a significant impact on OS (p=0.049). On multivariate analyses of LC and OS, extent of resection and treatment regimen were found to be significant factors. After STR, RT significantly improved LC but not OS. After GTR, RT did not significantly improve LC or OS. CONCLUSION: GTR was significantly superior to STR regarding LC and OS. STR+RT resulted in significantly better LC when compared to STR alone.

Primary Meningeal Melanocytic Tumors of the Central Nervous System: A Review from the Ultra-Rare Brain Tumors Task Force of the European Network for Rare Cancers (EURACAN).

BACKGROUND: Primary meningeal melanocytic tumors are ultra-rare entities with distinct histological and molecular features compared with other melanocytic or pigmented lesions, such as brain and leptomeningeal metastases from metastatic melanoma. METHODS: The European Network for Rare Cancers (EURACAN) Task Force on Ultra-Rare Brain Tumors (domain 10, subdomain 10) performed a literature review from January 1985 to December 2023 regarding the epidemiologic and clinical characteristics, histological and molecular features, radiological findings, and efficacy of local treatments (surgery and radiotherapy) and systemic treatments for these entities. RESULTS: Molecular analysis can detect specific mutations, including GNAQ, GNA11, SF3B1, EIF1AX, BAP1, that are typically found in circumscribed primary meningeal melanocytic tumors and not in other melanocytic lesions, whereas NRAS and BRAF mutations are typical for diffuse primary meningeal melanocytic tumors. The neuroimaging of the whole neuroaxis suggests a melanocytic nature of a lesion, depicts its circumscribed or diffuse nature, but cannot predict the tumor's aggressiveness. Gross-total resection is the first choice in the case of circumscribed meningeal melanocytoma and melanoma; conversely, meningeal biopsy may be reserved for patients with diffuse and multinodular leptomeningeal spread to achieve a definitive diagnosis. High-dose radiotherapy is rarely indicated in diffuse melanocytic tumors except as palliative treatment to alleviate symptoms. Last, a definitive advantage of a specific systemic treatment could not be concluded, as most of the data available derive from case reports or small cohorts. CONCLUSIONS: As primary meningeal melanocytic tumors are extremely rare, the correlations between the clinical characteristics, molecular profile, radiological findings at diagnosis and progression are weak, and poor evidence on the best therapeutic approach is available. There is a need to develop shared platforms and registries to capture more knowledge regarding these ultra-rare entities.

Malignant Transformation and Leptomeningeal Melanomatosis in a Primary Meningeal Melanocytoma: A Case Report and Review of Literature.

Meningeal melanocytomas of the central nervous system, although typically benign, rarely undergo malignant transformations. A 46-year-old man presented with headache and nausea 4 years after gross total resection of a craniovertebral junction meningeal melanocytoma at another hospital. The initial clinical course was previously reported.1) Computed tomography revealed the presence of multiple intracranial mass lesions. Furthermore, magnetic resonance imaging showed multiple intracranial lesions and meningeal dissemination. A biopsy was performed for a circumflex lesion located in the right frontal lobe. Pathological examination showed anaplastic changes and a Ki-67 index of 33%. Based on the pleomorphic changes and high mitotic activity, the patient was diagnosed with primary cerebral malignant melanoma. The patient received four cycles of nivolumab (80 mg) and ipilimumab (165 mg), followed by whole-brain radiotherapy (37.5 Gy). However, the disease progressed after the third cycle. Genome analysis revealed GNAQ Q209P and SF3B1 R625C mutations, but no treatments related to these gene mutations were available. Despite the seven cycles of nivolumab therapy, the patient eventually passed away 9 months after surgery. This case was a rare example of malignant transformation and leptomeningeal melanomatosis in a meningeal melanocytoma. It highlights the importance of careful follow up after gross total resection. Identification of molecular alterations can lead to better detection of melanocytic melanomas with poor prognosis and high risk of recurrence and metastasis. It can also facilitate the development of novel therapeutic options for these patients.

Intradural extramedullary meningeal melanocytoma: a case report and literature review.

Primary meningeal melanocytomas are extremely rare, benign tumours arising from the leptomeninges. While they are considered to be benign lesions, there is potential for their growth and transformation into malignant melanomas. They are commonly found in the cervical spine, with a decreased incidence in the thoracic and lumbar regions. We present a case report of a 56-year-old man who presented to our unit with a 4-month history of lower limb weakness and a sensory level at T6. Magnetic resonance imaging shows an intradural extramedullary tumour. The patient underwent a thoracic debulking of the lesion with neurophysiological monitoring. Histopathology confirmed the diagnosis of melanocytoma of meningeal origin, with a low mitotic count. Our patient recovered well post-operatively with no complications. Surgical resection is an effective method to manage this tumour; however, adjuvant radiotherapy is advised due to the risk of recurrence and malignant transformation.

Malignant Transformation and Metastatic Spread of Dumbbell-Shaped Meningeal Melanocytoma of the Cervical Spine: A Case Report and Literature Review.

Background: Meningeal melanocytoma is a rare disease that originates from leptomeningeal melanocytes in the central nervous system. Meningeal melanocytoma is generally considered benign, and has a good prognosis following complete surgical resection. Reports of the malignant transformation and spread of these tumors are scarce. Case Presentation: A 19 year old female presented with headache, progressive limb weakness, and dyspnea. Magnetic resonance imaging showed a dumbbell-shaped lesion at C1-C2 that was hyperintense on T1 weighted images and showed strong contrast enhancement. Total resection was achieved using a posterior midline approach. Post-operative pathology showed meningeal melanocytoma. The tumor recurred 9 months later with intracranial spread. Resection of the lesion revealed malignant transformation to meningeal melanoma. Conclusion: Meningeal melanocytoma harbors malignant potential even with total resection. Radiotherapy could be considered to prevent disease recurrence and progression.

Meningeal Melanomatosis with a Spinal Meningeal Melanocytoma Trigger by an in vitro Fertilization.

Meningeal melanomatosis is an infrequent tumor originating from the melanocytes in the leptomeninges and one of the recognized primary melanocytic tumors of the central nervous system. The average survival has known to be about 5 months. It can be associated with solid tumors, such as meningeal melanocytomas. The patient we present was diagnosed of a meningeal melanomatosis that developed two solid tumors related to an in vitro fertilization. The clinical course was rapidly fatal. Although the use of comprehensive diagnostic procedures, usually the final diagnosis of primary diffuse meningeal melanomatosis is postmortem, it would be advisable for the appropriate management of the patient to make a differential diagnosis and to be aware of the behavior of the tumor.

How Should We Treat Meningeal Melanocytoma? A Retrospective Analysis of Potential Treatment Strategies.

BACKGROUND: Meningeal melanocytomas (MM) are rare primary melanocytic tumors of the leptomeninges with an incidence of 1:10,000,000. Until now, there has been only sparse information about this tumor entity. Here, we provide a meta-analysis of all cases published in the English language since 1972. METHODS: A literature review was performed using PubMed and Web of Science. All published cases were evaluated for location, sex, age, therapeutic approach, and outcome. In total, we included 201 patient cases in our meta-analysis. RESULTS: The majority of MM was diagnosed more frequently in men between the third and fifth decade of life. Surgery is the preferred therapeutic approach, and total resection is associated with the best outcome. Patients with partial resection or tumor recurrence benefit from adjuvant radiotherapy, whereas chemo- or immunotherapies do not improve the disease course. Malignant transformation was described in 18 patients. Of these, 11 patients developed metastasis. CONCLUSIONS: We present the first retrospective meta-analysis of all MM cases published in the English language, including an evaluation of different treatment strategies allowing us to suggest a novel treatment guideline highlighting the importance of total resection for recurrence-free survival and characterizing those cases which benefit from adjuvant radiotherapy.

Intracranial Meningeal Melanocytomas: Clinicoradiologic Characteristics and Outcomes.

OBJECTIVE: Melanocytes are neural crest derivatives. Intracranial meningeal melanocytomas (MM) are rare tumors and the available literature is sparse for these neoplasms. This review aims to provide a detailed and comprehensive literature review of these tumors. METHODS: By searching online databases, a literature review was performed to include and analyze all cases of cranial MM reported to date. PRISMA guidelines were followed for the review process. RESULTS: A total of 109 cases were analyzed. The male/female ratio was 1.2:1. The mean age was 40.3 years. The mean duration of symptoms was 23.9 months (range, 2 days-15 years). Cerebellopontine (CP) angle, suprasellar, and Meckel cave were the most common locations. Sixty-four patients (58%) underwent gross total resection (GTR). Twenty-eight patients (26%) received some form of adjuvant radiation. There were 29 recurrences (26%) and mean time to recurrence was 50.2 months. The recurrence rates (RRs) for GTR and subtotal resection were 20% and 42%, respectively. The extent of resection (subtotal resection) and tumor locations (CP angle and Meckel cave) were significantly associated with higher RR. Six patients (6.5%) had higher-grade transformations. CONCLUSIONS: Complete surgical resection is the ideal treatment and adjuvant radiation is to be considered for residual/recurrent tumors. Adjuvant radiotherapy may also be prescribed despite GTR, in locations with higher RR such as CP angle and Meckel cave. Because of higher-grade transformations and delayed recurrences, long-term follow-up is required.

Spinal Nerve Root Extradural Melanocytoma Progressing to Malignant Melanoma: A Case Report with Review of Literature.

Melanocytomas are rare benign pigmented tumors arising from the leptomeninges with a very remote chance of progressing to malignant melanoma. They have a predilection for occurring in the posterior fossa or in the intradural extramedullary region of the cervical spine. We report the first case of malignant transformation of a nerve root (extradural) melanocytoma wherein immunotherapy has been added for its treatment. Only four such cases of malignant transformation of central nervous system melanocytoma have been reported in the literature. Definite diagnosis in such cases is based on immunohistochemistry evaluation. Surgical resection with adjuvant radiotherapy and immunotherapy is the recommended treatment.

Primary Meningeal Melanocytoma Located in the Craniovertebral Junction: A Case Report and Literature Review.

Primary meningeal melanocytoma is a rare benign tumor in the central nervous system (CNS), comprising less than 0.1% of all intracranial tumors. A 44-year-old man presented with occipital headache, nausea, and vomiting. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a well-defined intradural extramedullary mass lesion at the craniovertebral junction (CVJ). Gross total removal was achieved, and the patient improved symptomatically. The pathologic findings were consistent with meningeal melanocytoma. No tumor recurrence was seen on follow-up MRI two years after surgery. Cases of primary meningeal melanocytoma located at the CVJ are rare. The preoperative differential diagnosis of meningeal melanocytoma from meningioma is sometimes difficult because of their similar appearance on CT and MRI. Complete surgical removal is curative for most cases. We present a case of gross total removal of a meningeal melanocytoma located in the CVJ with references to the literature.

C6-T1 Intradural Extramedullary Ventral Meningeal Melanocytoma Resected Via Anterior Corpectomy with Reconstruction.

BACKGROUND: Melanocytic lesions of the nervous system are thought to arise from leptomeningeal melanocytes, which are derived from neural crest and include diffuse melanocytosis, melanocytomas, and malignant melanomas. Meningeal melanocytomas are extremely rare benign lesions. The usual treatment of intradural extramedullary melanocytomas involves surgical removal through a posterior approach using a laminectomy or laminotomy. CASE DESCRIPTION: We present a 30-year-old female harboring a C6-T1 ventrally located intradural extramedullary lesion compressing the cord anteriorly. The lesion was totally resected via an anterior approach with oblique corpectomy even if the usual treatment involves surgical removal through a posterior approach using a laminectomy or laminotomy. CONCLUSIONS: There is no evidence of recurrence at 4-year follow-up records of the patient. We discuss the surgical approach of these rare lesions.

Usefulness of Neuromelanin Sensitive MRI for En Plaque Meningeal Melanocytoma Involving the Cavernous Sinus: A Case Report.

Intracranial meningeal melanocytoma is a rare tumor. Here, we report a case of pathologically diagnosed en plaque meningeal melanocytoma involving the cavernous sinus along with a review of the pertinent literature. A 35-year-old female presented with progressing left oculomotor nerve palsy and melanosis oculi. Radiological examinations revealed a lesion spreading in an en plaque fashion and involving the left cavernous sinus, which was hyperintense on T1-weighted magnetic resonance imaging (MRI), and hypointense on T2-weighted MRI. The lesion was partially excised following a histopathological diagnosis of meningeal melanocytoma. For follow-up of the residual lesion, neuromelanin sensitive MRI was introduced, and it provided better contrast between the lesion and surrounding intracranial normal tissue than conventional T1-weighted MRI with or without gadolinium. The lesion remained stable without any growth for 3 years post-surgery. Neuromelanin sensitive MRI may be the method of choice for the follow-up of meningeal melanocytoma.

Meningeal Melanocytoma Associated with Nevus of Ota: Analysis of Twelve Reported Cases.

BACKGROUND: Primary melanocytic neoplasms (PMNs) are rare neoplasms, especially within the central nervous system. Meningeal melanocytomas, a subtype of PMN, are even rarer. Nevus of Ota results from the incomplete migration of melanocytes from the neural crest. Synchronous nevus of Ota and meningeal melanocytoma are infrequently encountered in clinical practice. OBJECTIVE: To evaluate and elucidate 12 cases of synchronous meningeal melanocytoma and nevus of Ota, thereby improving the understanding of the relationship between these 2 diseases. METHODS: We reviewed cases and searched the English-language literature from the PubMed database and collected clinical parameters of 12 cases of synchronously occurring nevus of Ota and meningeal melanocytoma. RESULTS: Among the 12 cases, 90.90% and 91.66% of the lesions were located ipsilaterally and supratentorially, respectively. CONCLUSIONS: Our findings indicated a trend for both types of lesion to be located ipsilaterally and supratentorially. When a patient with nevus of Ota is found to harbor an intracranial neoplasm, the most likely diagnosis is PMN.

8-Year Follow-up for Woman with Spinal Meningeal Melanocytoma in S1 Nerve Root: Case Report and Literature Review.

BACKGROUND: Primary melanocytic neoplasms in the central nervous system are rare lesions arising from leptomeningeal melanocytes. These lesions produce neural deficits that resemble those of a meningioma or a schwannoma radiologically. CASE DESCRIPTION: A tumor around the left S1 root with an extension into the left paraspinal compartment was identified in a 32-year-old female with persistent left leg pain for 6 months. The tumor was hyperintense on T1-weighted image and hypointense on T2-weighted image with a homogeneous enhancement. The clinical features, radiologic presentations, treatment choice, and pathologic characteristic were illustrated. The treatment outcome was compared with those reported in the previous literature. The tumor was en-bloc resected with the S1 nerve root reserved. Grossly, the tumor was a soft, capsulated, well-circumscribed, black pigmented lesion. Immunohistochemistry revealed that the tumor cells were positive for HMB-45, S-100 protein, and vimentin. The patient's symptoms were greatly relieved postoperatively. No signs of local recurrence were observed. CONCLUSIONS: Spinal meningeal melanocytoma inside the nerve root is rare and benign. It is difficult to diagnose and often misdiagnosed as schwannoma or meningioma. HMB-45 has been suggested as a significant marker for the diagnosis of meningeal melanocytoma. Complete surgical resection is recommended as the primary treatment. Radiotherapy, chemotherapy, and other treatments can be selected as adjuvant therapies, but their effects are controversial. The recurrence and metastasis rates also remain unclear.

Surgical Management of Primary Cerebellopontine Angle Melanocytoma: Outcome, Recurrence and Additional Therapeutic Options.

OBJECTIVE: Meningeal melanocytomas of the central nervous system are extremely rare, with an incidence of 1 per 10 million individuals. Cases of primary cerebellopontine angle melanocytoma (PCPAM) have only been described in single case reports. The goal of the present study was to analyze the surgical management of PCPAM, with a particular focus on early and late treatment outcomes and recurrence rates. METHODS: The patients who had undergone surgery for PCPAM from January 2004 to May 2018 were identified by a local database query. The patients were evaluated for initial symptoms, pre- and postoperative facial and cochlear nerve function, complications, and recurrence rate by reviewing the patients' medical records. RESULTS: We identified 4 patients with PCPAM of >1500 cerebellopontine angle lesions (∼0.2%) that had been surgically treated at our department in the past 14 years. Of the 4 patients, 2 were men and 2 were women, with a mean age of 47 years. Anatomical facial and cochlear nerve preservation was achieved in all 4 patients. One patient experienced a new moderate facial palsy immediately after surgery (House-Brackmann grade III). Of the 4 patients, 3 had undergone radiotherapy and 1 had undergone ion beam therapy for tumor recurrence (6 years after surgery). Of the 4 patients, 3 had presented with tumor recurrence at 2, 3, and 6 years of follow-up respectively. The long-term follow-up examination had not yet been conducted for 1 patient. CONCLUSIONS: At long-term follow-up, 3 patients had developed recurrence. Because of the high recurrence rate of PCPAM, we believe that radiotherapy in addition to surgery should be considered in the future to avoid early recurrence.

[Meningeal melanocytoma: aggressive evolution of a benign tumor: about 2 cases]. / Mélanocytome méningé: évolution agressive d'une tumeur bénigne: à propos de 2 cas.

Meningeal melanocytomas are rare pigmented tumors affecting the central nervous system and developing in the cerebrospinal leptomeninges. We report two cases of meningeal melanocytomas showing very marked disparity in their evolution: a very long-term development of meningocerebral lesion, with malignant transformation resulting in the death of the first patient after 32 years and intramedullary ectopic location with very fast massive meningeal diffusion in the second patient. These two cases show the uncertain evolutive profile of meningeal melanocytomas. These lesions may become aggressive with poor prognosis despite an intensive therapeutic strategy.

Diagnostic Clue of Meningeal Melanocytoma: Case Report and Review of Literature.

In this report, the patient was pre-diagnosed as meningioma before surgery, which turned out to be meningeal melanocytoma. Hence, we will discuss the interpretation of imaging and neurological statuses that may help avoid this problem. A 45-year-old man had increasing pain around the neck 14 months prior to admission. His cervical spine MR imaging revealed a space-occupying, contrast-enhancing mass within the dura at the level of C1. The neurologic examination revealed that the patient had left-sided lower extremity weakness of 4+, decreased sensation on the right side, and hyperreflexia in both legs. Department of Neuroradiology interpreted CT and MR imaging as meningiom. The patient underwent decompression and removal of the mass. We confirmed diagnosis as meningeal melanocytoma through pathologic findings. Afterwards, we reviewed the patient's imaging work-up, which showed typical findings of meningeal melanocytoma. However, it was mistaken as meningioma, since the disease is rare.