Intracranial meningeal melanocytoma is an uncommon tumor that is considered benign. We formerly reported an intracranial meningeal melanocytoma. Here we report a extremely rare case of malignant transformation of this tumor. A 49-year-old man complained of a headache. Magnetic resonance scanning revealed a mass in the left frontal region. The patient underwent gross total removal of the tomor in 1994. The histological findings showed a meningeal melanocytoma. In 1998, he underwent gamma-knife surgery for local recurrence. An additional operation was performed in 1999 became tumor growth was not stopped. The tumor was partially excised by left frontal craniotomy. Histopathological examination revealed a malignant melanoma originating from a melanocytoma. The tumor was composed of a proliferation of severely atypical melanocytoid cells with slightly irregular nuclei and prominent nucleoli, associated with necrosis and hemorrhage. Mitotic figures were encountered occasionally. After six months, he died from cerebrospinal fluid dissemination of this tumor. To our knowledge, this is the first report of malignant transformation of an intracranial meningeal melanocytoma.
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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
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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.
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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.
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Primary leptomeningeal melanocytic neoplasms: A clinicopathologic, immunohistochemical, and molecular study of 12 cases.
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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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