Lymphoproliferative disorders of the ocular adnexa orbit, eyelid, and conjunctiva include a group of diseases ranging from rlh and atypical lymphoid hyperplasia to lymphoma. The major longterm risk is a development of systemic lymphoma 3350% in 10 years after the initial diagnosis of an orbital lymphoproliferative disorder. Reactive lymphoid hyperplasia was diagnosed in 12 cases and lymphoma in 99 cases. No clinical or radiologic criteria facilitate a distinction among these lymphoproliferative lesions.
The 9 patients were 3 men and 6 women,withanagerangingfrom21to91mean,45years. Diffuse large bcell lymphoma and mantle cell lymphoma of the ocular adnexal region, and lymphoma of the. It has been recognized that most ocular adnexal lymphomas are lowgrade, extranodal, marginal zone bcell. This study involved 73 patients with lymphoproliferative lesions of the ocular adnexa. One percent of all lymphomas and approximately 8% of all extranodal lymphomas arise in the oa and the incidence is increasing. Bilateral ocular adnexal diffuse large cell lymphoma with. Histological results the various subtypes of lymphoma were diagnosed and their location and the extent of disease are summarised in table 1. When a specimen representing a tumor or tumorlike lesion of the eye or ocular adnexa is received in most general surgical pathology laboratories, the immediate reaction is one of panic, because these tumors are rarely encountered and often require complex gross dissections, based on a knowledge of ocular anatomy, which most. Lymphoid hyperplasia of the orbit and ocular adnexa. To evaluate the imaging characteristics of a cohort of patients with ocular adnexal lymphoproliferative disease oald. Ocular adnexal lymphoproliferative disorders oald range from the benign reactive. Benign reactive lymphoid hyperplasia blrh of the conjunctiva is a rare, lymphoproliferative process that belongs to the broad spectrum of ocular adnexal lymphocytic infiltrative disorders 1,2,3. Be aware of differential diagnoses for lymphoproliferative disorders 3. No patients between the ages of 11 and 20 years were reported to have benign lymphoid hyperplasia.
Oald may be easily mistaken for other conditions, and the eyecare provider may play a significant role in comanagement with other disciplines. Lymphoproliferative lesions of the ocular adnexa with morphological and immunohistological criteria of rlh were excluded from the study. To examine all lymphoproliferative lesions of the ocular adnexa diagnosed in iceland during 19832000 and to determine whether polymerase chain reaction pcr methods to determine clonality are helpful in characterizing these lesions. The major longterm risk is a development of systemic lymphoma 3350% in 10 years after the initial diagnosis of. Jan 15, 2006 the dna was extracted from a total of 59 fresh or paraffinembedded tumors, which included 49 specimens of emzl of ocular adnexa, 8 specimens of nonemzl of ocular adnexa including several specimens from patients seen for consultation only and thus not included in the cohort that formed the basis of clinical studies in this report, and 2. Cytologic evaluation of lymphoproliferative lesions of the orbitocular adnexa. To assess the characteristics and treatment outcomes of patients with primary ocular adnexal lymphoma oal in northern thailand. The ocular adnexal lymphoproliferative lesions were located as follows. Treatment of metastatic lesions in the orbit and ocular adnexa is usually palliative and may include radiotherapy, chemotherapy, hormonal therapy, surgery, or a combination of these modalities. One of the bacterial agents which in recent years has gained attention is the bacterium. Establishment of a combination scoring method for diagnosis. Out of 15 fine needle aspirates from orbital lesions received in last 5 years three female patients of age 56, 46 and 72 years presented to fnac clinic with upper eyelid swellings of 2 years, 3 years and 3 months duration.
Lymphoproliferative, myeloproliferative, and histiocytic. The most common locations involved in lymphoproliferative disorders of the ocular adnexa are the orbitlacrimal gland 3346%, conjunctiva 2342% and eyelid 1025%. Oal comprise 1% to 2% of all nonhodgkin lymphomas and about 8% of the extranodal lymphomas. Ninetyeight percent of conjunctival lymphomas arise from blymphocytes. However, these entities represent the majority of orbital malignancies. Lymphoma is a malignant lymphoproliferative tumor that can involve the conjunctiva. Lymphoproliferative lesions of the ocular adnexa cover a wide spectrum of entities ranging from brlh to malignant lymphomas. The exact etiology and pathogenesis of benign reactive lymphoid. Subconjunctival corticosteroids for benign lymphoid hyperplasia. Approximately 515% of all extranodal lymphomas are found in the ocular adnexal region, with approximately 25% of those involving the conjunctiva. Lymphoproliferative disease of the ocular adnexa is relatively uncommon. Mantle cell lymphoma and involvement of the orbit and ocular.
A pathological diagnosis of blh traditionally requires reactive follicles, polyclonality, and the absence of cytological atypia. Lymphoproliferative lesions of the ocular adnexa were analyzed to examine 1 the suitability of the revised europeanamerican lymphoma real classification for the subtyping of the lymphomas in these sites. Data from 69 patients treated for adnexal lymphoproliferative disease in the 12year period from 1991 to 2002 were retrospectively evaluated. Pdf the ocular adnexa include the eyelids, conjunctiva, lacrimal apparatus, and orbital soft tissue. Characteristics and treatment outcomes of patients with. Become familiar with how to manage a patient with suspected ocular adnexal lymphoproliferative disease 4. Ocular lymphoma constitutes less than 1% of all nonhodgkin lymphomas nhls and 5% to 15% of all extranodal cases. The incidence of lymphoproliferative ocular diseases, especially malignant lymphoma, has increased over the years.
Role of chlamydia in the development of ocular adnexal lymphoma. The lymph node processes and presents various antigens to either b or t lymphocytes. Lymphoproliferative disease of the ocular adnexa conjunctiva, eyelids and orbit is relatively uncommon. Ocular adnexal lymphoproliferative lesions consist of a spectrum of disease entities, including reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, and lymphoma. Igg4related systemic sclerosing disease of the ocular. Long term outcome of ocular adnexal lymphoma subtyped.
The ocular adnexa include the eyelids, conjunctiva, lacrimal apparatus, and orbital soft tissue. Imaging features of ocular adnexal lymphoproliferative disease. Please click on the hyperlink in additional links fieldpurpose. Pdf lymphoproliferative tumors of the ocular adnexa.
Department of ophthalmology, mayo clinic jacksonville, jacksonville, florida. Sixtynine cases of ocular adnexal lymphoproliferative disease, seen in a tertiary referral center from 1992 to 2003, were included in the study. Orbital lymphoproliferative disorders or ocular adnexal lymphoma oal comprises. Presented in part at the combined meeting of the international society for ocular. Dec, 2011 lymphoproliferative lesions of the ocular adnexa cover a wide spectrum of entities ranging from brlh to malignant lymphomas. Lymphoproliferative lesions of the ocular adnexa were analyzed to examine 1 the suitability of the revised europeanamerican lymphoma real classification for the subtyping of the lymphomas in. Lymphoproliferative lesions of the ocular adnexa ophthalmology. Lymphoproliferative lesions of the ocular adnexa analysis.
Understand the spectrum of ocular adnexal lymphoproliferative disease and be familiar with its typical appearance 2. It exhibits a polyclonal proliferation and presents in three different histologic types. Ocular adnexal lymphoproliferative lesions consist of a spectrum of disease entities, including reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, and. Ocular adnexal lymphoproliferative lesions sciencedirect. To access publisher full text version of this article. Orbital and ocular adnexal lymphomas are rare and represent around 12% of lymphomas and about 8% of the extranodal lymphomas. Orbital lymphoproliferative lesions radiology reference. Ocular adnexal lymphomas oals represent a small fraction of all systemic nonhodgkin lymphomas nhls. The two hyperplastic processes may evolve to localized or systemic lymphoma. Benign and malignant lymphoproliferative lesions and pseudotumors are among the most common primary tumors of the orbit or the ocular adnexa 3, 14, 22. Ocular adnexal lymphoproliferative disorders in an. To analyze the clinical features, treatment outcomes, and prognostic factors associated with lymphoproliferative lesions of the ocular adnexa in a korean population.
An update of ocular adnexal lymphomas diagnostic histopathology. The ocular adnexa oa include the eyelids, conjunctiva, lacrimal apparatus, and orbital soft tissue. The patients do not differ as to their age, sex, complaints, duration of symptoms and the ocular lesions themselves 5, 6, 17. Ocular adnexal lpds can arise within the intraconal and extraconal orbit soft tissues, lacrimal gland, extraocular muscles, lacrimal sac, eyelids, or conjunctiva. The 69 patients had a median age of 46 years range. Lesions were classified by using the world health organization modification of the revised europeanamerican classification of lymphoid neoplasms classification. Rose periocular lymphoproliferative diseases periocular lymphoid diseases range from benign hyperplasia to malignant lymphomas, the latter being solid tumors that arise from malignant transformation of leukocytes, particularly b lymphocytes. Asadiamoli f, nozarian z, bonaki hn, mehrtash v, entezari s. Clinicopathologic assessment of ocular adnexal lymphoproliferative lesions at tertiary eye hospital in iran. Lymphoid hyperplasia and malignant lymphoma occurring in the ocular adnexa orbit, conjunctiva, and eyelids. Ocular adnexal lymphoma oal is a rare manifestation of nonhodgkin lymphoma consisting of the tissues and structures surrounding the eye that include the conjunctiva, eyelids, lacrimal gland, and orbital soft tissues.
Flow cytometry in the diagnosis of lymphoproliferative. Jul 27, 2019 lymphoma is a malignant lymphoproliferative tumor that can involve the conjunctiva. The relative frequencies of ocular adnexal lymphoma presentation are orbit, 38%, conjunctiva, 31%, lacrimal apparatus, 18% and eyelid, %. For eyelid and orbital lesions, transcutaneous approach for may be utilized. Thus, further evaluation is necessary to characterize the lesions and determine whether they are localized or systemic. Lymphoproliferative lesions in the ocular adnexa encompass a wide spectrum of disorders, ranging from benign reactive lymphoid hyperplasia rlh, that is, a nonspecific orbital inflammation, to malignant lymphoma. Benign lymphoid hyperplasia of the conjunctiva in children. Subconjunctival corticosteroids for benign lymphoid. Mantle cell lymphoma and involvement of the orbit and. These diseases have a very similar clinical course. Ocular adnexal lymphoproliferative lesions mayo clinic.
Clinical, histopathological, and immunogenetic analysis of ocular adnexal lymphoproliferative disorders. Ocular adnexal lymphoproliferative lesions consist of a spectrum of disease entities, including. Role of chlamydia in the development of ocular adnexal. The eyelids show the highest proportion of secondary lymphoma involvement, 47% of all eyelid lymphoproliferative lesions, compared with 20% in the other ocular adnexa. Review article lymphoproliferative tumors of the ocular adnexa robert m. The most common subtype of conjunctival lymphoma is extranodal. Data was collected from electronic medical records and operative notes from chiang mai university hospital between january 2009 and december 2014. The lesions were categorized using the revised european american lymphoma classification of lymphoid tissues and analysed to determine the frequency and prognostic impact of tumour type, location, stage and patients age and sex. Igg4related orbital inflammation igg4roi often involves lacrimal glands and other orbital tissues and is an important differential diagnosis. Prognosis for patients in a korean population with ocular. Clinical aspects of igg4related orbital inflammation in a.
Lymphomas of the ocular adnexal region are primary or secondary lymphomas, and the majority of them are composed of small, mature lymphocytes, which provide a large differential diagnosis. Classification, diagnosis, and management of conjunctival. Lymphoproliferative disease lpd of the ocular adnexa is a relatively common orbital disease and is reported to account for 10. Lymphoproliferative lesions, including both malignant lymphoma and benign lymphoid hyperplasia, frequently involve. Role of chlamydia in the development of ocular adnexal lymphoma 663. Fine needle aspiration biopsy and molecular analysis in. The most frequent ocular adnexal tumors and simulating lesions are lymphoproliferative disorders lpds, including malignant lymphomas and orbital inflammation with lymphoid hyperplasia or infiltration. Of 164 biopsies of the ocular region for suspected lymphoma, we identified 6 cases of igg4 disease, 4 of which were previously unrecognized. Lymphoproliferative disorders of the ocular adnexa. Orbit and ocular adnexae are common sites 1015% for developing lymphoid lesions but have diagnostic and staging difficulties similar to other extranodal sites. The proportion of ocular adnexal lymphoproliferative disease. Lymphoproliferative, myeloproliferative, and histiocytic lesions of the orbit omar m. Dec 12, 2014 the most frequent primary lymphoma types of the ocular adnexa are extranodal marginal zone lymphoma, 63%, follicular lymphoma, 16%, diffuse large b cell lymphoma 10%.
Lymphoproliferative diseases of the ocular adnexa in korea. It has been recognized that most ocular adnexal lymphomas are lowgrade, extranodal. Lymphoproliferative lesions of the ocular adnexa were analyzed to examine 1 the suitability of the revised europeanamerican lymphoma real classif. Bilateral benign reactive lymphoid hyperplasia of the. However, they are among the most common tumors occurring in the ocular adnexa 26%. Lymphoproliferative lesions, including both malignant lym phoma and benign lymphoid hyperplasia, frequently involve the ocular adnexa, and present diagnostic challenges to oph. The most common subtype of conjunctival lymphoma is extranodal marginal zone lymphoma 80%. Lesions were classified by using the world health organization modification of the revised europeanamerican classification of. Igg4related sclerosing disease has been described in the orbit and ocular adnexa. In 15 cases the right orbit or ocular adnexa were involved, the lesions were situated on the left side in 11 patients, while 3 individuals presented with bilateral disease fig. Clinical, histopathological, and immunogenetic analysis of.
Characterization of malt lymphoma and reactive lymphoid hyperplasia tomohiko mannami m. Extranodal marginal zone lymphoma of the ocular adnexa. Rlh is believed to be a consequence of a chronic inflammatory response of lymphoid cells in the lacrimal gland, conjunctiva. Lymphoproliferative diseases lpds of the ocular adnexa encompass the majority of orbital diseases and include reactive follicular hyperplasia rfh, atypical lymphoid hyperplasia alh, and mucosaassociated lymphoid tissue lymphoma maltoma. Most of these are primary tumors and are usually nhls of bcell type. Ocular lymphoma constitutes less than 1% of all nonhodgkin lymphomas nhls and 515% of all extranodal cases. Lymphoid follicles lfs are usually observed during the histological examination of lpds. Lymphoproliferative lesions of the ocular adnexa analysis of 112 cases article in ophthalmology 1058.