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Melanocitoma meníngeo
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Introdução

O que você precisa saber de cara

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Um melanocitoma é um tumor pigmentado raro que tem sido descrito como uma variante do nevo melanocítico e é derivado da crista neural. O termo "melanocitoma" foi introduzido por Limas e Tio em 1972.

Publicações científicas
184 artigos
Último publicado: 2026
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Anos de pesquisa1desde 2026
Total histórico184PubMed
Últimos 10 anos72publicações
Pico20159 papers
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2026Hoje · 2026🧪 1994Primeiro ensaio clínico📈 2015Ano de pico
Publicações por ano (últimos 10 anos)

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Publicações mais relevantes

Timeline de publicações
73 papers (10 anos)
#1

Meningeal Melanocytoma of the Lumbar Spinal Region: A Unique and Unusual Presentation.

International journal of surgical pathology2026 Jan 23

Meningeal melanocytoma of the central nervous system is a rare primary melanocytic neoplasm arising from the leptomeninges' melanocytes. It chiefly occurs in the posterior cranial fossa and rarely involves the spinal cord. The literature is bereft of lumbar spinal involvement by meningeal melanocytomas. Herein, we present a 17-year-old male patient who was incidentally diagnosed with a lumbar spinal meningeal melanocytoma during an imaging workup for backache following a fall. It was an extramedullary intradural tumor and was amenable to complete surgical removal in the spinal region. This case report highlights the importance of keeping the possibility of meningeal melanocytoma as a differential diagnosis of circumscribed intradural extramedullary neoplasms.

#2

Cerebellopontine Angle Meningeal Melanocytoma: A Rare Case and Literature Review of Outcomes and Recurrences.

Neurology India2025 Jul 01

Melanocytes are neural crest derived cells. Intracranial meningeal melanocytomas (MM) are rare tumors that were first described in 1972. Authors report a rare case of a cerebellopontine (CP) angle MM. In addition, a literature review of CP angle MM is provided and compared with other cranial MMs in terms of recurrence and outcomes. A 45 year old male presented with tinnitus and occipital headache. He had mild sensorineural hearing loss. MRI showed an extra axial CP angle lesion that was hyperintense on T1 and hypointense on T2, with blooming on SWI sequences. A retrosigmoid craniotomy and near total resection was performed. Postoperatively, he developed paresis of the facial and lower cranial nerves, hemiparesis, developed pulmonary complications and died 3 months later. A total of 20 cases of CP angle MM were retrieved and analysed from literature. We noted significant differences between the overall cranial MM and CP angle MM in terms of recurrence and malignant transformation. The recurrence rate was 60%, the higher grade transformation rate was 10%, and mortality was 18%, which was higher than that of cranial MM in general. Authors believe that upfront adjuvant radiotherapy may possibly reduce the recurrence rates in this location.

#3

Aggressive Clinical Course and Malignant Transformation of a Meningeal Melanocytoma of the Pontomedullary Region: Diagnostic and Therapeutic Implications.

Journal of neurological surgery reports2025 Oct

Primary melanocytic tumors of the central nervous system (CNS) are rare neoplasms that range from benign melanocytomas to aggressive malignant melanomas. Although meningeal melanocytomas are generally considered indolent lesions, malignant transformation and distant metastasis can occur. We report the case of a 33-year-old male with a bulbopontine meningeal melanocytoma who developed systemic metastases, culminating in a fatal outcome. Despite initial histopathologic features of benignity and absence of BRAF mutation, the lesion showed aggressive behavior. This case underscores the diagnostic pitfalls associated with primary CNS melanocytic tumors and highlights the importance of long-term vigilance, even for histologically benign lesions.

#4

Benign or aggressive? Understanding spinal melanocytomas in comparison to malignant melanoma.

Journal of neuro-oncology2025 Dec 01

Spinal melanocytic tumors are rare, with limited data on their clinical course and aggressiveness. Since intraoperative dark pigmentation and infiltrative margins can lead to misclassification of MC as MM-especially in the absence of a known primary-this multicenter study characterizes melanocytomas (MC) and contrasts them with malignant melanoma (MM). We retrospectively analyzed 56 patients with spinal intradural melanocytic tumors (2010-2024) from seven European neurosurgical centers. Clinical, radiological, histological, and surgical features were analyzed. Univariate and multivariate analyses were performed to identify prognostic risk factors. The study included 22 patients with spinal MC and 34 patients with MM. Median age was comparable (61 vs. 58 years, p = 0.09), but MC patients had a longer symptom history (13 vs. 1.3 months, p = 0.0001) and more often intramedullary tumors (72.7% vs. 2.9%, p < 0.0001). Gross total resection was achieved in 40.9% of MC cases and 61.8% of MM cases (p = 0.07), with instrumentation required more often for MM (p = 0.0022). Ki-67 proliferation index was significantly higher in MM than MC (26.7% vs. 6.6%, p < 0.0001). Postoperative deficits were more frequent in MC (54.6% vs. 14.7%, p = 0.002) and MCs showed higher frequency of ataxia postoperatively (36.4% vs. 11.8%, p = 0.04). MM patients received more adjuvant therapy (88.2% vs. 31.8%, p = 0.0001), with a trend toward higher local recurrence (26.5% vs. 14.7%, p = 0.31). Our study demonstrates that MC, though considered benign, carries a high risk of postoperative deficits. Furthermore, GTR was achieved in only 40% of cases, underscoring its locally infiltrative nature and potential for semi-benign biology.

#5

Primary Intracranial Meningeal Melanocytoma with Malignant Transformation: A Case Report and Comparison of Early Versus Late Immunotherapy Interventions.

Current oncology (Toronto, Ont.)2025 Oct 24

Primary meningeal melanocytoma is an uncommon, pigmented neoplasm that rarely undergoes malignant transformation, and therapeutic guidelines remain undefined. We report a 43-year-old woman who initially presented with a sudden headache and a right temporal intraparenchymal mass. Subtotal resection revealed a melanocytoma (WHO grade I); residual tumor was treated with Gamma Knife. About 15 months later, she deteriorated rapidly due to malignant transformation with cerebral hemorrhage and spinal leptomeningeal metastasis. Pembrolizumab was initiated within four weeks of the malignant diagnosis and produced transient neurological improvement. Due to symptomatic progression, ipilimumab plus nivolumab was commenced and achieved temporary radiographic stabilization, but the patient succumbed to diffuse progression later. Including this case, only five intracranial melanocytomas with malignant transformation treated with immune checkpoint inhibitors have been reported. Our experience supports initiating immunotherapy promptly after malignant transformation and suggests that sequential dual-agent blockade may modestly extend disease control.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC144 artigos no totalmostrando 72

2026

Meningeal Melanocytoma of the Lumbar Spinal Region: A Unique and Unusual Presentation.

International journal of surgical pathology
2025

Aggressive Clinical Course and Malignant Transformation of a Meningeal Melanocytoma of the Pontomedullary Region: Diagnostic and Therapeutic Implications.

Journal of neurological surgery reports
2025

Benign or aggressive? Understanding spinal melanocytomas in comparison to malignant melanoma.

Journal of neuro-oncology
2025

Primary Intracranial Meningeal Melanocytoma with Malignant Transformation: A Case Report and Comparison of Early Versus Late Immunotherapy Interventions.

Current oncology (Toronto, Ont.)
2025

A T1-isointense meningeal melanocytoma mimicking meningioma: Case report and literature review.

Radiology case reports
2025

Cerebellopontine Angle Meningeal Melanocytoma: A Rare Case and Literature Review of Outcomes and Recurrences.

Neurology India
2025

Meningeal melanocytoma of the central nervous system in children.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2024

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
2024

Intracranial meningeal melanocytoma: a case report and literature review.

Journal of surgical case reports
2024

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

In vivo (Athens, Greece)
2024

A rare case of multifocal craniospinal leptomeningeal melanocytoma: A case report and scoping review.

Brain &amp; spine
2024

Invasive cellular blue nevus in the cervical spine: A case report.

Medicine
2023

Supratentorial meningeal melanocytoma mimicking meningioma: case report and literature review.

Pathology oncology research : POR
2023

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

NMC case report journal
2024

Intermediate grade meningeal melanocytoma of the posterior fossa, GNAQ mutation-positive.

Acta neurologica Belgica
2023

Primary cervical meningeal melanocytoma with a dumbbell shape: Case report and review of the literature.

Medicine
2023

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

Journal of surgical case reports
2022

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

Cancers
2022

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

Neurology India
2022

Meningeal Melanocytoma- A Clinicohistological Masquerader.

Neurology India
2022

Intracranial Meningeal Melanocytomas: Clinicoradiologic Characteristics and Outcomes.

World neurosurgery
2022

Clinical Reasoning: A 67-Year-Old Woman With Abdominal Pain, Constipation, and Urinary Retention.

Neurology
2022

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

Frontiers in surgery
2024

Intermediate-grade primary melanocytic neoplasm of the cerebellopontine angle: A diagnostic challenge.

Ear, nose, &amp; throat journal
2021

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

NMC case report journal
2022

GNA11 Mutation in an Intracranial Melanocytoma with Orbital Involvement and Nevus of Ota.

Ophthalmic plastic and reconstructive surgery
2021

Suprasellar Melanocytoma with Leptomeningeal Seeding: An Aggressive Clinical Course for a Histologically Benign Tumor.

Case reports in radiology
2022

Intermediate-Grade Meningeal Melanocytoma in a 19-Month- Old Child with Difficulties in Differential Diagnosis and Management.

Turkish neurosurgery
2021

S-100-negative, GNA11 mutation-positive intramedullary meningeal melanocytoma of the thoracic spine: A radiographic challenge and histologic anomaly.

Surgical neurology international
2021

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

Asian journal of neurosurgery
2021

Recurrent Meningeal Melanocytoma of Cervical Spine: A Rare Case.

Asian journal of neurosurgery
2022

A challenging case of sporadic melanocytoma of the jugular foramen.

Neuro-Chirurgie
2021

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

Future oncology (London, England)
2023

Non-dysraphic extramedullary intradural spinal lipoma with neurocutaneous melanocytosis.

British journal of neurosurgery
2021

Nivolumab as adjuvant treatment for a spinal melanocytoma: A case report.

Medicine
2023

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

British journal of neurosurgery
2023

Definitive radiotherapy for meningeal brainstem melanocytoma: a case report.

British journal of neurosurgery
2021

Parietal Convexity Meningeal Melanocytoma: Radiologic-Pathologic Correlation.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2020

Meningeal Melanocytoma Occurring at Epidural Region of the Cervical Spine.

Spine surgery and related research
2020

Identification of a Novel BRCA1 Alteration in Recurrent Melanocytoma Resulting in Increased Proliferation.

Journal of neuropathology and experimental neurology
2020

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

World neurosurgery
2020

Primary pigmented meningeal melanocytoma originating in Meckel cave in a patient with carney complex: A case report.

Medicine
2020

A Rapidly Growing Cervical Meningeal Melanocytoma with a Dumbbell-Shaped Extension.

World neurosurgery
2019

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

World neurosurgery
2019

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

World neurosurgery
2019

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

NMC case report journal
2019

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

World neurosurgery
2019

[Meningeal melanoma arising from a preexisting meningeal melanocytoma: A clinical, pathological and cytogenetic study about one case].

Annales de pathologie
2018

Primary meningeal melanocytoma of the cerebellopontine angle associated with ipsilateral nevus of Ota: A case report.

Surgical neurology international
2018

[Meningeal melanocytoma: aggressive evolution of a benign tumor: about 2 cases].

The Pan African medical journal
2018

Intracranial meningeal melanocytoma diagnosed using an interdisciplinary approach: a case report and review of the literature.

Journal of medical case reports
2018

Spinal meningeal melanocytoma in a 5-year-old child: a case report and review of literature.

The Egyptian journal of neurology, psychiatry and neurosurgery
2018

Primary Meningeal Melanocytoma in Sellar Region, Simulating a Nonfunctioning Pituitary Adenoma: Case Report and Literature Review.

World neurosurgery
2017

Supratentorial intermediate grade meningeal melanocytoma with intratumoral bleed in the background of neurocutaneous melanosis: Report of an unusual case and review of literature.

Asian journal of neurosurgery
2017

[Melanocytoma and meningeal melanocytosis, similar but different lesions].

Cirugia y cirujanos
2017

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

Yonsei medical journal
2016

Orbital meningeal melanocytoma: Histological, immunohistochemical and molecular characterization of a case and review of the literature.

Pathology, research and practice
2016

Primary scattered multifocal melanocytomas in spinal canal mimicking neurofibromatosis.

The spine journal : official journal of the North American Spine Society
2016

Spinal meningeal melanocytomas: clinical manifestations, radiological and pathological characteristics, and surgical outcomes.

Journal of neuro-oncology
2016

Primary meningeal melanocytoma mimicking a nonfunctioning pituitary adenoma.

Clinical neuropathology
2015

Cervical Spinal Meningeal Melanocytoma Presenting as Intracranial Superficial Siderosis.

Case reports in radiology
2016

Squash cytopathology of primary meningeal melanocytoma.

Neurology India
2015

Unusual lesions of the mediastinum.

Lung India : official organ of Indian Chest Society
2015

A case report on intermediate-grade malignant meningeal melanocytoma with elevated cell proliferation index.

International journal of clinical and experimental pathology
2016

The GNAQ in the haystack: intramedullary meningeal melanocytoma of intermediate grade at T9-10 in a 58-year-old woman.

Journal of neurosurgery
2015

Sudden Death and Primary Leptomeningeal Melanocytosis: A Case Report With an Autopsy Diagnosis.

The American journal of forensic medicine and pathology
2015

Cerebellar meningeal melanocytoma associated with nevus of Ota: An extremely rare case.

Neurology
2015

Meningeal melanocytoma of the middle cranial fossa (the Meckel's cave).

Neurology India
2015

Intermediate-grade meningeal melanocytoma associated with nevus of Ota: a case report and review of the literature.

Melanoma research
2015

Primary Meningeal Melanocytoma in the Left Temporal Lobe Associated with Nevus Ota: a Case Report and Review of the Literature.

World neurosurgery
2015

Primary meningeal melanocytoma of the sellar region: review of the literature and differential diagnosis with special reference to angiographical features.

Pituitary
2014

[Multifocal meningeal melanocytoma with repeated recurrence: report of a case].

Zhonghua bing li xue za zhi = Chinese journal of pathology
Ver todos os 144 no EuropePMC

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Meningeal Melanocytoma of the Lumbar Spinal Region: A Unique and Unusual Presentation.
    International journal of surgical pathology· 2026· PMID 41574906mais citado
  2. Cerebellopontine Angle Meningeal Melanocytoma: A Rare Case and Literature Review of Outcomes and Recurrences.
    Neurology India· 2025· PMID 40705302mais citado
  3. Aggressive Clinical Course and Malignant Transformation of a Meningeal Melanocytoma of the Pontomedullary Region: Diagnostic and Therapeutic Implications.
    Journal of neurological surgery reports· 2025· PMID 41346450mais citado
  4. Benign or aggressive? Understanding spinal melanocytomas in comparison to malignant melanoma.
    Journal of neuro-oncology· 2025· PMID 41324772mais citado
  5. Primary Intracranial Meningeal Melanocytoma with Malignant Transformation: A Case Report and Comparison of Early Versus Late Immunotherapy Interventions.
    Current oncology (Toronto, Ont.)· 2025· PMID 41294657mais citado
  6. Severe cord compression due to unique extradural meningeal melanocytoma of intermediate grade.
    Surg Neurol Int· 2026· PMID 41952727recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:252046(Orphanet)
  2. MONDO:0016746(MONDO)
  3. GARD:20740(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q18557059(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Melanocitoma meníngeo
Compêndio · Raras BR

Melanocitoma meníngeo

ORPHA:252046 · MONDO:0016746
CID-10
D32.9 · Neoplasia benigna das meninges, não especificada
MedGen
UMLS
C1266113
EuropePMC
Wikidata
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