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Melanoma maligno da conjuntiva
ORPHA:617910CID-10 · C69.0CID-11 · 2D00.0DOENÇA RARA

A seguir, uma lista dos tipos de câncer. O câncer é um grupo de doenças que envolvem aumentos anormais no número de células, com potencial para invadir ou se espalhar para outras partes do corpo. Nem todos os tumores ou nódulos são cancerosos; os tumores benignos não são classificados como câncer porque não se espalham para outras partes do corpo. Existem mais de 100 tipos diferentes de cânceres conhecidos que afetam os seres humanos.

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Introdução

O que você precisa saber de cara

📋

Tumor ocular raro caracterizado por uma massa conjuntival que pode apresentar-se sob a forma plana ou nodular com pigmentação variável. Pode surgir em qualquer parte da conjuntiva, mas mais frequentemente apresenta-se na superfície bulbar, perto do limbo. A invasão do tecido da córnea, margem da pálpebra, esclera ou órbita pode ocorrer em tumores avançados. A disseminação metastática envolve mais frequentemente linfonodos regionais ou pulmão, fígado e cérebro. Os critérios diagnósticos essenciais são a presença de invasão estromal e atipia citológica e arquitectural grave excedendo o expectável num nevo. Os critérios desejáveis são a presença de marcadores melanocíticos por imuno-histoquímica, crescimento pagetoide, atividade mitótica, invasão na córnea ou pele, ulceração e necrose tumoral.

Publicações científicas
113 artigos
Último publicado: 2026 Jan 30

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
32
pacientes catalogados
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C69.0
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico113PubMed
Últimos 10 anos35publicações
Pico20257 papers
Linha do tempo
2026Hoje · 2026🧪 2000Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Nenhum gene associado encontrado

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Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Melanoma maligno da conjuntiva

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Selecione um estado ou use sua localização para ver resultados.

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Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

1 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
35 papers (10 anos)
#1

Metastatic disease in conjunctival malignant melanoma: a retrospective analysis of 167 cases.

BMJ open ophthalmology2026 Jan 30

To analyse the data of a cohort of patients with conjunctival melanoma in order to analyse risk and guarding factors and to investigate the impact on metastatic disease with and without adjuvant therapy. We have retrospectively analysed the impact of clinical aspects and adjuvant therapies after tumour excision or biopsy in 167 patients cared for at the University Hospital Essen and the University Hospital Tübingen, Germany. Clinical as well as histopathological data and therapeutic approaches were analysed with regard to regional (lymphatic) and/or distant (haematogenous) spread during follow-up. The Kaplan-Meier estimate method was used to analyse survival and the Cox regression hazard model to define the probability of metastases depending on different factors. P value of <0.05 was considered statistically significant. 167 cases of malignant conjunctival melanoma were retrospectively analysed. The patients received treatment and were followed up for 78.3±67.5 months. Local tumour recurrence occurred in n=79 patients (47.3%) after a mean of 41.5±70.33 months. 30 patients (37.9%) with a recurrence had not received adjuvant therapy. The overall rate of metastasis was 24.5% (n=41). In n=31 cases, regional lymphatic metastases were found after a follow-up of 48.9±63.5 months; in n=24 cases, distant metastases were found, occurring after 55.5±67.4 months. In n=14, the metastatic disease took both pathways. Ruthenium-106 brachytherapy performed in localised melanoma of the bulbar conjunctiva showed a relevant effect of decreasing the risk for haematogenous metastases by 74% (HR=0.256, p=0.003). 4 out of 54 patients developed distant metastases. In conjunctival melanoma, it is important to perform an adjuvant therapy after excision. This reduces not only local recurrences but also significantly the risk for haematopoietic spread.

#2

Conjunctival Melanoma With Invasion of a LASIK Flap Interface.

JAMA ophthalmology2025 May 01

This case report describes invasion of a laser-assisted in situ keratomileusis (LASIK) flap interface by conjunctival malignant melanoma in a male patient aged 71 years.

#3

Malignant melanoma of the conjunctiva metastatic to the parotid gland: a case report.

Journal of medical case reports2025 Aug 11

Most malignant melanomas occurring in the parotid gland are presumed to be of metastatic origin from cutaneous, sporadically mucosal, or ocular primary sites in the head and neck region. We present an unusual case of a patient with a history of conjunctival malignant melanoma and subsequent metastasis to the parotid gland. A 39-year-old East Slavic male patient presented with a 6-month history of a gradually enlarging, painless swelling of the left preauricular area. The clinical examination disclosed a firm, hardly movable mass over the central part of the left parotid gland with no overlying skin changes. Fine-needle aspiration biopsy revealed a cytological image of a nonepithelial malignant tumor. Collecting a thorough past medical history unveiled that the patient underwent the surgical excision of the ipsilateral conjunctival nevus a few years earlier, with subsequent lesion recurrence and transformation into conjunctival malignant melanoma, treated with a wide local excision and an oral mucosa transplant. No signs of local melanoma recurrence or distant metastases were observed at the time of presentation. Left-sided total parotidectomy with the subsequent ipsilateral neck dissection was performed. Histopathological examination revealed melanoma metastasis to the intraparotid lymph node without extranodal extension. The patient was then qualified for adjuvant immunotherapy and remained disease-free at the 8-month follow-up. In the case of a suspected malignancy within the parotid gland, exhaustive medical history taking and analysis of the patient's previous photographs might direct to the malignant melanoma diagnosis. The high potential for regional and distant metastasis in individuals with conjunctival malignant melanoma underscores the significance of ongoing research to enhance the understanding of its biological behavior and develop a standardized algorithm for its management and surveillance.

#4

Conjunctival malignant melanoma in a young immunocompetent male patient: clinical presentation and management-a case report.

Journal of medical case reports2025 Jul 20

Conjunctival melanoma is a rare yet aggressive ocular surface malignancy, with an incidence of approximately 0.2-0.8 cases per million individuals annually. It predominantly affects older adults, making its occurrence in younger individuals exceedingly rare and diagnostically challenging. We report a 30-year-old Syrian male presenting with a progressively enlarging pigmented lesion on the temporal-inferior conjunctiva of the right eye. The lesion, noted since adolescence, demonstrated significant growth over the past 3 years. Comprehensive clinical assessment and imaging studies confirmed the presence of a well-vascularized, mobile conjunctival lesion, with no evidence of intraocular extension. Surgical excision with 1 mm safety margins was performed, followed by ocular surface reconstruction using a cryopreserved amniotic membrane graft. Histopathological analysis revealed a malignant melanoma with epithelioid and spindle cell morphology and tumor-free margins. Postoperative recovery was uneventful, with full epithelialization by 1 week and no evidence of recurrence at the 1-year follow-up. Timely diagnosis and a multidisciplinary surgical approach are essential for achieving optimal outcomes in conjunctival melanoma, particularly in young patients. Complete excision with negative margins combined with amniotic membrane grafting ensures effective oncologic control and functional ocular surface restoration.

#5

Malignant melanoma of the conjunctiva metastasizing to the submandibular gland: a case report and review of the literature.

BMC ophthalmology2025 Mar 13

Conjunctival malignant melanoma is an aggressive tumor with the potential for metastasis and death. It is rare in the black population because of the protective effect of melanin. Metastases are usually to regional lymph nodes, most commonly the preauricular nodes, deep cervical lymph nodes, and the salivary gland. We described the case of a 41-year-old black African woman who presented to our ophthalmology clinic with a painless, right medial pigmented bulbar conjunctival swelling and right preauricular and submandibular swelling. Two years ago, she had similar swelling at the same location in the right eye. She subsequently underwent surgical excision under general anesthesia. Histology of both tissues showed proliferating malignant melanocytes that showed heavy pigmentation disposed of in solid nests, tongues, and sheets obscuring cellular details in these areas. Immunohistochemistry showed tumor cells to stain for HMB45 and Melan A. This led us to conclude that the tumor was a malignant melanoma. The patient died a year after making the diagnosis. Conjunctival melanoma is a rare ocular malignancy that should be considered in cases of pigmented lesions of the eye, especially in people with dark pigmentation. Primary acquired melanosis with atypia or a nevus are known as preexisting melanocytic lesions from which conjunctival melanomas might develop. Metastases are often to preauricular and cervical lymph nodes as well as to the salivary gland. This demonstrates the significance of early presentation, thorough history taking, physical examination, and surgical excision for histopathology.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC61 artigos no totalmostrando 35

2026

Metastatic disease in conjunctival malignant melanoma: a retrospective analysis of 167 cases.

BMJ open ophthalmology
2025

Malignant melanoma of the conjunctiva metastatic to the parotid gland: a case report.

Journal of medical case reports
2025

Conjunctival malignant melanoma in a young immunocompetent male patient: clinical presentation and management-a case report.

Journal of medical case reports
2025

Conjunctival Melanoma With Invasion of a LASIK Flap Interface.

JAMA ophthalmology
2025

Malignant melanoma of the conjunctiva metastasizing to the submandibular gland: a case report and review of the literature.

BMC ophthalmology
2025

Clinicopathologic Characteristics of 40 Cases of Juvenile Conjunctival Nevus.

The American Journal of dermatopathology
2025

Local Control of Conjunctival Malignant Melanoma by Proton Beam Therapy in a Patient With No Metastasis in Six Years From in Situ to Nodular Lesions.

Journal of medical cases
2024

[Significance of the preferentially expressed antigen in melanoma in discriminating the conjunctival malignant melanoma and pigmented nevus].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2024

Unilateral amelanotic conjunctival malignant melanoma: a case report.

Journal of medical case reports
2024

Isolated conjunctival cavernous hemangioma: a rare case report.

BMC ophthalmology
2025

Case report: Drug-induced vitiligo during treatment with BRAF/MEK inhibitors in a patient with metastatic conjunctival melanoma.

Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners
2022

Progress of Bulbar Conjunctival Microcirculation Alterations in the Diagnosis of Ocular Diseases.

Disease markers
2022

Conjunctival malignant melanoma treated successfully with BRAF inhibitor: encorafenib plus binimetinib.

Dermatology online journal
2022

Proton beam therapy followed by pembrolizumab for giant ocular surface conjunctival malignant melanoma: A case report.

Molecular and clinical oncology
2021

Epidermal Growth Factor Is Increased in Conjunctival Malignant Melanoma.

In vivo (Athens, Greece)
2021

Case of Rapidly Expanding Conjunctival Malignant Melanoma Initially from Primary Acquired Melanosis Diagnosed 14 Years Earlier.

International medical case reports journal
2021

Case of a Giant Conjunctival Melanocytic Nevus.

Ocular oncology and pathology
2020

Intradural Extramedullary Metastatic Conjunctival Malignant Melanoma.

World neurosurgery
2019

A rare case of conjunctival malignant melanoma with orbital invasion.

Nepalese journal of ophthalmology : a biannual peer-reviewed academic journal of the Nepal Ophthalmic Society : NEPJOPH
2019

Expression and clinical significance of p16 and Ki-67 in malignant melanoma of the conjunctiva.

Journal of biological regulators and homeostatic agents
2020

Incidence trends of conjunctival malignant melanoma in Canada.

The British journal of ophthalmology
2019

Checkpoint inhibition immunotherapy for advanced local and systemic conjunctival melanoma: a clinical case series.

Journal for immunotherapy of cancer
2018

Reconstruction of the Lacrimal Excretory System in the Medial Canthal Region.

Plastic and reconstructive surgery. Global open
2018

Conjunctival melanocytic tumors in children - a challenge in diagnosis and treatment.

Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie
2018

Conjunctival melanoma in Southwestern Nigeria: a case series and review of literature.

International ophthalmology
2017

Vascular-Associated Muc4/Vwf Co-Localization in Human Conjunctival Malignant Melanoma Specimens-Tumor Metastasis by Migration?

Current eye research
2017

Can the onset of orbital cancer be the result of a prosthetic eye?

Acta otorhinolaryngologica Italica : organo ufficiale della Societa italiana di otorinolaringologia e chirurgia cervico-facciale
2016

Conjunctival malignant melanoma in Denmark. Epidemiology, treatment and prognosis with special emphasis on tumorigenesis and genetic profile.

Acta ophthalmologica
2016

A case report of a patient with metastatic ocular melanoma who experienced a response to treatment with the BRAF inhibitor vemurafenib.

BMC cancer
2016

Conjunctival malignant melanoma in Denmark: epidemiology, treatment and prognosis with special emphasis on tumorigenesis and genetic profile.

Acta ophthalmologica
2016

Palindromic Rheumatism-Like Migratory Non-Erosive Seronegative Polyarthritis in a Patient With Metastatic Conjunctival Malignant Melanoma.

Archives of rheumatology
2017

Infarcted Tarsal Pyogenic Granuloma Simulating Malignant Melanoma.

Ophthalmic plastic and reconstructive surgery
2015

[Prognosis of primary malignant tumors of the conjunctiva].

Journal francais d'ophtalmologie
2014

Management of conjunctival malignant melanoma: a review and update.

Expert review of ophthalmology
2015

Sentinel lymph node biopsy for conjunctival malignant melanoma: surgical techniques.

Clinical ophthalmology (Auckland, N.Z.)
Ver todos os 61 no EuropePMC

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Doenças relacionadas

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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. Metastatic disease in conjunctival malignant melanoma: a retrospective analysis of 167 cases.
    BMJ open ophthalmology· 2026· PMID 41617257mais citado
  2. Conjunctival Melanoma With Invasion of a LASIK Flap Interface.
    JAMA ophthalmology· 2025· PMID 40146165mais citado
  3. Malignant melanoma of the conjunctiva metastatic to the parotid gland: a case report.
    Journal of medical case reports· 2025· PMID 40790597mais citado
  4. Conjunctival malignant melanoma in a young immunocompetent male patient: clinical presentation and management-a case report.
    Journal of medical case reports· 2025· PMID 40685334mais citado
  5. Malignant melanoma of the conjunctiva metastasizing to the submandibular gland: a case report and review of the literature.
    BMC ophthalmology· 2025· PMID 40082862mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:617910(Orphanet)
  2. MONDO:0002096(MONDO)
  3. GARD:10744(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)

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

Melanoma maligno da conjuntiva
Compêndio · Raras BR

Melanoma maligno da conjuntiva

ORPHA:617910 · MONDO:0002096
Prevalência
<1 / 1 000 000
Casos
32 casos conhecidos
CID-10
C69.0 · Neoplasia maligna da conjuntiva
CID-11
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0346360
Repurposing
9 candidatos
binimetinibMEK inhibitor
cobimetinibRAF inhibitor
dabrafenibDNA alkylating agent
+6 outros
EuropePMC
Wikipedia
Papers 10a
DiscussaoAtiva

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