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Eye (London, England) Apr 2023Lymphoma of the conjunctiva is an ocular malignancy derived from clonal proliferation of lymphocytes. The majority of conjunctival lymphoma is extranodal marginal zone... (Review)
Review
Lymphoma of the conjunctiva is an ocular malignancy derived from clonal proliferation of lymphocytes. The majority of conjunctival lymphoma is extranodal marginal zone B-Cell lymphoma (EMZL), however diffuse large B-cell (DLBCL), follicular (FL), mantle cell (MCL) and T- cell subtypes are also seen. Clinical manifestations are non-specific, but include unilateral or bilateral painless salmon-pink conjunctival lesions. Approaches to treatment have centered around local immunomodulation, often with Interferon-α2b or Rituximab (anti-CD20 monoclonal antibody) with or without radiation. Although conjunctival lymphoma is generally considered an indolent disease, recent advances in next-generation sequencing have improved clinicians' ability to predict future recurrence or systemic disease through assessment of cytogenic and molecular features. In this paper, we review the classification, clinical features, diagnostic techniques, and emerging strategies for management and prognostication of conjunctival lymphomas.
Topics: Humans; Lymphoma; Rituximab; Conjunctiva; Eye Neoplasms; Antineoplastic Agents; Lymphoma, B-Cell, Marginal Zone; Conjunctival Neoplasms
PubMed: 35882984
DOI: 10.1038/s41433-022-02176-2 -
Indian Journal of Ophthalmology Dec 2019Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired... (Review)
Review
Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Melanocytic lesions include nevus, racial melanosis, primary acquired melanosis, melanoma, and other ocular surface conditions like ocular melanocytosis and secondary pigmentary deposition. The most frequent nonmelanocytic neoplastic lesions include squamous cell carcinoma and lymphoma, both of which have typical features appreciated on clinical examination. The caruncle displays a slightly different array of tumors compared to those elsewhere on the conjunctiva, as nevus and papilloma are most common, but oncocytoma and sebaceous gland hyperplasia, adenoma, and carcinoma can be found. In this report, we provide clinical description and illustration of the many conjunctival and corneal tumors and we discuss tumor management.
Topics: Conjunctival Neoplasms; Corneal Diseases; Eye Neoplasms; Humans
PubMed: 31755426
DOI: 10.4103/ijo.IJO_2040_19 -
Eye (London, England) Jun 2021Ocular surface squamous neoplasia (OSSN) is the most common ocular tumour with an incidence ranging from 0.03 to 1.9 per 100,000 persons/year. The diagnosis is made on... (Review)
Review
Ocular surface squamous neoplasia (OSSN) is the most common ocular tumour with an incidence ranging from 0.03 to 1.9 per 100,000 persons/year. The diagnosis is made on clinical suspicion and confirmed with anterior-segment optical coherence tomography (AS-OCT), cytology, or histology. The purpose of this review is to provide an overview of the management options available for OSSN and review their success and recurrence rates. Surgery is the gold standard for the management of small OSSN lesions. With the increased use of less invasive diagnostic modalities such as AS-OCT and cytology, there has been a move to use topical therapies for the management of OSSN. The most commonly used agents are interferon-α2b (IFN), mitomycin-C (MMC) and 5-fluorouracil (5FU). They have been shown to have similar resolution and recurrence rates but differ in cost and side effect profile. IFN has the lowest side effect profile, but is also the most expensive, whereas MMC has the greatest surface toxicity and is priced midway between the three. 5FU is the cheapest of the three topical agents with less surface toxicity than MMC. Radiotherapy is mostly employed as adjuvant therapy. Newer novel therapies are available but have not been widely adopted as mainstream therapy due to cost and lack of clinical evidence. OSSN has the benefit of many management options. No single modality has been shown to superior and some patients will need the use of combination therapy to achieve an optimal clinical outcome.
Topics: Carcinoma, Squamous Cell; Conjunctival Neoplasms; Eye Neoplasms; Fluorouracil; Humans; Neoplasm Recurrence, Local
PubMed: 33564137
DOI: 10.1038/s41433-021-01422-3 -
Asia-Pacific Journal of Ophthalmology... 2017Conjunctiva and cornea tumors represent a large spectrum of conditions ranging from benign lesions to aggressive and life-threatening malignancies. Topical... (Review)
Review
Conjunctiva and cornea tumors represent a large spectrum of conditions ranging from benign lesions to aggressive and life-threatening malignancies. Topical pharmacotherapies and immunotherapies have recently acquired a relevant role in the management of conjunctival tumors and, in the past 2 decades, there has been a shift from surgery alone toward the use of these agents, both as a sole therapy or as adjunct to surgery (before or after surgery). The 3 main agents that have been used for topical medical treatment of conjunctival tumors are mitomycin-C, 5-fluorouracil, and interferon-α2b. Advantages of topical pharmacotherapies and immunotherapies include the ability to treat the entire ocular surface and prevention of surgical side effects and complications. The aim of this review is to summarize the current use of topical pharmacotherapy and immunotherapy in the management of conjunctival tumors.
Topics: Antineoplastic Agents; Combined Modality Therapy; Conjunctiva; Conjunctival Neoplasms; Humans; Immunotherapy
PubMed: 28399343
DOI: 10.22608/APO.201751 -
Archives of Pathology & Laboratory... May 2022Conjunctival melanocytic lesions consist of a variety of neoplastic and nonneoplastic conditions. These include benign processes such as primary intraepithelial...
CONTEXT.—
Conjunctival melanocytic lesions consist of a variety of neoplastic and nonneoplastic conditions. These include benign processes such as primary intraepithelial hypermelanosis and melanocytic hyperplasia, secondary forms of intraepithelial hypermelanosis and melanocytic hyperplasia, melanocytic nevi, melanocytic proliferations with malignant potential, and melanoma.
OBJECTIVE.—
To provide a concise yet comprehensive resource regarding the histopathologic diagnosis of conjunctival melanocytic lesions. We aim to detail and clarify the numerous classification schemes that exist for junctional melanocytic proliferations of the conjunctiva (known as primary acquired melanosis or PAM; also termed conjunctival melanocytic intraepithelial neoplasia or C-MIN). Although not uniformly adopted, C-MIN is classified by using a numeric system based on a defined set of criteria. A less complex scheme (conjunctival melanocytic intraepithelial lesion or CMIL) has recently been proposed by the World Health Organization. Additionally, we aim to update the reader regarding molecular features and prognostic indicators.
DATA SOURCES.—
Peer-reviewed literature and archived cases for illustration.
CONCLUSIONS.—
Accurate histologic classification is essential, as PAM/C-MIN/CMILs that have a significant potential to progress to invasive melanoma may be clinically indistinguishable from low-risk lesions. Conjunctival melanoma (CM) more closely resembles cutaneous melanoma in terms of its pathogenesis and molecular features, compared to melanoma arising at other mucosal sites or to uveal melanoma. Depth of invasion and ulceration status, among other factors, have emerged as important prognostic indicators in CM. Sentinel lymph node biopsy may provide further prognostic information. Lastly, integration of pathologic and clinical findings is essential at this anatomically sensitive location to determine appropriate clinical management.
Topics: Conjunctival Neoplasms; Humans; Hyperpigmentation; Hyperplasia; Melanoma; Skin Neoplasms; Melanoma, Cutaneous Malignant
PubMed: 34424954
DOI: 10.5858/arpa.2021-0006-RA -
Pathology Oncology Research : POR Oct 2018Conjunctival melanoma is a rare but sight and life threatening malignancy. It accounts for 2%-5% of all ocular tumours and 5%-7% of all ocular melanomas with an... (Review)
Review
Conjunctival melanoma is a rare but sight and life threatening malignancy. It accounts for 2%-5% of all ocular tumours and 5%-7% of all ocular melanomas with an incidence of 0.2-0.8 per million in the Caucasian population with rare cases reported in the non-Caucasians. In recent decades the incidence of uveal melanoma has been relatively stable whilst conjunctival and cutaneous melanoma have shown increasing incidence which may be connected to the result of environmental exposure to ultraviolet light. The dissimilarity in incidence between light and dark pigmented individuals observed in conjunctival melanomas compared to uveal and cutaneous melanomas may be related to differences in their histological structures and genetic profile. Recent molecular biological studies support the fact that each type of melanoma undergoes its own molecular changes and has characteristic biological behaviour. Further studies are required for each type of melanoma in order to ascertain their individual etiology and pathogenesis and based on this knowledge develop relevant preventative and treatment procedures.
Topics: Conjunctival Neoplasms; Humans; Incidence; Melanoma
PubMed: 29802540
DOI: 10.1007/s12253-018-0419-3 -
International Ophthalmology Clinics 2017To evaluate technologies and techniques available for the diagnosis of ocular surface tumors. (Review)
Review
PURPOSE
To evaluate technologies and techniques available for the diagnosis of ocular surface tumors.
METHODS
A review of the literature from 1947 to 2017, through the PubMed Database, was conducted in order to evaluate current diagnostic methods for ocular surface tumors.
RESULTS
Ocular surface squamous neoplasia, conjunctival melanoma, and conjunctival lymphoma are the three most common ocular surface malignancies. Technologies available to assist with diagnosis of these conditions, in addition to full thickness biopsy, include vital dyes, aspiration and impression cytology, in vivo confocal microscopy, ultrasound biomicroscopy, genetic testing, and anterior segment optical coherence tomography.
CONCLUSIONS
Histology remains the gold standard for diagnosis for all 3 of these malignancies. However, multiple diagnostic techniques are available to assist in making preliminary and early diagnoses, in differentiating between similar-appearing lesions, and in some cases, avoiding biopsy prior to initiating treatment. As imaging and technology continue to evolve, these adjunctive techniques will likely continue to play a greater role in clinical practice.
Topics: Carcinoma, Squamous Cell; Conjunctival Neoplasms; Corneal Diseases; Diagnostic Techniques, Ophthalmological; Eye Neoplasms; Humans; Lymphoma; Melanoma
PubMed: 28590280
DOI: 10.1097/IIO.0000000000000174 -
Oncology (Williston Park, N.Y.) Jan 2016Ocular melanoma is a rare but potentially devastating malignancy arising from the melanocytes of the uveal tract, conjunctiva, or orbit; it represents less than 5% of... (Review)
Review
Ocular melanoma is a rare but potentially devastating malignancy arising from the melanocytes of the uveal tract, conjunctiva, or orbit; it represents less than 5% of all melanoma cases in the United States. The management of ocular melanoma varies depending on its anatomic origin, since uveal and conjunctival melanoma have distinct biologies and thus different treatment strategies. Uveal melanoma is the most common type of ocular melanoma and is characterized by activation of the mitogen-activated protein kinase (MAPK) pathway (among other signaling pathways) via mutations in GNAQ or GNA11. Despite primary radiation or surgical therapy, up to 50% of patients will eventually develop metastatic disease, for which there is no standard therapy and no treatment that has been shown to improve overall survival. The biology of conjunctival melanoma is less well characterized but has been associated with BRAF and NRAS mutations, and results in metastatic disease in 20% to 30% of cases. Clinical trials are currently ongoing to further evaluate and optimize the role of targeted therapies, as well as immunotherapies, as both adjuvant and metastatic treatment in uveal and conjunctival melanoma.
Topics: Conjunctival Neoplasms; Humans; Melanoma; Uveal Neoplasms
PubMed: 26791842
DOI: No ID Found -
British Medical Bulletin Jan 2017Retinoblastoma, uveal and conjunctival melanomas are important malignancies within the remit of ocular oncology. Outlined are the diagnostic features and management... (Review)
Review
BACKGROUND
Retinoblastoma, uveal and conjunctival melanomas are important malignancies within the remit of ocular oncology. Outlined are the diagnostic features and management principles, as well as advancements in the field and current challenges.
SOURCES OF DATA
Original papers, reviews and guidelines.
AREAS OF AGREEMENT
Most eyes with retinoblastoma (International Intraocular Retinoblastoma Classification (IIRC) Group A-D) are salvaged, whereas advanced cases (Group E) remain a challenge. Despite a high rate of local tumour control in uveal melanoma, metastatic spread commonly occurs. Conjunctival melanoma is treated by complete resection, but high rates of local recurrence occur, with the possibility of systemic relapse and death.
AREAS OF CONTROVERSY
Use of the IIRC in retinoblastoma, and systemic screening in melanomas.
GROWING POINTS
Utilization of novel treatment modalities in retinoblastoma and an increasing understanding of the genetic basis of melanomas.
AREAS TIMELY FOR DEVELOPING RESEARCH
Improvements in chemotherapy delivery in retinoblastoma and prognostic tests in melanomas.
Topics: Antineoplastic Agents; Clinical Trials as Topic; Conjunctival Neoplasms; Early Detection of Cancer; Humans; Intravitreal Injections; Melanoma; Neoplasm Recurrence, Local; Prognosis; Retinal Neoplasms; Retinoblastoma; Uveal Neoplasms
PubMed: 28069617
DOI: 10.1093/bmb/ldw053 -
BMJ Case Reports Jul 2019
Topics: Aged; Antineoplastic Agents, Alkylating; Antineoplastic Agents, Immunological; Bendamustine Hydrochloride; Conjunctival Neoplasms; Humans; Lymphoma, Non-Hodgkin; Rituximab
PubMed: 31296640
DOI: 10.1136/bcr-2019-229599