Primary Ocular Lymphoma Research Articles

Incidence and Survival Rates of Primary Ocular Lymphoma: A Population-Based Study.

Incidence and Survival Rates of Primary Ocular Lymphoma: A Population-Based Study.

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

BackgroundOcular mucosa-associated lymphoid tissue (MALT) lymphoma involving orbit and conjunctiva usually has an indolent clinical course with “salmon patch” mass as typical presentation. This study is to report a series of rare cases and investigate the clinical and pathological features of ocular MALT lymphoma that involved uveal tissue primarily and presented as posterior scleritis.MethodsThis retrospective, observational study was conducted at Beijing Tongren Hospital. From 2018 to 2020, 3 cases of 3 eyes (2 female patients and 1 male patient) with ocular MALT lymphoma that involved uveal tissue primarily and presented as posterior scleritis were included in the study. All patients had complaints of red eyes with blurred vision. The average age was 56.33 ± 2.08 years old and the average time from initial diagnosis to pathological diagnosis was 3.00 ± 1.73 months. Ophthalmic examinations including best-corrected visual acuity (BCVA), intraocular pressure (IOP), slit lamp microscope examinations, fundus photography, B-scan ultrasonography, ultrasound biomicroscope (UBM), optical coherence tomography (OCT), fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were conducted. Systemic workups including orbital magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT) and blood autoimmune antibody tests were also conducted. Pathological tissue from patients were obtained through surgeries. Biopsy examinations were performed to accurately determine pathological diagnosis. All the information of clinical, imaging and pathological changes were collected and analyzed.ResultsAt the initial diagnosis, the BCVA of involved eyes decreased seriously while the IOP were normal. All involved eyeball showed extensive hyperemia and local thickening in the wall of eyeballs. B-scan ultrasonography showed mass with abundant blood and irregular cysts inside the eyeball wall and in the retrobulbar orbit, surrounding the ocular wall and optic nerve. UBM showed solid lesions with low and medium echo under the conjunctiva and inside the ciliary body of 2 cases. OCT showed posterior polar wavy rise of RPE and local neuroepithelial detachment in all cases. FFA and ICGA showed vascular abnormalities (patch-like strong fluorescence and fluorescence leakage) and local thickening in retina and choroid (Rectangle-like weak fluorescence below the macula). The posterior wall of the eyeball was thickened and enhanced in MRI. PET-CT also showed thickening of posterior wall of eyeballs and increased metabolic activity but there was no sign of autoimmune disease. All patients were diagnosed as MALT lymphoma through pathologic examinations of biopsy tissue.ConclusionsThe onset of primary ocular MALT lymphoma in uvea is hidden. The early clinical manifestations are lack of specificity and misleading. B-scan ultrasonography has characteristic manifestations and is valuable in diagnosis. However, pathological diagnosis through tissue biopsy is irreplaceable.

European Association of Neuro-Oncology (EANO) guidelines for treatment of primary central nervous system lymphoma (PCNSL).

The management of primary central nervous system (PCNSL) is one of the most controversial topics in neuro-oncology because of the complexity of the disease and the limited number of controlled studies available. In 2021, given recent advances and the publication of practice-changing randomized trials, the European Association of Neuro-Oncology (EANO) created a multidisciplinary task force to update the previously published evidence-based guidelines for immunocompetent adult patients with PCNSL and added a section on immunosuppressed patients. The guideline provides consensus considerations and recommendations for the treatment of PCNSL, including intraocular manifestations and specific management of the elderly. The main changes from the previous guideline include strengthened evidence for the consolidation with ASCT in first-line treatment, prospectively assessed chemotherapy combinations for both young and elderly patients, clarification of the role of rituximab even though the data remain inconclusive, of the role of new agents, and the incorporation of immunosuppressed patients and primary ocular lymphoma. The guideline should aid the clinicians in everyday practice and decision making and serve as a basis for future research in the field.

Case study on the clinical and anatomopathological descriptions of dog with primary uveal lymphoma

Intraocular lymphoma is a malignant neoplasm, which commonly affects the uveal tract of dogs, most of the time, a consequence of the multicentric by the hematogenous spread of neoplastic lymphocytes. The symptoms are varied and the chronicity can cause serious eye problems such as glaucoma and loss of visual acuity. In view of the unusual occurrence of primary ocular lymphoma in dogs, the objective was to discuss the case of a dog with uveal involvement, presenting conjunctival congestion, increased episcleral vessels, hypopyon, hyphema and increased intraocular pressure. After seven days of conservative treatment, symptoms worsened, especially the intraocular pressure. The tutor returned only two months after the consultation with the sonographic report of neoplasia in the uvea. There was an increase in the ocular bulb, vascularization and corneal edema, posterior synechia, discomfort, loss of visual acuity and neoformation in the iris. Was opted for enucleation and the histopathology suggested large cell lymphoma in the uveal tract. Two years after surgery, no metastases were observed. It is concluded that lymphoma can affect the uveal tract primarily in dogs, demonstrating different clinical signs and, in addition, early diagnosis and correctly instituted treatment favor quality of life and survival of the affected.

Unmasking of infectious retinitis and intraocular lymphoma in patients after uneventful dropless cataract surgery.

In this case series, 3 unique cases of severe immune-mediated pathologies (primary ocular lymphoma, toxoplasmosis chorioretinitis, and syphilitic retinitis) after dropless cataract surgery with intraocular steroid injection are reported. Patient immune status and subsequent local immunosuppression after dropless cataract surgery played roles in the worsening of these pathologies. This report demonstrates the need for vigilance when administering immunosuppressive agents locally during cataract surgery.

Primary Ocular Lymphoma: A SEER Database Analysis of Patterns of Involvement and Outcomes

BACKGROUND: Malignant lymphomatous involvement of the eye and its surrounding structures is a rare phenomenon. Due to the unique location, non-specific symptomatology, limitations for invasive procedures, physician experience, and significant effect on the quality of life related to vision, it poses many challenges in the diagnosis and management. Ocular involvement can be primary or secondary in lymphoma. Primary ocular lymphoma, based on its clinical and pathological characteristics, has been divided into 2 main groups: those involving the orbit and adnexal tissues including lacrimal gland, conjunctiva and cornea and, those affecting intra-ocular structures mainly vitreous and retina. We aim to evaluate the SEER database to assess patterns and outcomes in patients with primary ocular involvement with lymphoma. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify patients (≥18 years) diagnosed with primary ocular lymphomas between 1975 and 2016. B-cell and T-cell lymphomas with stage I disease and primary site labeled as ocular (orbital, retina, conjunctiva, cornea, choroid, ciliary, lacrimal gland) were included. Treatment effect (surgery, radiation, systemic therapy) was analyzed when available.Overall survival (OS) was estimated using the Kaplan-Meier method and compared using the Log-Rank test. Cox proportional hazards models were used for adjusted survival analyses. RESULTS: We identified 3610 patients with primary lymphoma of the eye and adnexa. The median age was 66 years (18-103). 70% of the patients were Caucasians, followed by Hispanic (10%). Slight female preponderance was noted (55%). Most common site of involvement was orbit (n = 2063; 57%) followed by conjunctiva (n = 986; 27.3%). 8% (n = 292) of the patients were noted to have bilateral involvement. MALT (n = 2208; 61.2%) was the most common histology followed by DLBCL (n = 576; 16%) and follicular lymphoma (n = 519; 14.4%). Other histologies were rare (see Table 1). Most common treatment modality was radiation (n = 2117; 58.6%). Systemic therapy was utilized in only about 25% of the patients. Radiation treatment was associated with improved survival HR = 0.44 (0.23-0.83 95%CI, p = 0.01). Interestingly, patients that received systemic therapy had worse survival in univariate analysis (p<0.05) but was not significant in multivariate analysis. Site-specific survival suggested conjunctiva had the best median OS (19 years [16.3 - 21.7 95% CI]), followed by orbital and lacrimal gland involvement (median OS 12 years [10.92-13.23 95% CI]) - Figure 1. Retina (median OS 1.9 years) and choroid/ ciliary body (median OS 6.3 years) involvement were associated with shorter survival (p<0.05). DISCUSSION: Ocular lymphoma is a rare occurrence. Overall MALT was the most common subtype followed by DLBCL and FL. Very few instances of Burkitt's lymphoma, T-cell, and CLL were identified. Site-specific data in our subset, albeit with low numbers, suggest worse survival in the ciliary body and choroid similar to what is seen within vitreoretinal involvement. Due to small numbers in our subset, this requires confirmation in a larger dataset to identify potential change in management similar to primary vitreoretinal lymphoma, which is now recognized and managed as primary CNS lymphoma. Patients receiving systemic therapy showed a trend towards worse survival, which may be explained by a selection bias for more aggressive disease. Our study highlights the small but relevant prevalence of primary ocular lymphomas and reports the interesting observation of different survival patterns based on the site of involvement. Disclosures Yazbeck: Celgene: Membership on an entity's Board of Directors or advisory committees; Gilead Sciences: Research Funding; Seattle Genetics: Membership on an entity's Board of Directors or advisory committees.

Linfoma de Burkitt orbitario en una paciente de 3 años de edad

Los linfomas primarios de ojo y anexos oculares representan el 1.5% de los linfomas no Hodgkin en la población anglosajona. Uno de sus tipos histológicos, el linfoma de Burkitt en la localización orbitaria, es una condición muy poco frecuente. Presentamos el caso de una paciente femenina de 3 años de edad con aparición de masa a nivel del parpado inferior izquierdo asociada a proptosis, con 15 días de evolución, a quien se le realizó una resonancia magnética nuclear de órbitas que confirmó procesos neoproliferativos. Posteriormente, fue sometida a una resección quirúrgica. Los estudios de inmunohistoquímica confirmaron el diagnóstico de linfoma de Burkitt. La importancia del reporte de este caso radica en que muestra que este padecimiento se debe tener en cuenta dentro de los posibles diagnósticos diferenciales cuando se presenta la aparición de masas orbitarias.

Limited Role of Workup Including PET-CT, Esophagogastroduodenoscopy and Bone Marrow Biopsy in Staging of Ocular Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT) Type

BackgroundLymphoma of the ocular adnexa accounts for approximately 1 to 2% of non-Hodgkin lymphomas and the most common subtype is extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type (80-90%). Ocular MALT lymphoma is mostly localized disease with indolent clinical course. The workup for non-gastric MALToma is usually done similar to the workup for other non-Hodgkin lymphoma. However, as there are paucity of data in the role of PET, esophagogastroduodenoscopy and bone marrow biopsy,weaimed toinvestigate these issues in patients with ocular MALT lymphoma.Patients and MethodOne hundred thirty patients diagnosed as ocular MALT lymphoma between Jan 2001 and Dec 2015 at a single tertiary hospital were included in this retrospective analysis. We retrospectively reviewed clinical characteristics, diagnosis, natural history of disease, treatment and outcomes.ResultThe median age of all patients was 48 years (range, 20-70 years). Fifty-nine (45%) were male patients. The majority of patients (n=87, 67%) presented with stage I disease, followed by bilateral involvement, defined as stage I+I (n=21, 16%), stage IV (n=16, 12%), and stage II (n=6, 5%). Nodal involvement was present in 8% (n=10), and 12% (n=16) of patents accompanied extra-nodal involvement other than orbits at initial diagnosis. Positron emission tomography (PET) had low sensitivity in detecting primary orbit lesions (n=44/120, 37%). Moreover, only in 3% of cases (n=4), PET imaging after CT scans on neck, chest, abdomen and pelvis could provide additional clinically meaningful information that led to upstaging. Accompanying gastric MALToma was found in 2% (n=2/82) of patients by EGD. Those two patients all survived until last follow-up. Bone marrow (BM) involvement was found in 6% (n=7/120). None of them had clinically significant cytopenia. With a median follow-up period of 74 months, 5-year overall survival (OS) rate was 99% in all patients. There was no significant difference in 5-year OS rates according to BM involvement (100% with and 99% without BM involvement).ConclusionPET-CT, EGD and BM biopsy has limited role in staging workup of primary ocular MALT lymphoma. Those workups should be performedonly in selected cases. DisclosuresNo relevant conflicts of interest to declare.

Primary central nervous system lymphomas.

Primary central nervous system lymphoma (PCNSL) is an aggressive B cell lymphoma that occurs in immunocompetent and immunosuppressed patients. Primary ocular lymphoma represents an important variant of this disease. The outcome for patients with PCNSL is rapidly improving with new treatment strategies. This review focuses on recent advances, investigations, and controversies in the management of these increasingly important malignancies. The incidence of PCNSL is rising in immunocompetent patients over the age of 60 years for unexplained reasons. However, the incidence of AIDS-related PCNSL, which is related to Epstein-Barr virus infections, has fallen with the institution of highly active antiretroviral therapy. During the past decade, standard therapy has evolved from cranial irradiation to high dose methotrexate-based regimens. This shift in therapy has been associated with an increase in the median survival of patients from 1 year to longer than 3 years, and with an equally striking reduction in neurotoxicity if whole brain radiation is avoided. Optimal therapeutic results require careful attention to opportunistic infections, thromboembolic disease, and renal toxicities associated with high dose methotrexate. Overall, PCNSLs are increasing in frequency and the results of therapy for this disease have dramatically improved during the past decade. Many important therapeutic questions remain unanswered. These include the optimal dose, frequency, and duration of methotrexate, as well as the roles of additional systemic chemotherapy drugs, intrathecal therapy, and cranial irradiation. Further progress in this uncommon disease will depend on entering patients into carefully designed multicenter clinical trials to build upon the significant advances that have been made.

Primary Diffuse Large B-Cell Lymphoma Localized to the Lacrimal Sac: A Case Presentation and Review of the Literature.

We report a rare case of diffuse large B-cell lymphoma (DLBCL) of the lacrimal sac in a 50-year-old male. The incidence of primary ocular lymphoma is low and it is considered a rare disease. Moreover, reports of ocular DLBCL are uncommon and the disease remains poorly characterized. Our patient presented for management of osteomyelitis and was incidentally found to have a painless swelling and cyst around his right eye. A PET/CT scan revealed hypermetabolic activity within the lacrimal sac and a subsequent excisional biopsy of the mass yielded histopathology consistent with DLBCL. Consequently, the patient underwent treatment with R-CHOP therapy. The patient responded well to chemotherapy with a substantial shrinkage in tumor burden and the disease remained localized. Herein, we present a rare case of primary ocular lymphoma, highlight the importance of early diagnosis, and review current treatment modalities.

Dose Response to Radiation Therapy for Primary Ocular Lymphomas

Lympho proliferative lesions are the most common primary orbital malignancies. Primary lymphoma of the orbit and ocular adnexa accounts for 59 years [4]. Orbital Lymphoma (OL) commonly presents with proptosis and is typically a painless, progressive orbital lesion; however, the conjunctival lesions appear clinically as fleshy salmon patch lesions [5,6]. The treatment options are surgical excision, chemotherapy, Radiation Therapy (RT), or combination of chemoradiation. Radiation therapy alone is the most effective treatment modality for the orbital lymphoma with excellent local tumor control [7-12]. The local and systemic relapse rate was reported to be less after treatment of OL by RT due to the indolent nature of the MALT variants. Different radiation techniques and doses (with or without adjuvant chemotherapy) were used by the several authors depending on the extent of the disease and severity. Kennerdell et al. reported 100% complete response of the lymphoid lesions of the orbit and ocular adnexa with low-dose RT (24-25.5 Gy) with very few acute and chronic side effects [8]. Several authors advocated 30-40 Gy for low to intermediate grade lymphomas with good local control and better 5 year survival. In a retrospective chart review of 31 cases of orbital MALT lymphoma, Le et al. observed a complete local control of the tumor with an overall 10 year survival of 73%. However, they did not find a significant difference in the outcomes among patients treated with RT dose of ≤ 30 Gy versus the >30 Gy and recommended that RT dose of 30-30.6 Gy provides excellent local control of tumor with minimal long-term side effects [9]. Galieni et al. observed complete response to the radiotherapy in seven patients of low-grade MALT lymphoma treated with RT dose of 36-40 Gy over a median follow-up of 51 months. However, among the patients treated with chemotherapy, local relapse was observed in 3/15, which resolved completely to the local irradiation [13]. In a multi-institutional, retrospective study by Uno et al. complete tumor response was seen in 26 patients (mean RT dose 36 Gy) and partial response in 20 others (mean RT dose 37.5 Gy) [11]. The local tumor recurrence was noted in 6 of the patients with partial or no response. They concluded that the age ( 60 years), gender, location (conjunctiva versus others), laterality (unilateral versus bilateral) or the dose of radiation therapy (≤ 30 Gy versus >30 Gy) did not contribute to the local tumor recurrence and felt the initial tumor response to RT influenced the probability of local recurrence. The overall 5-year survival rate for all the patients was 91% with a median follow-up of 46 months. Fung et al. noted 4 cases with local recurrence of the 98 cases of MALT lymphoma. Three of the 4 had stage I disease (primary orbital tumor treated with RT dose of 30 Gy) [7]. The systemic relapse-free survival rate among the stage I MALT lymphoma patients which comprise major group in their study was 75% at 5 years and the overall 5-year survival rate was 95%. However for the stage III-IV MALT lymphoma in the same study the 5 year disease specific survival rate was 49% and the overall survival rate was 39%. To summarize, excellent local tumor control rates were observed with radiation therapy with doses 2430 Gy in most studies.

A case of oculo‐cerebral B‐cell lymphoma in a cat

To describe a case of a cat with primary B-cell lymphoma affecting the eye and brain and which shared features similar to oculo-cerebral lymphoma in humans. A 13-year-old castrated male Persian cat presented with clinical signs of anterior uveitis and increased intraocular pressure (IOP) in the left eye (OS). A complete diagnostic work-up was declined, and left-eye enucleation was performed. The globe was submitted for histopathology. One week after surgery, the cat became inappetent, hypothermic, and aggressive. Euthanasia was requested by the owner, and a necropsy was permitted. Histopathology of the enucleated globe revealed an extensive neoplastic infiltration consistent with large-cell lymphoma, affecting the anterior uvea, neuroretina and optic nerve. At necropsy, all organs were unremarkable except for the brain, where there was a neoplastic cell population consistent with that described in the left eye, infiltrated and expanded meninges, and perivascular spaces. Immunohistochemically, the neoplastic cells were positive for B-cell marker (CD20) and negative for T-cell marker (CD3). Histology and immunophenotyping suggested a diagnosis of primary central nervous system and ocular large B-cell lymphoma. The lymphoma in this cat resembled oculo-cerebral lymphoma in humans, sharing similar clinical features and histopathological findings, including the perivascular pattern of neoplastic cell infiltration. To the best of the authors' knowledge, this is the first description of a primary oculo-cerebral B-cell lymphoma in a cat.

Supposed primary conjunctival lymphoma in a dog

A 7-year-old, male mixed-breed dog was presented for evaluation of a well-delineated, pink, oval and slightly firm mass with a smooth regular surface that was expanding approximately 60% of the lower eyelid conjunctiva and the lateral canthus. The dog had a supposed primary B-cell lymphoma at the temporal canthus of the upper eyelid conjunctiva of the same eye that had been removed approximately 3 years earlier. No metastases were detected at either presentation. Histologically, the conjunctival lamina propria was effaced by a well-delineated, unencapsulated and expansile highly cellular neoplasm composed of sheets of round cells that were immunohistochemically positive for CD20 and CD79a negative for CD3. Based on the microscopic and immunohistochemical findings, a diagnosis of supposed primary B cell conjunctival lymphoma was made. Primary ocular and adnexal ocular lymphomas in dogs are rarely reported and their behavior is poorly characterized. Further tumor recurrence was not observed one year post operatively. This case was considered unusual because of its conjunctival involvement and the clinical course with recurrence after three years of a surgical therapeutic procedure.

Primary Lymphoma of the Ocular Adnexa (Orbital Lymphoma) and Primary Intraocular Lymphoma

Lymphomas of the orbit and eye are rare conditions that should be treated as separate entities due to the differences in presumed aetiology, investigations, management and outcomes. Orbital lymphoma is most often of low-grade histology; thyroid eye disease may predispose and chlamydial infection has been suggested as a trigger. Commonly, stage IE, in most cases, can be managed with radiotherapy alone using either a kilovoltage portal for conjunctival disease or a wedged pair of megavoltage beams for more infiltrative disease to a dose of 30 Gy in 15 fractions over 3 weeks. However, medical therapy is being investigated, including a rituximab-only approach for conjunctival-only presentations. The cure rate for stage IE disease is very high. In contrast, primary ocular lymphoma is often of high-grade histology, in particular diffuse large B-cell lymphoma, and can be regarded as one end of primary central nervous system lymphoma — both eyes and brain being at risk. Immunosuppression predisposes to the disease. Management consists of an initial high-dose chemotherapy regimen with methotrexate. In most cases, this should be followed by radiotherapy to the whole brain and globes to a dose of 30–36 Gy with a boost to bulk/presenting disease. Cure rates are rarely above 50%.

Curative Radiation Therapy for Primary Ocular Lymphomas: Demonstration of Dose Response

Curative Radiation Therapy for Primary Ocular Lymphomas: Demonstration of Dose Response

Curative radiation therapy for primary ocular lymphomas: Demonstration of dose response.

e18503 Background: Orbital lymphomas (OL) are rare and account for less than 1% of all cases of non-Hodgkin lymphoma. Radiotherapy (RT) alone provides good local control rates (97-100%) for extranodal marginal zone lymphoma (MALT) of the orbit. We report the outcomes of OL patients treated at FCCC. Methods: Between 1991 and 2008, 23 patients (28 eyes, 5 bilateral, 20%) treated at FCCC with local radiation therapy for orbital lymphoma were reviewed. Treatment consisted of 24-36 Gy,1.5-2.5 Gy per fraction (median 30 Gy in 20 fractions) of RT to the orbit using 6 MV photons. Patient, tumor and treatment related factors including age, stage, histology, type of treatment, RT details and late side effects were analyzed for the local control rates (LC), relapse free and overall survival (OS) by number of patients and orbits independently. Results: The median follow-up was 27 months (range: 1-123 months). The median age was 72 years (range: 37-87), 11 were females, 12 males. Most common presenting symptom was proptosis (74%). 20 patients had tumor in orbit only, 2 in conjunctiva only and 1 in both conjunctiva + orbit. Histology was MALT in 13 patients, and other low grade B cell NHL in 10. The 5-year LC and OS rates were 95%, 63% by patients and 96%, 68% by orbits respectively. The 5-year LC rate at patient level for MALToma was 91% versus 100% for other NHL histologies (p=0.3) and 100% for RT dose ≥ 30 Gy versus 75% for < 30 Gy (p = 0.04). One patient had both systemic + orbital relapse and systemic relapse was seen in two. The median interval to first relapse was 25 months (0.99- 122.37). On univariate analysis including age (≤ 70 versus > 70 years), stage (IAE vs others), histology (MALT versus others), and location of tumor (orbit vs others), there was no difference in outcomes except for RT dose (≥ 30 Gy vs < 30 Gy) for LC (p = 0.04). 4/23 patients had three lines of improvement in Snellen's visual acuity after one year of treatment. Minimal dry eye was seen in 77% patients. Five patients developed cataracts. There were no late effects like corneal ulcer, papillopathy or secondary glaucoma. Conclusions: Radiation therapy alone is the extremely effective in the curative management of primary localized orbital lymphoma. RT dose of 30 Gy provides better LC rates. No significant financial relationships to disclose.

Elevated risk for squamous cell carcinoma of the conjunctiva among adults with AIDS in the United States

Squamous cell carcinoma of the conjunctiva (SCCC) has been associated with HIV infection in equatorial Africa, but the evidence for association with HIV in developed countries, where SCCC is rarer, is controversial. We investigated the risk for SCCC and other eye cancers in the updated U.S. HIV/AIDS Cancer Match Registry Study. We calculated standardized incidence ratios (SIRs) to estimate excess risk for SCCC, primary ocular lymphoma, ocular Kaposi sarcoma (KS), and other eye tumors among 491, 048 adults (age > 15 years or older) with HIV/AIDS diagnosed from 1980 to 2004. We calculated relative proportions (per 105) to gain insight into risk factors. We identified 73 eye cancers (15 SCCC, 35 primary ocular lymphoma, 17 ocular KS, and 6 other). Overall SIRs were elevated for SCCC (SIR, 12.2, 95% CI 6.8 – 20.2), primary ocular lymphoma (21.7, 95% CI 15.1–30.2), and ocular KS (109, 95% CI 63.5–175). Risk for SCCC was elevated regardless of HIV acquisition category, CD4 lymphocyte count, and time-relative to AIDS-onset. Relative proportions of SCCC risk were highest with age ≥50 (8/105), Hispanic ethnicity (7/105), and residence in regions with high solar ultraviolet radiation (10/105). We show significantly increased incidence of SCCC among persons with HIV/AIDS in the United States. The associations with age and geography are in accord with etiological role for ultraviolet radiation in SCCC.

High-dose methotrexate combined with procarbazine and CCNU for primary CNS lymphoma in the elderly: results of a prospective pilot and phase II study

BackgroundTo improve survival of elderly patients with primary central nervous system lymphoma (PCNSL), we conducted a phase II study with high-dose methotrexate (MTX) combined with procarbazine and CCNU. To reduce neurotoxicity, whole-brain irradiation was reserved for patients not responding to chemotherapy. Patients and methodsHigh-dose MTX was applied on days 1, 15, and 30, procarbazine on days 1–10, and CCNU on day 1. Study treatment comprised up to three 45-day cycles. There was no lower limit of Karnofsky performance status (KPS). ResultsThirty patients with PCNSL (n = 29) or primary ocular lymphoma (n = 1) were included (median age 70 years, range 57–79 years). The median initial KPS was 60% (range 30%–90%). Best documented response in 27 assessable patients were 12 of 27 (44.4%) complete remissions, 7 of 27 (25.9%) partial remissions, and 8 of 27 (29.6%) disease progressions. Two patients died of probable treatment-related causes. With a median follow-up of 78 months (range 34–105), the 5-year overall survival is 33%. Eight of 30 patients (26.7%) are currently alive and well, six without signs of leukoencephalopathy. ConclusionThe combination of high-dose MTX with procarbazine and CCNU is feasible and effective and results in a low rate of leukoencephalopathy. Comorbidity and toxicity remain of concern when treating PCNSL in elderly patients.

Elevated risk for squamous cell carcinoma of the conjunctiva among adults with AIDS in the United States

Squamous cell carcinoma of the conjunctiva (SCCC) has been associated with HIV infection in equatorial Africa, but the evidence for association with HIV in developed countries, where SCCC is rarer, is controversial. We investigated the risk for SCCC and other eye cancers in the updated U.S. HIV/AIDS Cancer Match Registry Study. We calculated standardized incidence ratios (SIRs) to estimate excess risk for SCCC, primary ocular lymphoma, ocular Kaposi sarcoma (KS) and other eye tumors among 491, 048 adults (aged > 15 years or older) with HIV/AIDS diagnosed from 1980 to 2004. We calculated relative proportions (per 10(5)) to gain insight into risk factors. We identified 73 eye cancers (15 SCCC, 35 primary ocular lymphoma, 17 ocular KS and 6 other). Overall SIRs were elevated for SCCC (SIR, 12.2, 95% CI 6.8-20.2), primary ocular lymphoma (21.7, 95% CI 15.1-30.2) and ocular KS (109, 95% CI 63.5-175). Risk for SCCC was elevated regardless of HIV acquisition category, CD4 lymphocyte count and time relative to AIDS-onset. Relative proportions of SCCC risk were highest with age >or=50 (8/10(5)), Hispanic ethnicity (7/10(5)) and residence in regions with high-solar ultraviolet radiation (10/10(5)). We show significantly increased incidence of SCCC among persons with HIV/AIDS in the U.S. The associations with age and geography are in accord with etiological role for ultraviolet radiation in SCCC.

Gastric recurrence of an ocular mucosa-associated lymphoid tissue lymphoma

Extra-nodal B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type was first described by Isaacson and Wright in 1983.1 The majority of MALT lymphomas present as localized disease with an indolent clinical course.2-4 We report a rare case of primary ocular MALT lymphoma presenting as a conjunctival mass, with subsequent high-grade transformation and recurrence within the stomach after an anterior orbitotomy. Only one additional report has described gastric recurrence of an ocular MALT lymphoma.