V. Kakkassery

Vitreous microRNA levels as diagnostic biomarkers for vitreoretinal lymphoma

To the editor: Primary vitreoretinal lymphoma (PVRL) is often associated with primary central nervous system lymphoma (PCNSL) and is a rare high-grade diffuse large-cell B-cell lymphoma occurring within the vitreous and/or retina.[1][1][⇓][2]-[3][3] Overlapping clinical signs observed in PVRL

Femtosecond Laser-Assisted Cataract Surgery Without OVD: A Prospective Intraindividual Comparison

PURPOSE To compare femtosecond laser-assisted cataract surgery without the use of ophthalmic viscosurgical device (OVD) with standard phacoemulsification using OVD. METHODS This was a prospective, randomized, single-center trial of 37 patients (74 eyes) diagnosed as having significant cataract in both eyes. Randomly, one eye underwent standard phacoemulsification with OVD (the OVD group); this group acted as controls. The other eye was treated with the femtosecond laser; the subsequent manual part of the procedure was performed without OVD (non-OVD group). Time of surgery, amount of fluid used during phacoemulsification, central corneal thickness, intraocular pressure, endothelial cell count, and visual acuity were documented over the 6-month follow-up. RESULTS There were no major complications and no significant difference in overall surgery time (non-OVD: 375 ± 81 seconds; OVD: 362 ± 43 seconds; P = .713) and in the quantity of fluid passing through the eye (non-OVD: 187 ± 35 mL; OVD: 186 ± 27 mL; P = .952) between groups. Endothelial cell loss after 6 months was not significantly different between groups (non-OVD: -2.4%; OVD: -2.7%; P = .880). Central corneal thickness was not different at 1 week postoperatively between groups (non-OVD: 575 ± 45 µm; OVD: 573 ± 46 µm; P = .820). Three patients in the OVD group and one patient in the non-OVD group experienced intraocular pressure greater than 25 mm Hg at 1 day postoperatively. There were no significant differences in corrected distance visual acuity between groups (logMAR non-OVD: 0.024; OVD: 0.038; P = .461). CONCLUSIONS Femtosecond laser-assisted treatment allows the cataract surgeon to perform phacoemulsification and intraocular lens implantation without the use of OVD at no additional risk to the corneal endothelium. Furthermore, there was a tendency toward fewer increases in intraocular pressure in patients treated with the femtosecond laser.

Diagnostik und Therapie des primären vitreoretinalen Lymphoms

ZusammenfassungHintergrundBeim primären vitreoretinalen Lymphom (PVRL), einem seltenen hämatopoetischen okulären Tumor, handelt es sich meist um ein großzelliges diffuses B-Zell-Lymphom. Das PVRL, das früher auch als primäres intraokulares Lymphom (PIOL) bezeichnet wurde, ist eine Unterart des primären ZNS-Lymphoms (PZNSL). DiagnostikDie Diagnose gestaltet sich häufig sehr schwierig, da klinisch eine intermediäre bzw. posteriore Uveitis imitiert wird. Dies wird auch als sog. „Masquerade-Syndrom“ bezeichnet. Notwendig für die endgültige Diagnose sind immunhistochemische, zytologische, pathologische und auch molekularpathologische Untersuchungen der operativ gewonnenen okulären Gewebe. Als hilfreich hat sich auch die Zytokinbestimmung (Interleukin-10) entweder im Vorderkammerpunktat oder im Glaskörpergewebe erwiesen. TherapieLeider zeigen sich die Therapien, die erfolgreich in der Behandlung der systemischen Lymphome sind, weniger effektiv in der Therapie des PVRLs und des PZNSLs. Daher werden heutzutage beim PVRL, zeitweise in Kombination mit einer Radiatio, aggressive chemotherapeutische Substanzen wie Methotrexat und Rituximab appliziert. Hierbei werden beide Chemotherapeutika sowohl systemisch als auch lokal verabreicht. Lediglich beim monokularen PVRL wird die alleinige intravitreale Gabe einer dieser Substanzen diskutiert. Bisher herrscht noch Uneinigkeit, ob die lokale Monotherapie ausreichend ist. Das PVRL reagiert meist sehr gut auf die initiale Therapie, jedoch kommt es im Verlauf häufig zu Rezidiven wie auch zum Auftreten einer ZNS-Manifestation, welche die Prognose quoad vitam deutlich limitieren.AbstractBackgroundPrimary vitreoretinal lymphoma (PVRL) is a rare ocular lymphoid malignancy, mostly a diffuse large B-cell lymphoma. The PVRL, previously called primary intraocular lymphoma (PIOL), is a subset of primary central nervous system lymphoma (PCNSL). DiagnosisThe diagnosis of PVRL is often difficult as it often mimics chronic intermediate or posterior uveitis; therefore, PVRL requires various procedures for the diagnostics, e.g. immunohistochemistry, cytology, pathology, molecular pathology and cytokine analysis (interleukin 10) after surgically obtaining ocular specimens.TherapyTreatment forms that are effective for systemic lymphomas have not been reliably successful for PVRL and PCNSL. Current management of PVRL consists of chemotherapy, such as methotrexate or rituximab, possibly combined with external beam radiation whereby both chemotherapeutic agents are administered systemically as well as intravitreally. Intravitreal treatment alone is recommended solely in the case of monocular PVRL, which is highly controversial. A PVRL usually responds well to initial treatment; however, relapse rates and CNS involvement are high, resulting in a poor prognosis and limited survival.

Lymphome der okulären Adnexe

ZusammenfassungHintergrundLymphome der okulären Adnexe stellen ein heterogenes onkologisches Krankheitsbild dar. ZielsetzungZiel des Beitrags ist die Vermittlung der wesentlichen interdisziplinären diagnostischen Schritte vor der zielgerichteten individuell angepassten Therapie.DiagnostikIn der okulären Diagnostik erfolgt je nach Lokalisation am Augenlid, in der Bindehaut oder in der Orbita eine Befunddokumentation mit einem Foto oder mit einer weiterführenden Bildgebung. Funktionelle Aspekte spielen gerade bei Befunden im Sehnervenbereich eine Rolle und sollten mit untersucht werden. Die histopathologische Begutachtung sichert die Diagnose und charakterisiert die Lymphomeigenschaften näher. Ein systemisches „Staging“ nach weiteren Lymphommanifestationen im Körper sowie Blut- und Knochenmarkuntersuchungen auf das Vorliegen einer Leukämie runden die Diagnostik ab.TherapieSowohl die Bestrahlung als auch eine lokale oder systemische Chemotherapie bzw. eine Antibiotikagabe kommen nach einer möglichen chirurgischen Exzision bei der Behandlung des Lymphoms infrage. Stadieneinteilungen wie die Ann-Arbor-Klassifikation für Lymphome bzw. die TNM-Klassifikation für Lymphome der okulären Adnexe sowie die Leitlinien der Deutschen Gesellschaft für Hämatologie und Medizinische Onkologie sind wegweisend für die Entscheidung einer individuell angepassten Therapie, die durch eine psychoonkologische Betreuung ergänzt werden sollen.AbstractBackgroundLymphomas of the ocular adnexa are heterogeneous and demonstrate a wide range of clinical, histological, immunohistochemical and molecular genetic characteristics. AimThe aim of this article is to give an overview of the interdisciplinary diagnostics and individually adapted lymphoma subtype-based therapy.DiagnosticsDepending on the lymphoma localisation, i.e. whether in the eyelid, the conjunctiva or in the orbit, a photograph or a radiological scan is required to record the tumor extent. Visual function is more likely to be impacted when the lymphoma arises in the posterior orbit, close to the optic nerve and imaging diagnostics are therefore necessary. Histological investigations are essential for confirming the lymphoma diagnosis and give information about the particular subtype, which in turn will determine subsequent patient management, Clinical staging investigations for determining the systemic extent of the lymphoma manifestation (e.g. imaging, blood analyses as well as bone marrow biopsy) are mandatory.TherapyExternal beam radiation, local and systemic chemotherapy or in some cases antibiotics are treatment options after surgical excision in isolated ocular adnexal lymphoma. The TNM classification of the American Joint Committee on Cancer or the Ann Arbor staging system, as well as the guidelines of the German Society of Hematology and Medical Oncology are all tools to aid the choice of the appropriate individually adapted therapy for systemic disease, which includes psycho-oncological care.AbstractSection StopBACKGROUND Lymphomas of the ocular adnexa are heterogeneous and demonstrate a wide range of clinical, histological, immunohistochemical and molecular genetic characteristics. AIM The aim of this article is to give an overview of the interdisciplinary diagnostics and individually adapted lymphoma subtype-based therapy. DIAGNOSTICS Depending on the lymphoma localisation, i.e. whether in the eyelid, the conjunctiva or in the orbit, a photograph or a radiological scan is required to record the tumor extent. Visual function is more likely to be impacted when the lymphoma arises in the posterior orbit, close to the optic nerve and imaging diagnostics are therefore necessary. Histological investigations are essential for confirming the lymphoma diagnosis and give information about the particular subtype, which in turn will determine subsequent patient management, Clinical staging investigations for determining the systemic extent of the lymphoma manifestation (e.g. imaging, blood analyses as well as bone marrow biopsy) are mandatory. THERAPY External beam radiation, local and systemic chemotherapy or in some cases antibiotics are treatment options after surgical excision in isolated ocular adnexal lymphoma. The TNM classification of the American Joint Committee on Cancer or the Ann Arbor staging system, as well as the guidelines of the German Society of Hematology and Medical Oncology are all tools to aid the choice of the appropriate individually adapted therapy for systemic disease, which includes psycho-oncological care.

Untypische Masquerade einer okulären Karzinommetastase

BACKGROUND Idiopathic and therapy resistant uveitis especially of unclear origin, is a diagnostic challenge for ophthalmologists. Metastases to the anterior chamber or vitreous body can occasionally mimic the clinical picture of uveitis, a variant on the usual lymphomatous masquerade syndrome. The underlying pathological pathways leading to the metastatic spread of tumor cells within the fluid compartments of the eye remain unclear. CASE REPORT We present an unusual case of vitreous metastases to the right eye of a patient in whom an underlying primary malignancy was unknown. After recurrent episodes of cortisone-refractive panuveitis with pseudohypopyon, a diagnostic vitreous biopsy was performed. Cytopathological examination of the vitreous sample revealed carcinoma cells with an immune profile suggestive of lung cancer metastasis. Subsequent staging investigations revealed a primary lung adenocarcinoma as well as cerebral, adrenal and osseous metastases. THERAPY Due to the extent of dissemination of this non-small cell lung cancer (NSCLC), only palliative treatment including external beam irradiation and systemic chemotherapy was possible to reduce pain and to maintain vision as well as an attempt at systemic control of the disease.ZusammenfassungHintergrundEine therapieresistente Uveitis, gerade mit unklarer Genese, stellt eine diagnostische Herausforderung für den Augenarzt dar. Eine Karzinommetastasierung in die Vorderkammer oder den Glaskörper als Ursache darf in diesem Zusammenhang als absolute Rarität angesehen werden. Die Pathologie der Tumorstreuung, die zu einem solchen Masquerade-Syndrom führt, ist bis heute weitestgehend unbekannt.FallberichtIn dem hier vorgestellten, ungewöhnlichen Fall einer Glaskörpermetastase am rechten Auge kam es zur Diagnosestellung eines bislang nicht bekannten, aber weit fortgeschrittenen, metastasierten Bronchialkarzinoms. Nach wiederholt auftretendem Bild einer massiven Panuveitis mit eindrucksvollem Pseudohypopyon, trotz intensiver Kortisontherapie, stellte sich der Verdacht auf ein intraokulares Malignom. Die zytopathologische Untersuchung einer Glaskörperprobe sicherte die Diagnose einer vitrealen Bronchialkarzinommetastase. Anschließende Staginguntersuchungen fanden ein nichtkleinzelliges Adenokarzinom als Primarius in der Lunge. Des Weiteren bestanden multiple zerebrale, adrenale und ossäre Metastasen.BehandlungAufgrund des metastasierten nichtkleinzelligen Bronchialkarzinoms (NSCLC) war einzig eine palliative Bestrahlung der Metastasengebiete und systemische Chemotherapie zur Schmerzsymptomatikreduktion am Auge sowie der Versuch einer systemischen Erkrankungskontrolle möglich.AbstractBackgroundIdiopathic and therapy resistant uveitis especially of unclear origin, is a diagnostic challenge for ophthalmologists. Metastases to the anterior chamber or vitreous body can occasionally mimic the clinical picture of uveitis, a variant on the usual lymphomatous masquerade syndrome. The underlying pathological pathways leading to the metastatic spread of tumor cells within the fluid compartments of the eye remain unclear.Case reportWe present an unusual case of vitreous metastases to the right eye of a patient in whom an underlying primary malignancy was unknown. After recurrent episodes of cortisone-refractive panuveitis with pseudohypopyon, a diagnostic vitreous biopsy was performed. Cytopathological examination of the vitreous sample revealed carcinoma cells with an immune profile suggestive of lung cancer metastasis. Subsequent staging investigations revealed a primary lung adenocarcinoma as well as cerebral, adrenal and osseous metastases.TherapyDue to the extent of dissemination of this non-small cell lung cancer (NSCLC), only palliative treatment including external beam irradiation and systemic chemotherapy was possible to reduce pain and to maintain vision as well as an attempt at systemic control of the disease.

Evaluation of Predictive Factors for Successful Intravitreal Dexamethasone in Pseudophakic Cystoid Macular Edema

Purpose To determine the efficacy, safety, and predictive outcome factors for intravitreal dexamethasone implant (DEX) in pseudophakic cystoid macular edema (PCME). Methods Retrospective, interventional, controlled study. Patients included had to have clinically significant PCME and have been treated with the DEX between 2012 and 2015. Charts and one-year data were selected consecutively, and efficacy and safety were abstracted. Visual acuity (VA) and central foveal thickness (CFT) were analysed. Results Nineteen patient data sets were analysed. After treatment with DEX, mean VA increased significantly by 0.2 logMAR (p = 0.034), while the mean CFT was reduced significantly by 162.79 μm (p < 0.001). Five patients receiving a combination of DEX/bevacizumab have not experienced a higher mean VA gain or CFT reduction compared to fourteen patients receiving DEX alone. Decision rules, when to combine DEX with bevacizumab, have not been defined before the study. Only posttreatment VA gains in the nonhypertensive subgroup (n = 11) were significantly better (p = 0.026). Analysis of data from diabetes patients (n = 4) versus nondiabetics yielded no significant differences in efficacy. There have been no adverse events within follow-up time. Conclusion The use of DEX in PCME showed significant improvements in VA and CFT. The VA seems to show greater improvements in patients without hypertension.

Untypische Masquerade einer okulären Karzinommetastase@@@Unusual masquerade of an ocular carcinoma metastasis

BACKGROUND Idiopathic and therapy resistant uveitis especially of unclear origin, is a diagnostic challenge for ophthalmologists. Metastases to the anterior chamber or vitreous body can occasionally mimic the clinical picture of uveitis, a variant on the usual lymphomatous masquerade syndrome. The underlying pathological pathways leading to the metastatic spread of tumor cells within the fluid compartments of the eye remain unclear. CASE REPORT We present an unusual case of vitreous metastases to the right eye of a patient in whom an underlying primary malignancy was unknown. After recurrent episodes of cortisone-refractive panuveitis with pseudohypopyon, a diagnostic vitreous biopsy was performed. Cytopathological examination of the vitreous sample revealed carcinoma cells with an immune profile suggestive of lung cancer metastasis. Subsequent staging investigations revealed a primary lung adenocarcinoma as well as cerebral, adrenal and osseous metastases. THERAPY Due to the extent of dissemination of this non-small cell lung cancer (NSCLC), only palliative treatment including external beam irradiation and systemic chemotherapy was possible to reduce pain and to maintain vision as well as an attempt at systemic control of the disease.ZusammenfassungHintergrundEine therapieresistente Uveitis, gerade mit unklarer Genese, stellt eine diagnostische Herausforderung für den Augenarzt dar. Eine Karzinommetastasierung in die Vorderkammer oder den Glaskörper als Ursache darf in diesem Zusammenhang als absolute Rarität angesehen werden. Die Pathologie der Tumorstreuung, die zu einem solchen Masquerade-Syndrom führt, ist bis heute weitestgehend unbekannt.FallberichtIn dem hier vorgestellten, ungewöhnlichen Fall einer Glaskörpermetastase am rechten Auge kam es zur Diagnosestellung eines bislang nicht bekannten, aber weit fortgeschrittenen, metastasierten Bronchialkarzinoms. Nach wiederholt auftretendem Bild einer massiven Panuveitis mit eindrucksvollem Pseudohypopyon, trotz intensiver Kortisontherapie, stellte sich der Verdacht auf ein intraokulares Malignom. Die zytopathologische Untersuchung einer Glaskörperprobe sicherte die Diagnose einer vitrealen Bronchialkarzinommetastase. Anschließende Staginguntersuchungen fanden ein nichtkleinzelliges Adenokarzinom als Primarius in der Lunge. Des Weiteren bestanden multiple zerebrale, adrenale und ossäre Metastasen.BehandlungAufgrund des metastasierten nichtkleinzelligen Bronchialkarzinoms (NSCLC) war einzig eine palliative Bestrahlung der Metastasengebiete und systemische Chemotherapie zur Schmerzsymptomatikreduktion am Auge sowie der Versuch einer systemischen Erkrankungskontrolle möglich.AbstractBackgroundIdiopathic and therapy resistant uveitis especially of unclear origin, is a diagnostic challenge for ophthalmologists. Metastases to the anterior chamber or vitreous body can occasionally mimic the clinical picture of uveitis, a variant on the usual lymphomatous masquerade syndrome. The underlying pathological pathways leading to the metastatic spread of tumor cells within the fluid compartments of the eye remain unclear.Case reportWe present an unusual case of vitreous metastases to the right eye of a patient in whom an underlying primary malignancy was unknown. After recurrent episodes of cortisone-refractive panuveitis with pseudohypopyon, a diagnostic vitreous biopsy was performed. Cytopathological examination of the vitreous sample revealed carcinoma cells with an immune profile suggestive of lung cancer metastasis. Subsequent staging investigations revealed a primary lung adenocarcinoma as well as cerebral, adrenal and osseous metastases.TherapyDue to the extent of dissemination of this non-small cell lung cancer (NSCLC), only palliative treatment including external beam irradiation and systemic chemotherapy was possible to reduce pain and to maintain vision as well as an attempt at systemic control of the disease.

Lymphome der okulären Adnexe@@@Lymphoma of the ocular adnexa

ZusammenfassungHintergrundLymphome der okulären Adnexe stellen ein heterogenes onkologisches Krankheitsbild dar. ZielsetzungZiel des Beitrags ist die Vermittlung der wesentlichen interdisziplinären diagnostischen Schritte vor der zielgerichteten individuell angepassten Therapie.DiagnostikIn der okulären Diagnostik erfolgt je nach Lokalisation am Augenlid, in der Bindehaut oder in der Orbita eine Befunddokumentation mit einem Foto oder mit einer weiterführenden Bildgebung. Funktionelle Aspekte spielen gerade bei Befunden im Sehnervenbereich eine Rolle und sollten mit untersucht werden. Die histopathologische Begutachtung sichert die Diagnose und charakterisiert die Lymphomeigenschaften näher. Ein systemisches „Staging“ nach weiteren Lymphommanifestationen im Körper sowie Blut- und Knochenmarkuntersuchungen auf das Vorliegen einer Leukämie runden die Diagnostik ab.TherapieSowohl die Bestrahlung als auch eine lokale oder systemische Chemotherapie bzw. eine Antibiotikagabe kommen nach einer möglichen chirurgischen Exzision bei der Behandlung des Lymphoms infrage. Stadieneinteilungen wie die Ann-Arbor-Klassifikation für Lymphome bzw. die TNM-Klassifikation für Lymphome der okulären Adnexe sowie die Leitlinien der Deutschen Gesellschaft für Hämatologie und Medizinische Onkologie sind wegweisend für die Entscheidung einer individuell angepassten Therapie, die durch eine psychoonkologische Betreuung ergänzt werden sollen.AbstractBackgroundLymphomas of the ocular adnexa are heterogeneous and demonstrate a wide range of clinical, histological, immunohistochemical and molecular genetic characteristics. AimThe aim of this article is to give an overview of the interdisciplinary diagnostics and individually adapted lymphoma subtype-based therapy.DiagnosticsDepending on the lymphoma localisation, i.e. whether in the eyelid, the conjunctiva or in the orbit, a photograph or a radiological scan is required to record the tumor extent. Visual function is more likely to be impacted when the lymphoma arises in the posterior orbit, close to the optic nerve and imaging diagnostics are therefore necessary. Histological investigations are essential for confirming the lymphoma diagnosis and give information about the particular subtype, which in turn will determine subsequent patient management, Clinical staging investigations for determining the systemic extent of the lymphoma manifestation (e.g. imaging, blood analyses as well as bone marrow biopsy) are mandatory.TherapyExternal beam radiation, local and systemic chemotherapy or in some cases antibiotics are treatment options after surgical excision in isolated ocular adnexal lymphoma. The TNM classification of the American Joint Committee on Cancer or the Ann Arbor staging system, as well as the guidelines of the German Society of Hematology and Medical Oncology are all tools to aid the choice of the appropriate individually adapted therapy for systemic disease, which includes psycho-oncological care.AbstractSection StopBACKGROUND Lymphomas of the ocular adnexa are heterogeneous and demonstrate a wide range of clinical, histological, immunohistochemical and molecular genetic characteristics. AIM The aim of this article is to give an overview of the interdisciplinary diagnostics and individually adapted lymphoma subtype-based therapy. DIAGNOSTICS Depending on the lymphoma localisation, i.e. whether in the eyelid, the conjunctiva or in the orbit, a photograph or a radiological scan is required to record the tumor extent. Visual function is more likely to be impacted when the lymphoma arises in the posterior orbit, close to the optic nerve and imaging diagnostics are therefore necessary. Histological investigations are essential for confirming the lymphoma diagnosis and give information about the particular subtype, which in turn will determine subsequent patient management, Clinical staging investigations for determining the systemic extent of the lymphoma manifestation (e.g. imaging, blood analyses as well as bone marrow biopsy) are mandatory. THERAPY External beam radiation, local and systemic chemotherapy or in some cases antibiotics are treatment options after surgical excision in isolated ocular adnexal lymphoma. The TNM classification of the American Joint Committee on Cancer or the Ann Arbor staging system, as well as the guidelines of the German Society of Hematology and Medical Oncology are all tools to aid the choice of the appropriate individually adapted therapy for systemic disease, which includes psycho-oncological care.

Diagnostik und Therapie des primären vitreoretinalen Lymphoms@@@Diagnostics and treatment of primary vitreoretinal lymphoma

ZusammenfassungHintergrundBeim primären vitreoretinalen Lymphom (PVRL), einem seltenen hämatopoetischen okulären Tumor, handelt es sich meist um ein großzelliges diffuses B-Zell-Lymphom. Das PVRL, das früher auch als primäres intraokulares Lymphom (PIOL) bezeichnet wurde, ist eine Unterart des primären ZNS-Lymphoms (PZNSL). DiagnostikDie Diagnose gestaltet sich häufig sehr schwierig, da klinisch eine intermediäre bzw. posteriore Uveitis imitiert wird. Dies wird auch als sog. „Masquerade-Syndrom“ bezeichnet. Notwendig für die endgültige Diagnose sind immunhistochemische, zytologische, pathologische und auch molekularpathologische Untersuchungen der operativ gewonnenen okulären Gewebe. Als hilfreich hat sich auch die Zytokinbestimmung (Interleukin-10) entweder im Vorderkammerpunktat oder im Glaskörpergewebe erwiesen. TherapieLeider zeigen sich die Therapien, die erfolgreich in der Behandlung der systemischen Lymphome sind, weniger effektiv in der Therapie des PVRLs und des PZNSLs. Daher werden heutzutage beim PVRL, zeitweise in Kombination mit einer Radiatio, aggressive chemotherapeutische Substanzen wie Methotrexat und Rituximab appliziert. Hierbei werden beide Chemotherapeutika sowohl systemisch als auch lokal verabreicht. Lediglich beim monokularen PVRL wird die alleinige intravitreale Gabe einer dieser Substanzen diskutiert. Bisher herrscht noch Uneinigkeit, ob die lokale Monotherapie ausreichend ist. Das PVRL reagiert meist sehr gut auf die initiale Therapie, jedoch kommt es im Verlauf häufig zu Rezidiven wie auch zum Auftreten einer ZNS-Manifestation, welche die Prognose quoad vitam deutlich limitieren.AbstractBackgroundPrimary vitreoretinal lymphoma (PVRL) is a rare ocular lymphoid malignancy, mostly a diffuse large B-cell lymphoma. The PVRL, previously called primary intraocular lymphoma (PIOL), is a subset of primary central nervous system lymphoma (PCNSL). DiagnosisThe diagnosis of PVRL is often difficult as it often mimics chronic intermediate or posterior uveitis; therefore, PVRL requires various procedures for the diagnostics, e.g. immunohistochemistry, cytology, pathology, molecular pathology and cytokine analysis (interleukin 10) after surgically obtaining ocular specimens.TherapyTreatment forms that are effective for systemic lymphomas have not been reliably successful for PVRL and PCNSL. Current management of PVRL consists of chemotherapy, such as methotrexate or rituximab, possibly combined with external beam radiation whereby both chemotherapeutic agents are administered systemically as well as intravitreally. Intravitreal treatment alone is recommended solely in the case of monocular PVRL, which is highly controversial. A PVRL usually responds well to initial treatment; however, relapse rates and CNS involvement are high, resulting in a poor prognosis and limited survival.