Treatment and prognosis of primary ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma: a report from a single center / 中华血液学杂志

Objective: This study aimed to see how different initial treatment regimens affected the long-term prognosis of patients with extranodal marginal zone mucosa-associated lymphoid tissue lymphoma confining to the ocular adnexal (OAML) . Methods: Between April 2008 and April 2019, 109 patients with initial mucosa-associated lymphoid tissue confining to ocular adnexal were evaluated and followed-up, and the prognosis of various initial treatment regimens were examined. Results: A total of 36 patients underwent complete surgical resection of the lesions, and 73 patients had residual lesions after surgery, of which 37 patients chose watchful waiting, and 36 patients chose treatment. The treatment regimen included local radiotherapy and systemic treatment (chemotherapy, immunochemotherapy, the combination of radiotherapy and chemotherapy, etc.) , and no serious toxic and side effects were observed in patients receiving systemic treatment. The median follow-up time was 61 (10-142) months. The 5-year and 10-year progression-free survival (PFS) of monocular involvement patients were 78.2% and 76.0% . The 5-year and 10-year PFS rates of patients with binocular involvement were 64.4% and 23.5%. There was significant diference in PFS between patients with monocular and binocular involvement (P=0.010) . Patients who received additional treatment had higher PFS than those patients in the watchful waiting group (P=0.046) . The 5-year PFS was 71.4% and 90.1% among patients in the watchful waiting group and those who received additional treatment, whereas the 10-year PFS was 63.5% and 75.1% , respectively. Patients with OAML were still a risk of disease progression after 5 years. Conclusions: Patients with binocular involvement OAML at the start of the disease had a poor prognosis, but treatment could reduce the risk of recurrence/progression. Systemic therapy is one of the first-line treatment options for patients with OAML, who require long-term monitoring.

Complicaciones tardías de la radioterapia ocular / Late complications of the ocular radiotherapy

La radioterapia es uno de los pilares terapéuticos utilizados en la oncología, no exenta de producir daño ocular, lo que constituye un fenómeno bien establecido. La aparición de complicaciones continúa siendo la principal preocupación de la aplicación de la radioterapia, a pesar del empleo de nuevas estrategias que buscan reducir las complicaciones y mejorar el índice terapéutico. Se presenta una paciente femenina de 63 años con diagnóstico de carcinoma epidermoide de la conjuntiva, moderadamente diferenciado G-II, invasor (T3N0M0) en ojo izquierdo, que llevó tratamiento quirúrgico y radioterapia superficial complementaria. Como complicación tardía, la paciente presentó catarata radiógena, que recibió tratamiento quirúrgico, además de aneurismas conjuntivales en el área tratada(AU)

Radiotherapy is one of the therapeutic pillars used in oncology, but ocular damage may occur and this is a well-established phenomenon. The occurrence of complications remains the main concern for the application of radiotherapy, despite the use of new strategies to reduce complications and to improve the therapeutic rate. Here is a 63 years old female patient, who was diagnosed with epidermoid carcinoma of the conjunctiva, moderately differentiated G-II invader (T3N0M0) in the left eye. She was surgically treated and received supplementary superficial radiotherapy As a late complication, the patient presented with radiogenic cataract treated by surgery, in addition to conjunctival aneurysms in the treated area as well(AU)

Risk Potentiality of Frontline Radiotherapy Associated Cataract in Primary Ocular Adnexal Mucosa-associated Lymphoid Tissue Lymphoma

PURPOSE: To elucidate risk potentiality of frontline radiotherapy associated cataracts in primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML). METHODS: Data from eight consecutive patients of 41 total OAML patients who had undergone cataract surgery after frontline radiotherapy were analyzed. RESULTS: The median patient age was 46 years (range, 36 to 69 years). The median total radiation dose was 3,780 cGy (range, 3,060 to 4,500 cGy), and the mean duration from radiation irradiation to cataract surgery was 36.60 +/- 8.93 months. Preoperative lens opacification was primarily at the posterior lens subcapsule, and best-corrected visual acuity (BCVA) was 0.43 +/- 0.21. Patients underwent the phacoemulsification surgical procedure with posterior chamber intraocular lens insertion. The average BCVA improved to 0.90 +/- 0.14 after cataract surgery. Two patients underwent posterior continuous curvilinear capsulorhexis, and one had posterior capsule rupture. For posterior capsule opacification (PCO), three patients received Nd:YAG laser posterior capsulotomy after the initial surgery, and one patient is currently under consideration for laser posterior capsulotomy. CONCLUSIONS: Radiotherapy increased posterior subcapsule opacification at a relatively young age in primary OAML. Phacoemulsification was a manageable procedure without severe complications, and final visual outcomes were good. However, because after-cataracts progressed earlier than did senile cataracts, close follow-up should be considered for PCO management.

Extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type of the ocular adnexa: Retrospective single institution review of 95 patients.

Context: There are few reports on the management of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type (MALT), which are based on the experience of a single institution, as opposed to large multicenter randomized trials. Aim: The aim of this study was to report on the clinical experience of a single institution. Materials and Methods: A retrospective review was undertaken of records of 95 patients with MALT lymphoma of the ocular adnexa. Histologic diagnosis of MALT lymphoma was made according to established criteria, and clinical staging was carried out to determine treatment modalities. All patients were treated by external beam irradiation (30.6–45.0 Gy) after biopsy. Additional chemotherapy was performed in accordance with the clinical stage of the disease. All the patients were treated by the same hemato-oncologist and radio-oncologist. Results: Almost all patients showed complete response, except for four patients who showed partial response. In two of 95 patients, contralateral eye showed recurrence, and they were salvaged by additional radiotherapy. The 3-year overall survival and event-free survival rates were 100 and 97%, respectively, by Kaplan–Meier survival analysis. Moreover, there were no serious radiation-associated complications. Conclusions: Radiotherapy alone can be an important treatment modality for the local control and survival in patients with localized MALT lymphoma of ocular adnexa. Systemic chemotherapy should be considered in patients with advanced stage disease.

Plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma: case report / Plasmocitoma da órbita envolvendo glândula lacrimal com transformação secundária para mieloma múltiplo: relato de caso

The authors present a rare case of plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma in a 42-year-old woman. The lesion was surgically removed and analyzed. Histopathological examination with immunostaining revealed it to be positive for immunoglobulin G and Kappa chains, demonstrating monoclonality. However, no abnormality was observed on serum electrophoresis, skeletal survey and bone marrow aspiration. Therefore, the tumor was diagnosed solitary plasmacytoma of bone. The patient was treated with external beam radiotherapy and has remained disease free for 5 years and 6 months until 2007, when she presented a pathological fracture due to multiple myeloma. Extensive medical work-up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving orbit or ocular adnexa is needed when the diagnosis of solitary plasmacytoma of bone is suspected because treatment and prognosis are very different.

Os autores relatam um raro caso de plasmocitoma da órbita envolvendo glândula lacrimal com transformação secundária para mieloma múltiplo em uma paciente de 42 anos. A lesão foi removida cirurgicamente e o exame anatomopatológico, aliado à imuno-histoquímica, revelou positividade para imunoglobulina G e cadeias leve tipo Kappa. Após extensa avaliação sistêmica, nenhuma anormalidade foi observada, sendo feito o diagnóstico de plasmocitoma solitário ósseo. A paciente foi submetida à radioterapia, permanecendo livre da doença por 5 anos e 6 meses até 2007, quando apresentou uma fratura patológica devido à infiltração plasmocitária na coluna lombar, sendo feito diagnóstico de mieloma múltiplo. Uma vez que o plasmocitoma solitário ósseo pode ser a primeira manifestação do mieloma múltiplo, esses pacientes necessitam de adequado diagnóstico e seguimento a longo prazo, pois o tratamento e prognóstico dessas duas condições é diferente.

Spectrum of ocular surface squamous neoplasia

To describe the pattern of ocular surface squamous neoplasia [OSSN], clinical presentations, the risk factors and treatment options. An observational case series. Khyber Institute of Ophthalmic Medical Sciences, Hayatabad Medical Complex, Peshawar, from April 2003 till August 2006. The study included 36 eyes of 35 patients with biopsy-proven ocular surface neoplasia. The details of patients regarding age, gender, laterally and risk factors were entered into a specially-designed proforma. Each patient was also assessed blomtcroscoplcally for type and complications of ocular surface neoplasia. The frequency of OSSN was 0.37 among admitted hospital patients. Among 36 cases of OSSN, squamous cell carcinoma of the conjunctiva was the most common type of OSSN seen in 63.9%, followed by carcinoma in situ of conjunctiva in 25% and carcinoma in situ of cornea in 11.1%. Male patients outnumbered female [65.7% vs 34.3%] with 71.42% of patients above 60 years of age. The risk factors identified were: old age, ultraviolet B exposure and xeroderma pigmentosa. Treatment consisted of local resection with or without adjuvant therapy in 61.1%, exenteration in 30.5%, enucleation in 5.5% and chemo/radiotherapy in 2.7%. Intraocular invasion was seen in 5.5% and orbital spread in 30.5%. The frequency of OSSN was 0.37% among admitted patients. Identification of exact etiological factors will enable to formulate strategies that are likely to decrease the incidence of this disease and the associated morbidity and mortality

Experiencia de 10 años en linfomas oculares y de anexos oculares / Ten year experience with ocular lymphomas and ocular adnexae lymphomas

Antecedentes. Los linfomas oculares (LO) y de los anexos oculares (LAO) tienen una incidencia de 7- 8% de todos los tumores extraganglionares. Objetivo. Describir las características clínicas, morfológicas e inmunofenotípicas de los LO y LAO atendidos en el Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. Material y métodos. Se revisaron los expedientes de los pacientes con diagnóstico de LO y LAO de julio de 1994 a julio del 2005. Se analizaron los siguientes datos: presentación clínica, tratamiento, respuesta al tratamiento, supervivencia global, y supervivencia libre de enfermedad (SVLE). Resultados. Se analizaron 10 pacientes, 8 mujeres y 2 hombres. La media de edad fue de 50 años. La remisión completa (RC) se presentó en 8 de 10 pacientes y en los 6 pacientes con linfoma de tejido linfoide asociado a mucosas (MALT). Se encontraron dos con enfermedad refractaria, los cuales estaban en estadio IV. Conclusiones. En este estudio los LO y LAO correspondieron a 0.02% de todos los linfomas no Hodgkin (LNH) estudiados (498 casos). Los linfomas tipo MALT se presentan a edad más avanzada, se encontraron en estadios más tempranos y en todos hubo RC, con una SVLE promedio de 868 días y una media de supervivencia de 442 días.

BACKGROUND: Ocular lymphomas and ocular adnexae lymphomas (OL and OAL) constitute 7-8% of all extranodal lymphomas. OBJECTIVE: Describe the clinical, morphologic and immunophenotypic characteristics of OAL seen in our hospital. MATERIAL AND METHODS: Retrospective analysis of patient records with OL and OAL between July 1994 and July 2005. The following data was analyzed: Clinical presentation, therapy, treatment response, overall survival and disease free survival. RESULTS: Ten patients with OL and OAL were identified. Of these, 8 were women and 2 men. Median age was 50. Eight of 10 patients achieved complete remission, 6 of the 6 presenting MALT Lymphoma. Two patients with stage IV had refractory disease. CONCLUSIONS: In our series 0.02% of lymphomas were OL and OAL of a total 498 LNH. MALT lymphomas appear at a more advanced age, sixty percent of the cases were MALT lymphomas and were diagnosed during their early stages. Patients were followed during 21 months, global survival was 100%, free illness survival had a mean of 868 days and a survival median of 442 days.

Rehabilitación ocular de un niño de 2 años en el Centro de Rehabilitación de la Cara y Prótesis Bucomaxilofacial / A 2 years-old boy's ocular rehabilitation in the Face and Buccomaxillofacial Denture Rehabilitation Center

Se presenta el caso de un niño de 2 años, rehabilitado con prótesis ocular en el Centro de Rehabilitación de la Cara y Prótesis Bucomaxilofacial de la provincia Santiago de Cuba, donde fue operado y tratado con radioterapia por presentar retinoblastoma. La conducta terapéutica mejoró la apariencia física del paciente y contribuyó a su adecuado desarrollo biológico, psicológico y social

Primary lymphoma of the eye

Lymphomas in the eye tend to be localized and to have a better prognosis. We reviewed the clinical presentation, treatment outcome and complications of primary lymphomas of the eye at a single institute focusing on the clinical significance of adequate staging and the prognostic value of pathological subtypes. Twenty-four patients with lymphoproliferative lesion of the eye were treated and followed up. The orbit was the most commonly involved site. Twenty-one patients had unilateral diseases and three patients had bilateral. Histological findings revealed 22 malignant lymphomas, one inflammatory pseudotumor and one atypical lymphoid hyperplasia. Using the NCI Working Formulation, most of the malignant lymphomas were primarily low grade: diffuse small lymphocytic, 15; diffuse small cleaved three; diffuse large cell, three; and diffuse mixed, one. The stages of lymphoma were IAE in 21 patients, IBE in one patient, and IIAE in two patients. Twenty-five of the 27 treated eyes remained disease-free during the median follow-up period of 16 months. Nineteen patients received radiotherapy, five patients received chemo-radiotherapy and one patient received only surgical excision. Two patients relapsed outside the treated field. None of the patients complained of severe dry eye syndrome or cataract formation. In conclusion, after complete staging work-up, locally-confined low grade orbital lymphomas can be up to 90% controlled with radiotherapy alone. By contrast, combined treatment is advised in patients with intermediate and high grade lesions.

Radiation treatment of retinoblastoma: experience in the past two decades.

This series of 193 patients represents the results in management of retinoblastoma. In cases treated 15 years ago when definitive radiotherapy and fat soluble chemotherapy were not utilized, the results of the treatment were very disappointing. The outcome was more promising after October 1985. Even if all the patients were in the advanced stages, we had a promising outcome, so effective modality of treatment is the most important prognostic factor. The other factors that influence the prognosis include the time for initial treatment, the time for starting the postoperative radiation and the efficiency of the radiation treatment. The follow-up period of 2 years after treatment is adequate to predict the outcome.

Tumores intra-oculares / Intraocular tumours

O autor apresenta a sua casuística de tumores oculares demonstrando a possibilidade do tratamento conservador nos casos específicos. Em 13 pacientes foram usadas placas radioativas contendo cobalto e suturadas à esclera. O autor discute os resultados

Duplo padräo de regressäo no retinoblastoma / Double regression pattern in retinoblastoma

O retinoblastoma é suscetível de apresentar regressäo espontânea ou pós-terapia espontânea ocorre em 1-2% dos casos, sendo unilateral e unifocal geralmente. A regressäo após o uso de radioterapia classifica-se em quatro tipos diferentes conforme o aspecto morfológico. Apresentamos um paciente acompanhado no Serviço de Oftalmologia do Hospital de Clínicas de Porto Alegre, com 3 anos de idade, no qual foi diagnosticado retinoblastoma no olho esquerdo, aos quatro meses de idade, sendo enucleado neste momento. Juntamente, observou-se no olho contralateral duas massas tumorais, caracterizando o estagio Ia, de acordo com a classificaçäo de Howarth. Após dois ciclos de radioterapia e dois protocolos de quimioterapia realizados intercaladamente, no período de três anos, esses focos tumorais apresentaram sinais claros de regressäo diferentes entre si: massa tumoral periférica com padräo tipo I e outra, paramacular, com padräo tipo II de regressäo. Salientamos a coexistência de dois tipos distintos de regressäo tumoral pós-terapia no mesmo olho, fato raramente descrito na literatura pesquisada

Retinoblastoma: tratamento conservador versus estádio da lesäo / Retinoblastoma: globe saving treatment versus classification

O trabalho apresenta os resultados obtidos no tratamento de 44 olhos portadores de retinoblastoma, com a conservaçäo do globo ocular. Utilizou-se a radioterapia, a crioterapia e a fotocoagulaçäo pela luz de xenônio. A eficiência destes métodos foi tanto melhor quanto menos avançado era o estádio da doença. A tentativa de tratamento conservador quando mal sucedida pode piorar o prognóstico de vida do paciente. O uso da radioterapia pode induzir tumores secundários em portadores de retinoblastoma