Raras
Buscar doenças, sintomas, genes...
Linfoma primário da conjuntiva
ORPHA:319667CID-10 · C85.7CID-11 · 2B33.5DOENÇA 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

📋

Neoplasia linfoide primária rara que afeta a conjuntiva, apresentando-se tipicamente como lesão salmão, infiltrativa e indolor. Pode ocorrer em qualquer local da conjuntiva, sendo mais comum no saco conjuntival.

Publicações científicas
923 artigos
Último publicado: 2026 Jan-Dec
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SUS: Sem cobertura SUSScore: 0%
CID-10: C85.7
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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 pesquisa11
Total histórico923PubMed
Últimos 10 anos80publicações
Pico201911 papers
Linha do tempo
20202015Hoje · 2026📈 2019Ano 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

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Onde tratar no SUS

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

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

Corneal Lymphoma in Humans: First Population-Based Evidence of Occurrence and Clinical Characteristics.

Clinical lymphoma, myeloma & leukemia2026 Jan 12

Lymphomas involving ocular structures are well documented, particularly in the conjunctiva, vitreoretina, and choroid. In contrast, no primary lymphoma of the cornea has previously been reported in humans, whereas it has only been described in animals, essentially in rare, small series in horses. This study aimed to determine whether corneal lymphoma exists in humans and to describe its epidemiologic and clinical characteristics. A retrospective, population-based analysis was conducted using the SEER 17 registries (November 2024 submission). All lymphoma cases coded as C69.1 (cornea) between 2000 and 2022 were included. Demographic, pathological, treatment, and survival data were extracted using SEER*Stat version 9.0.42.0. Incidence rates were age-adjusted to the 2000 U.S. standard population. Survival was calculated from diagnosis to death or last follow-up. Five cases of corneal lymphoma were identified, all strictly localized to the cornea, corresponding to an incidence of 0.0028 cases per million persons per year. The mean age at diagnosis was 75 years, with a 4:1 male predominance. Histologic subtype was available in 3 cases, all of which were mucosa-associated lymphoid tissue (MALT) lymphomas. All patients underwent local surgical management, and radiotherapy was administered in unilateral cases. No systemic therapy was reported. Median follow-up was 99 months. Two deaths occurred (1 unrelated to lymphoma and 1 lymphoma-related) while 3 patients remained alive, including 2 long-term survivors with follow-up exceeding 14 years. Corneal lymphoma does occur in humans, is exceptionally rare, and appears to share the favorable prognosis of other ocular MALT lymphomas. Radiotherapy may provide durable control in localized disease. These findings represent the first evidence of this entity and support heightened diagnostic awareness in ocular oncology.

#2

An Indolent Inflammatory Myofibroblastic Tumor of the Conjunctiva.

Cornea2026 Apr 01

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate biological potential, predominantly affecting children and young adults. While IMT most commonly involves the lungs, abdomen, pelvis, and retroperitoneum, primary occurrences in the orbit or ocular surface are rare. Case report. Here we present a case of a 26-year-old woman with a small, indolent conjunctival IMT harboring anaplastic lymphoma kinase rearrangement. Following surgical resection, the patient showed no evidence of recurrence after 9 months of follow-up. Notably, this report firstly provided anterior segment optical coherence tomography scan and ultrasound biomicroscope images of conjunctival IMT. IMT should be considered in the differential diagnosis of localized conjunctival lesions unresponsive to steroid therapy. Anterior segment optical coherence tomography and ultrasound biomicroscope serve as valuable tools, aiding in differential diagnosis, surgical planning, and postoperative recurrence monitoring.

#3

A Compilation of Orbital and Ocular Adnexal Lymphomas on 18 F-FDG PET/CT.

Clinical nuclear medicine2026 Jan 01

The most common primary orbital cancer in adults is orbital lymphoma. Lymphomas involving the orbit, eyelid, or conjunctiva are described as ocular adnexal lymphoma (OAL). OAL comprises 2% of non-Hodgkin lymphomas but around 8% of extranodal lymphomas. OAL can appear bilaterally in any region of orbit with systemic involvement or restricted to orbit/adnexal region. When identifying systemic involvement from orbital lymphoma and other extranodal lymphomas, PET/CT has been demonstrated to have a higher sensitivity than CT. The predominant subtype of OAL is mucosa-associated lymphoid tissue (MALT)-type lymphoma, followed by follicular lymphoma.

#4

Primary Localized Conjunctival Amyloidosis Mimicking Lymphoma.

Beyoglu eye journal2025

A 27-year-old healthy male presented to our clinic with complaints of redness and burning in his left eye for the past 6 months. Biomicroscopic examination revealed a salmon-colored, elevated, painless mass lesion involving the entire lower fornix conjunctiva of the left eye. A conjunctival incisional biopsy was performed due to suspicion of lymphoproliferative disease. Histopathological examination confirmed amyloidosis. Further investigations did not reveal any evidence of systemic amyloidosis, lymphoma, or other lymphoproliferative diseases. During the 18-month follow-up after surgery, no recurrence or new findings were observed in the patient's ophthalmologic or systemic evaluations. Amyloidosis in the periocular region can present with various clinical findings. Although a classic salmon-colored conjunctival mass initially may suggest lymphoproliferative diseases, conjunctival amyloidosis should be considered in the differential diagnosis.

#5

Ocular Adnexal Natural Killer- and T-cell Lymphomas: A Review.

International ophthalmology clinics2025 Oct 01

Natural Killer- (NK) and T-cell lymphomas presenting in the ocular adnexa are rare. They account for ~3% of all ocular adnexal lymphomas and 17% of eyelid lymphomas. This article presents the epidemiology, clinics, treatment, and prognosis of ocular adnexal NK- and T-cell lymphomas. Orbital involvement is the most common in systemic NK- and T-cell lymphomas, including extranodal NK/T-cell lymphoma (ENKTL), systemic anaplastic large cell lymphoma (sALCL), and peripheral T-cell lymphoma (not otherwise specified) (PTCL-NOS). Primary cutaneous T-cell lymphomas, including mycosis fungoides (MF) and primary cutaneous anaplastic large cell lymphoma (C-ALCL), may present in the eyelids or periorbital skin. Several of the NK- and T-cell lymphomas, including ENKTL, sALCL, C-ALCL, PTCL-NOS, and MF, show a male predilection and present in adults. However, some subtypes including T-lymphoblastic leukemia/lymphoma are frequent in adolescents and young adults. Systemic ocular adnexal NK- and T-cell lymphomas often mimic inflammatory orbital conditions. Early biopsies, even in younger patients are therefore important to not miss these rare malignancies, which often have advanced stage disease and a poor prognosis. Primary cutaneous lymphomas can present with erythematous patches or as ulcerated eyelid tumors. In general, cutaneous lymphomas have a better prognosis, although recurrences may occur, leading to increased patient morbidity. The treatment for systemic NK- and T-cell lymphomas includes chemotherapy regimens depending on the lymphoma subtype, combined with ocular adnexal radiotherapy, and in some cases hematological stem-cell transplantation. Primary cutaneous lymphomas are frequently treated with radiotherapy, skin-directed therapies, and in some advanced cases with systemic treatments.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC2 artigos no totalmostrando 79

2026

Corneal Lymphoma in Humans: First Population-Based Evidence of Occurrence and Clinical Characteristics.

Clinical lymphoma, myeloma & leukemia
2025

Primary Localized Conjunctival Amyloidosis Mimicking Lymphoma.

Beyoglu eye journal
2025

Ocular Adnexal Natural Killer- and T-cell Lymphomas: A Review.

International ophthalmology clinics
2025

Clinical features and outcomes of primary ocular adnexal follicular lymphoma: a retrospective analysis.

International journal of hematology
2026

An Indolent Inflammatory Myofibroblastic Tumor of the Conjunctiva.

Cornea
2025

The clinical outcomes of ultra-low-dose radiotherapy for primary conjunctival mucosa-associated lymphoid tissue lymphoma.

International ophthalmology
2026

A Compilation of Orbital and Ocular Adnexal Lymphomas on 18 F-FDG PET/CT.

Clinical nuclear medicine
2025

An International Lymphoma Radiation Oncology Group Study of Radiation Therapy for Bilateral Indolent Orbital Adnexal Lymphomas.

International journal of radiation oncology, biology, physics
2025

Primary CD4 + T-Cell Lymphoproliferative Disorder of Conjunctiva.

Cornea
2025

A rare case of primary unilateral conjunctival small lymphocytic lymphoma: A case report.

International journal of surgery case reports
2025

Can high-resolution optical coherence tomography provide an optical biopsy for ocular surface lesions?

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2024

Primary Conjunctival Basal Cell Carcinoma Mimicking an Ocular Surface Squamous Neoplasia in a Young Adult Filipino: A Case Report and Literature Review.

Case reports in ophthalmological medicine
2024

Primary ocular adnexal mantle cell lymphoma with distant spread and involvement of the contralateral eye one year later; a case report and literature review.

Journal of surgical case reports
2024

Primary third eyelid lymphoma with aggressive local behavior in a dog.

Topics in companion animal medicine
2024

Variable presentations of six conjunctival/limbal ocular surface squamous neoplasia (OSSN) cases: How good is our clinical judgment evidenced by the correlation to the histopathological findings and diagnosis?

International journal of surgery case reports
2024

Primary anaplastic lymphoma kinase-positive large B-cell lymphoma of the left bulbar conjunctiva: A case report.

World journal of clinical cases
2024

Lymphoma involvement of the eyelid and eye.

Clinics in dermatology
2023

A case of primary conjunctival follicular lymphoma presenting as nasolacrimal obstruction.

Journal of surgical case reports
2023

[Clinical pathological characteristics analysis of ocular adnexal follicular lymphoma].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2023

Primary unilateral conjunctival small lymphocytic lymphoma in a child: Case report and literature review.

Oman journal of ophthalmology
2023

Generalized Reddish Skin Nodules.

Acta medica Indonesiana
2023

Clinical features and prognosis of patients with metastatic ocular and orbital melanoma: A bi-institutional study.

Cancer medicine
2023

Radiation therapy for primary orbital and ocular adnexal lymphoma.

Clinical and translational radiation oncology
2022

Mucosal associated Lymphoid Tissue Lymphoma of the uvea: an analysis of 3 cases.

BMC ophthalmology
2023

Watchful waiting is an acceptable treatment option for asymptomatic primary ocular adnexal mucosa-associated lymphoid tissue lymphoma: A retrospective study.

Cancer medicine
2022

Prospective Study of 4 Gy Radiotherapy for Orbital Mucosa-Associated Lymphoid Tissue Lymphoma (FORMAL).

Cancers
2023

Thirteen cancers associated with HIV infection in a Black South African cancer patient population (1995-2016).

International journal of cancer
2022

Clinicopathologic Characteristics Associated with Prognosis in Ocular Extranodal Marginal Zone B Cell Lymphoma.

Medicina (Kaunas, Lithuania)
2022

Long-Term Clinical Outcomes in Treatment-Naïve Patients With Orbital Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma: A Single-Center Study.

Frontiers in oncology
2023

Specific location of ocular adnexal lymphoma and mortality: an international multicentre retrospective study.

The British journal of ophthalmology
2022

Primary Low Grade Follicular Lymphoma of the Conjunctivae Mimicking Nodular Anterior Scleritis: A Case Report.

Clinical medicine & research
2022

Management of primary choroidal lymphoma presenting as extensive serous retinal detachment and salmon patch of bulbar conjunctiva: a case report.

Journal of medical case reports
2022

Primary radiation therapy in stage I/II indolent orbital lymphoma - a comprehensive retrospective recurrence and toxicity analysis.

European journal of haematology
2021

Prospective Phase II trial of radiation therapy in localised non-gastric marginal zone lymphoma with prospective evaluation of autoimmunity and Helicobacter pylori status: TROG 05.02/ALLG NHL15.

European journal of cancer (Oxford, England : 1990)
2021

Incidence and trends of ophthalmic cancer in Singapore: Data from Singapore Cancer Registry.

Annals of the Academy of Medicine, Singapore
2021

[Clinical and pathological analysis of ocular adnexal diffuse large B-cell lymphoma].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2021

A Case Report of a Primary Conjunctival Diffuse Large B-Cell Lymphoma: Keeping an Eye out Following a Nasopharyngeal Carcinoma.

Cureus
2020

[The preliminary observation of intralesional rituximab administration in primary ocular adnexal mucosa-associated lymphoid tissue lymphoma].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2021

[Primary unilateral conjunctival MALT lymphoma].

Journal francais d'ophtalmologie
2021

Orbital and ocular adnexal lymphoma: a review of epidemiology and prognostic factors in Taiwan.

Eye (London, England)
2021

Second primary malignancies of eye and ocular adnexa after a first primary elsewhere in the body.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2020

Primary hepatic extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue.

Journal of pathology and translational medicine
2019

Tumors of the conjunctiva and cornea.

Indian journal of ophthalmology
2019

Role of F-18 FDG PET/CT in non-conjunctival origin ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphomas.

EJNMMI research
2019

Unilateral MALT conjunctival lymphoma: a case report.

La Tunisie medicale
2019

[Clinical features of malignant orbital lymphoma].

Vestnik oftalmologii
2019

[Current Therapies in Superficial Malignant Tumors].

Laryngo- rhino- otologie
2019

[Conjunctival malignancies].

Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
2019

Patterns of care for orbital marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue in Korea throughout 2016: Results from a multicenter cross-sectional cohort study (KROG 16-19).

Asia-Pacific journal of clinical oncology
2020

Cancer in Africa 2018: The role of infections.

International journal of cancer
2020

International multicentre retrospective cohort study of ocular adnexal marginal zone B-cell lymphoma.

The British journal of ophthalmology
2019

[Ocular natural killer/T cell lymphoma: a clinicopathologic analysis].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2019

Conjunctival lymphomas in Japanese monozygotic twins: A case report.

Oncology letters
2019

Case Report: Primary Conjunctival Non-Hodgkin Marginal Zone Lymphoma.

Optometry and vision science : official publication of the American Academy of Optometry
2018

The treatment results in patients with lmphoma disease in the orbit.

Ceska a slovenska oftalmologie : casopis Ceske oftalmologicke spolecnosti a Slovenske oftalmologicke spolecnosti
2018

[Primary uveal lymphoma effectively treated with radiotherapy: a case report and literature review].

Nan fang yi ke da xue xue bao = Journal of Southern Medical University
2018

Unilateral localized conjunctival amyloidosis in a patient with a history of contralateral orbit/eyelid lymphoma.

Experimental and molecular pathology
2018

Population-based incidence of conjunctival tumours in Olmsted County, Minnesota.

The British journal of ophthalmology
2017

Primary hepatic mucosa-associated lymphoid tissue lymphoma in a patient with no chronic liver disease: Case report.

Radiology case reports
2019

A case of conjunctival MALT lymphoma: successfully treated with solely extended rituximab therapy.

International ophthalmology
2017

[Malignant lymphomas of the eye].

Der Pathologe
2017

Association of Disease Location and Treatment With Survival in Diffuse Large B-Cell Lymphoma of the Eye and Ocular Adnexal Region.

JAMA ophthalmology
2018

Characteristics of primary extranodal marginal zone B-cell lymphoma in Korea: conjunctiva versus other ocular adnexa.

The British journal of ophthalmology
2018

Low-Dose Radiation Therapy for Primary Conjunctival Marginal Zone B-Cell Lymphoma.

Cancer research and treatment
2017

Target volume dose and clinical outcome in radiotherapy for primary marginal zone lymphoma of the ocular adnexa.

Molecular and clinical oncology
2017

Conjunctival Tumors: Review of Clinical Features, Risks, Biomarkers, and Outcomes--The 2017 J. Donald M. Gass Lecture.

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.)
2017

Lymphoproliferative Tumors of the Ocular Adnexa.

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.)
2017

Clinical Features Differentiating Benign From Malignant Conjunctival Tumors in Children.

JAMA ophthalmology
2017

A case of anaplastic lymphoma kinase (ALK)-positive ciliated muconodular papillary tumor (CMPT) of the lung.

Pathology international
2017

Intralesional rituximab in primary conjunctival follicular lymphoma relapsed.

Archivos de la Sociedad Espanola de Oftalmologia
2016

Favourable response to rituximab by an ocular adnexal primary lymphoma.

Archivos de la Sociedad Espanola de Oftalmologia
2016

[The clinical and histopathological analysis of 2 053 cases of conjunctival neoplasms].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2016

Extranodal Marginal Zone B-cell Lymphoma of the Ocular Adnexa.

Cancer control : journal of the Moffitt Cancer Center
2016

Partial orbit irradiation achieves excellent outcomes for primary orbital lymphoma.

Practical radiation oncology
2016

Infectious Aetiology of Marginal Zone Lymphoma and Role of Anti-Infective Therapy.

Mediterranean journal of hematology and infectious diseases
2016

Primary conjunctival follicular lymphoma mimicking chronic conjunctivitis.

Archivos de la Sociedad Espanola de Oftalmologia
2015

[Prognosis of primary malignant tumors of the conjunctiva].

Journal francais d'ophtalmologie
2015

Ophthalmic manifestations of relapsing acute childhood leukemia.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2015

Conjunctival extranodal natural killer/T-cell lymphoma, nasal type.

Cornea

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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. Corneal Lymphoma in Humans: First Population-Based Evidence of Occurrence and Clinical Characteristics.
    Clinical lymphoma, myeloma & leukemia· 2026· PMID 41654479mais citado
  2. An Indolent Inflammatory Myofibroblastic Tumor of the Conjunctiva.
    Cornea· 2026· PMID 40607701mais citado
  3. A Compilation of Orbital and Ocular Adnexal Lymphomas on 18 F-FDG PET/CT.
    Clinical nuclear medicine· 2026· PMID 40247460mais citado
  4. Primary Localized Conjunctival Amyloidosis Mimicking Lymphoma.
    Beyoglu eye journal· 2025· PMID 41059241mais citado
  5. Ocular Adnexal Natural Killer- and T-cell Lymphomas: A Review.
    International ophthalmology clinics· 2025· PMID 40996231mais citado
  6. Obstructive Jaundice Revealing Primary Diffuse Large B-Cell Lymphoma of the Ampulla of Vater in a Child: A Case Report.
    Case Rep Oncol· 2026· PMID 41705106recente
  7. Rare diagnosis: the second documented report of primary diffuse large B-cell lymphoma of the fallopian tube.
    BMJ Case Rep· 2026· PMID 41611332recente
  8. Small bowel lymphoma presenting with frequent perforation: a single center experience focusing on the monomorphic epitheliotropic intestinal T-cell lymphoma.
    BMC Gastroenterol· 2026· PMID 41578186recente
  9. FDG PET/CT in Primary Mucosa-associated Lymphoid Tissue Lymphoma of the Bladder.
    Clin Nucl Med· 2026· PMID 41543873recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:319667(Orphanet)
  2. MONDO:0017907(MONDO)
  3. GARD:21426(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55787510(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

Linfoma primário da conjuntiva
Compêndio · Raras BR

Linfoma primário da conjuntiva

ORPHA:319667 · MONDO:0017907
CID-10
C85.7 · Outros tipos especificados de linfoma não-Hodgkin
CID-11
MedGen
UMLS
C4706485
EuropePMC
Wikidata
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