Objective: Meningeal melanocytoma generally occurs in the posterior fossa. Orbital manifestation is rarely encountered.
Methods: A thirty-five year-old man presented with progressive proptosis of his right eye. Computed tomography (CT) and Magnetic Resonance Imaging (MRI) of the brain showed an expansive intraconal mass lesion occupying the superior orbital compartment, the entire orbital apex, and the optic foramen. Histological analysis and Immunohistochemical staining for S-100 and HMB-45 monoclonal antibodies confirmed melanocytoma.
Findings: Microsurgical removal was accomplished through a fronto-orbital craniotomy. Chemotherapy and irradiation followed the initial intervention. The patient returned for follow up two years after surgery, complaining of headache and right visual loss. A subfrontal tumor with massive edema was found on follow up CT scan.
Interpretation: Meningeal melanocytomas are rare benign pigmented tumors of the central nervous system. They are predominant in the posterior fossa and spinal cord and frequently mistaken for melanomas, especially on frozen sections. Orbital presentation is rare. The natural history is poorly defined.
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Meningeal melanocytoma of the central nervous system in children.
Childs Nerv Syst
January 2025
Department of Pediatric Neurosurgery, Medical University of Silesia in Katowice, Medykow 16, 40-752, Katowice, Poland.
Purpose: This study aimed to summarize the existing English-language literature on central nervous system (CNS) meningeal melanocytomas in children, and additionally describe our institutional case report.
Methods: PubMed database was screened on September 2, 2024, for English-language papers reporting on pediatric patients with CNS meningeal melanocytoma.
Results: A total of 17 papers reporting on 18 patients with 19 CNS meningeal melanocytomas were found in the literature.
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).
Cancers (Basel)
July 2024
Center for Neurosurgery, Department of General Neurosurgery, University of Cologne, 50923 Cologne, Germany.
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.
Intracranial meningeal melanocytoma: a case report and literature review.
J Surg Case Rep
May 2024
School of Clinical Medicine, Addenbrooke's Hospital, Hills Rd, Cambridge CB20QQ, United Kingdom.
Primary intracranial melanocytoma is an uncommon benign pigmented tumor arising from leptomeningeal melanocytes. Neuroimaging characteristics of central nervous system melanocytoma are distinct from similarly presenting intracranial neoplasms and can aid in diagnosis prior to histopathological examination. In rare cases, there may be more than one lesion present.
View Article and Find Full Text PDFThe Role of Radiotherapy for Meningeal Melanocytomas - A 20 Year Update.
In Vivo
April 2024
Department of Radiation Oncology, Mayo Clinic, Phoenix, AZ, U.S.A.
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.
Primary leptomeningeal melanocytic neoplasms: A clinicopathologic, immunohistochemical, and molecular study of 12 cases.
Hum Pathol
June 2024
Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, 200032, People's Republic of China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 200032, People's Republic of China. Electronic address:
This study investigated the clinicopathological, immunohistochemical, and molecular features of primary leptomeningeal melanocytic neoplasms (LMNs). Twelve LMN cases were retrospectively reviewed. We performed Fluorescence in-situ hybridization (including a 4-probe FISH assay with CDKN2A and MYC assay) and Next-Generation sequencing analyses on available cases.
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