Overview
Meningeal melanocytoma is a rare, typically benign pigmented tumor arising from melanocytes within the leptomeninges (the membranes surrounding the brain and spinal cord). These melanocytes are normally present in the meninges and are derived from the neural crest during embryonic development. Meningeal melanocytoma is classified as a low-grade (Grade I) melanocytic neoplasm of the central nervous system, distinguishing it from the more aggressive primary meningeal melanoma. The tumor most commonly occurs in the posterior fossa, spinal cord (particularly the thoracic region), and around Meckel's cave, though it can arise at any meningeal site. Symptoms depend on the tumor's location and size and result from compression of adjacent neural structures. Patients may experience headaches, seizures, focal neurological deficits, spinal cord compression symptoms (such as weakness, sensory changes, or bowel and bladder dysfunction), cranial nerve palsies, or signs of increased intracranial pressure. The tumor typically presents as a well-circumscribed, heavily pigmented mass that may be mistaken radiologically for a meningioma. The primary treatment for meningeal melanocytoma is complete surgical resection, which is often curative when achievable. However, complete removal may be difficult depending on the tumor's location and its relationship to critical neural and vascular structures. In cases of incomplete resection, adjuvant radiation therapy may be considered to reduce the risk of recurrence. Although generally benign, meningeal melanocytomas can occasionally recur and, in rare instances, undergo malignant transformation to meningeal melanoma. Long-term follow-up with serial imaging is therefore recommended. There are no established chemotherapy regimens for this tumor, and management decisions are typically made on a case-by-case basis.
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Meningeal melanocytoma.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Meningeal melanocytoma.
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Common questions about Meningeal melanocytoma
What is Meningeal melanocytoma?
Meningeal melanocytoma is a rare, typically benign pigmented tumor arising from melanocytes within the leptomeninges (the membranes surrounding the brain and spinal cord). These melanocytes are normally present in the meninges and are derived from the neural crest during embryonic development. Meningeal melanocytoma is classified as a low-grade (Grade I) melanocytic neoplasm of the central nervous system, distinguishing it from the more aggressive primary meningeal melanoma. The tumor most commonly occurs in the posterior fossa, spinal cord (particularly the thoracic region), and around Meckel
How is Meningeal melanocytoma inherited?
Meningeal melanocytoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Meningeal melanocytoma typically begin?
Typical onset of Meningeal melanocytoma is adult. Age of onset can vary across affected individuals.
Which specialists treat Meningeal melanocytoma?
6 specialists and care centers treating Meningeal melanocytoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.