Introdução
O que você precisa saber de cara
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.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
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Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
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🇧🇷 Atendimento SUS — Melanocitoma meníngeo
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Pesquisa e ensaios clínicos
10 ensaios clínicos encontrados.
Publicações mais relevantes
Meningeal Melanocytoma of the Lumbar Spinal Region: A Unique and Unusual Presentation.
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.
Cerebellopontine Angle Meningeal Melanocytoma: A Rare Case and Literature Review of Outcomes and Recurrences.
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.
Aggressive Clinical Course and Malignant Transformation of a Meningeal Melanocytoma of the Pontomedullary Region: Diagnostic and Therapeutic Implications.
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.
Benign or aggressive? Understanding spinal melanocytomas in comparison to malignant melanoma.
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.
Primary Intracranial Meningeal Melanocytoma with Malignant Transformation: A Case Report and Comparison of Early Versus Late Immunotherapy Interventions.
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
Severe cord compression due to unique extradural meningeal melanocytoma of intermediate grade.
Meningeal Melanocytoma of the Lumbar Spinal Region: A Unique and Unusual Presentation.
Aggressive Clinical Course and Malignant Transformation of a Meningeal Melanocytoma of the Pontomedullary Region: Diagnostic and Therapeutic Implications.
Benign or aggressive? Understanding spinal melanocytomas in comparison to malignant melanoma.
Primary Intracranial Meningeal Melanocytoma with Malignant Transformation: A Case Report and Comparison of Early Versus Late Immunotherapy Interventions.
📚 EuropePMC144 artigos no totalmostrando 72
Meningeal Melanocytoma of the Lumbar Spinal Region: A Unique and Unusual Presentation.
International journal of surgical pathologyAggressive Clinical Course and Malignant Transformation of a Meningeal Melanocytoma of the Pontomedullary Region: Diagnostic and Therapeutic Implications.
Journal of neurological surgery reportsBenign or aggressive? Understanding spinal melanocytomas in comparison to malignant melanoma.
Journal of neuro-oncologyPrimary Intracranial Meningeal Melanocytoma with Malignant Transformation: A Case Report and Comparison of Early Versus Late Immunotherapy Interventions.
Current oncology (Toronto, Ont.)A T1-isointense meningeal melanocytoma mimicking meningioma: Case report and literature review.
Radiology case reportsCerebellopontine Angle Meningeal Melanocytoma: A Rare Case and Literature Review of Outcomes and Recurrences.
Neurology IndiaMeningeal melanocytoma of the central nervous system in children.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryPrimary 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).
CancersIntracranial meningeal melanocytoma: a case report and literature review.
Journal of surgical case reportsThe Role of Radiotherapy for Meningeal Melanocytomas - A 20 Year Update.
In vivo (Athens, Greece)A rare case of multifocal craniospinal leptomeningeal melanocytoma: A case report and scoping review.
Brain & spineInvasive cellular blue nevus in the cervical spine: A case report.
MedicineSupratentorial meningeal melanocytoma mimicking meningioma: case report and literature review.
Pathology oncology research : PORMalignant Transformation and Leptomeningeal Melanomatosis in a Primary Meningeal Melanocytoma: A Case Report and Review of Literature.
NMC case report journalIntermediate grade meningeal melanocytoma of the posterior fossa, GNAQ mutation-positive.
Acta neurologica BelgicaPrimary cervical meningeal melanocytoma with a dumbbell shape: Case report and review of the literature.
MedicineIntradural extramedullary meningeal melanocytoma: a case report and literature review.
Journal of surgical case reportsHow Should We Treat Meningeal Melanocytoma? A Retrospective Analysis of Potential Treatment Strategies.
CancersMeningeal Melanomatosis with a Spinal Meningeal Melanocytoma Trigger by an in vitro Fertilization.
Neurology IndiaMeningeal Melanocytoma- A Clinicohistological Masquerader.
Neurology IndiaIntracranial Meningeal Melanocytomas: Clinicoradiologic Characteristics and Outcomes.
World neurosurgeryClinical Reasoning: A 67-Year-Old Woman With Abdominal Pain, Constipation, and Urinary Retention.
NeurologyMalignant Transformation and Metastatic Spread of Dumbbell-Shaped Meningeal Melanocytoma of the Cervical Spine: A Case Report and Literature Review.
Frontiers in surgeryIntermediate-grade primary melanocytic neoplasm of the cerebellopontine angle: A diagnostic challenge.
Ear, nose, & throat journalPrimary Meningeal Melanocytoma Located in the Craniovertebral Junction: A Case Report and Literature Review.
NMC case report journalGNA11 Mutation in an Intracranial Melanocytoma with Orbital Involvement and Nevus of Ota.
Ophthalmic plastic and reconstructive surgerySuprasellar Melanocytoma with Leptomeningeal Seeding: An Aggressive Clinical Course for a Histologically Benign Tumor.
Case reports in radiologyIntermediate-Grade Meningeal Melanocytoma in a 19-Month- Old Child with Difficulties in Differential Diagnosis and Management.
Turkish neurosurgeryS-100-negative, GNA11 mutation-positive intramedullary meningeal melanocytoma of the thoracic spine: A radiographic challenge and histologic anomaly.
Surgical neurology internationalSpinal Nerve Root Extradural Melanocytoma Progressing to Malignant Melanoma: A Case Report with Review of Literature.
Asian journal of neurosurgeryRecurrent Meningeal Melanocytoma of Cervical Spine: A Rare Case.
Asian journal of neurosurgeryA challenging case of sporadic melanocytoma of the jugular foramen.
Neuro-ChirurgieA National Cancer Database analysis of the patterns of care for meningeal melanocytoma.
Future oncology (London, England)Non-dysraphic extramedullary intradural spinal lipoma with neurocutaneous melanocytosis.
British journal of neurosurgeryNivolumab as adjuvant treatment for a spinal melanocytoma: A case report.
MedicineRecurrent spinal meningeal melanocytoma at lumbar spine level: a case report.
British journal of neurosurgeryDefinitive radiotherapy for meningeal brainstem melanocytoma: a case report.
British journal of neurosurgeryParietal Convexity Meningeal Melanocytoma: Radiologic-Pathologic Correlation.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesMeningeal Melanocytoma Occurring at Epidural Region of the Cervical Spine.
Spine surgery and related researchIdentification of a Novel BRCA1 Alteration in Recurrent Melanocytoma Resulting in Increased Proliferation.
Journal of neuropathology and experimental neurologyC6-T1 Intradural Extramedullary Ventral Meningeal Melanocytoma Resected Via Anterior Corpectomy with Reconstruction.
World neurosurgeryPrimary pigmented meningeal melanocytoma originating in Meckel cave in a patient with carney complex: A case report.
MedicineA Rapidly Growing Cervical Meningeal Melanocytoma with a Dumbbell-Shaped Extension.
World neurosurgery8-Year Follow-up for Woman with Spinal Meningeal Melanocytoma in S1 Nerve Root: Case Report and Literature Review.
World neurosurgerySurgical Management of Primary Cerebellopontine Angle Melanocytoma: Outcome, Recurrence and Additional Therapeutic Options.
World neurosurgeryUsefulness of Neuromelanin Sensitive MRI for En Plaque Meningeal Melanocytoma Involving the Cavernous Sinus: A Case Report.
NMC case report journalMeningeal Melanocytoma Associated with Nevus of Ota: Analysis of Twelve Reported Cases.
World neurosurgery[Meningeal melanoma arising from a preexisting meningeal melanocytoma: A clinical, pathological and cytogenetic study about one case].
Annales de pathologiePrimary meningeal melanocytoma of the cerebellopontine angle associated with ipsilateral nevus of Ota: A case report.
Surgical neurology international[Meningeal melanocytoma: aggressive evolution of a benign tumor: about 2 cases].
The Pan African medical journalIntracranial meningeal melanocytoma diagnosed using an interdisciplinary approach: a case report and review of the literature.
Journal of medical case reportsSpinal meningeal melanocytoma in a 5-year-old child: a case report and review of literature.
The Egyptian journal of neurology, psychiatry and neurosurgeryPrimary Meningeal Melanocytoma in Sellar Region, Simulating a Nonfunctioning Pituitary Adenoma: Case Report and Literature Review.
World neurosurgerySupratentorial 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[Melanocytoma and meningeal melanocytosis, similar but different lesions].
Cirugia y cirujanosDiagnostic Clue of Meningeal Melanocytoma: Case Report and Review of Literature.
Yonsei medical journalOrbital meningeal melanocytoma: Histological, immunohistochemical and molecular characterization of a case and review of the literature.
Pathology, research and practicePrimary scattered multifocal melanocytomas in spinal canal mimicking neurofibromatosis.
The spine journal : official journal of the North American Spine SocietySpinal meningeal melanocytomas: clinical manifestations, radiological and pathological characteristics, and surgical outcomes.
Journal of neuro-oncologyPrimary meningeal melanocytoma mimicking a nonfunctioning pituitary adenoma.
Clinical neuropathologyCervical Spinal Meningeal Melanocytoma Presenting as Intracranial Superficial Siderosis.
Case reports in radiologySquash cytopathology of primary meningeal melanocytoma.
Neurology IndiaUnusual lesions of the mediastinum.
Lung India : official organ of Indian Chest SocietyA case report on intermediate-grade malignant meningeal melanocytoma with elevated cell proliferation index.
International journal of clinical and experimental pathologyThe GNAQ in the haystack: intramedullary meningeal melanocytoma of intermediate grade at T9-10 in a 58-year-old woman.
Journal of neurosurgerySudden Death and Primary Leptomeningeal Melanocytosis: A Case Report With an Autopsy Diagnosis.
The American journal of forensic medicine and pathologyCerebellar meningeal melanocytoma associated with nevus of Ota: An extremely rare case.
NeurologyMeningeal melanocytoma of the middle cranial fossa (the Meckel's cave).
Neurology IndiaIntermediate-grade meningeal melanocytoma associated with nevus of Ota: a case report and review of the literature.
Melanoma researchPrimary Meningeal Melanocytoma in the Left Temporal Lobe Associated with Nevus Ota: a Case Report and Review of the Literature.
World neurosurgeryPrimary meningeal melanocytoma of the sellar region: review of the literature and differential diagnosis with special reference to angiographical features.
Pituitary[Multifocal meningeal melanocytoma with repeated recurrence: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologyAssociações
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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.
- Meningeal Melanocytoma of the Lumbar Spinal Region: A Unique and Unusual Presentation.
- Cerebellopontine Angle Meningeal Melanocytoma: A Rare Case and Literature Review of Outcomes and Recurrences.
- Aggressive Clinical Course and Malignant Transformation of a Meningeal Melanocytoma of the Pontomedullary Region: Diagnostic and Therapeutic Implications.
- Benign or aggressive? Understanding spinal melanocytomas in comparison to malignant melanoma.
- Primary Intracranial Meningeal Melanocytoma with Malignant Transformation: A Case Report and Comparison of Early Versus Late Immunotherapy Interventions.
- Severe cord compression due to unique extradural meningeal melanocytoma of intermediate grade.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:252046(Orphanet)
- MONDO:0016746(MONDO)
- GARD:20740(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
