Clinical characteristics and treatment outcomes of secondary ocular adnexal lymphoma.

BACKGROUND: To describe the clinical characteristics and treatment outcomes of secondary ocular adnexal lymphoma (OAL), and emphasise the impact of timely biopsy and systemic evaluation on the diagnosis and treatment. METHODS: The data of patients with secondary OAL in our hospital from January 2010 to June 2021 were retrospectively reviewed. RESULTS: A total of 54 patients (30 men and 24 women) were included in the study. The mean age at presentation was 60 years (median 62 years; range 37-83 years). The main symptoms included ocular mass (74%), periorbital swelling (43%), and proptosis (39%). The main histopathological types were mucosa-associated lymphoid tissue lymphoma (30%), diffuse large B-cell lymphoma (28%), and natural killer/T-cell lymphoma (18%). In particular, three patients exhibited different pathological types of ocular lymphoma compared to previously diagnosed systemic lymphoma. The most common site of ocular and systemic involvement was the orbit (85%) and lymph nodes (56%), respectively, and 25 (46%) patients had occult extraocular lesions. Additionally, the 5-year overall survival rate of the entire cohort was 56%, and significant differences were observed between the three main pathological types and stages of the disease (all p < 0.001). CONCLUSIONS: Secondary OAL is a rare disease with a poor prognosis. That the disease is associated with a more aggressive pathological type is well established, indolent lymphoma is not uncommon. Furthermore, OAL and previously diagnosed systemic lymphoma may be pathologically distinct in some patients. Therefore, we recommend a prompt excision biopsy and a thorough systemic evaluation of patients with suspected OAL.

Complete unilateral vision loss and optic nerve cupping from metastatic prostate cancer to the optic canal.

A 71-year-old man, living with metastatic castrate-resistant prostate cancer to the lymph nodes, spine and skull, presented with acute on chronic left eye vision loss. Examination revealed no-light-perception vision, a relative afferent pupillary defect and optic disc cupping. MRI brain revealed optic canal narrowing from metastatic sphenoid bone expansion and extraosseous tumour compressing the intracanalicular optic nerve. The optic disc cupping and excavation without significant pallor of the remaining neuroretinal rim was likely secondary to chronic compression of the optic nerve. The patient was treated with radiation therapy, but did not regain vision and was referred to palliative care as his condition continued to worsen. As patients live longer with advanced cancer, there is a greater risk of metastasis to atypical areas of the body including the optic nerve. This case demonstrates the unique combination of optic disc cupping from optic canal metastasis due to prostate cancer.

Exoftalmos unilateral de corta evolución. Una forma de debut de un neuroblastoma / Unilateral exophthalmos with short evolution. One way to debut a neuroblastoma

El neuroblastoma (NB) es el tumor extracraneal sólido más frecuente en la infancia. Representa el 7% de los cánceres pediátricos. Se origina de la cresta neural, y puede aparecer en cualquiera de los sitios anatómicos a lo largo de la cadena ganglionar simpática, así como en la glándula suprarrenal. El 90% ocurre en menores de 5 años. Presenta un amplio espectro de comportamiento clínico. Los estudios muestran metástasis orbitarias en el 10-20% de los casos. La proptosis y equimosis periorbitaria son consideradas dos de los signos clásicos del neuroblastoma en niños. Presentamos el caso de una paciente de 4 años de edad con un neuroblastoma metastásico que se manifestó como una proptosis de corta evolución al diagnóstico, sin otro tipo de sintomatología ni hallazgos en la exploración física. Tras las pruebas complementarias, se la diagnosticó de neuroblastoma estadio IV de la INSS (The International Neuroblastoma Staying System). No se localizó el tumor primario. Se realizó tratamiento con quimioterapia de inducción, terapia de acondicionamiento y trasplante autólogo de progenitores hematopoyéticos, entrando en remisión completa. Posteriormente inmunoterapia, manteniendo enfermedad residual negativa. Queremos destacar la importancia de un diagnóstico precoz en esta patología de cara a la supervivencia del paciente, y que siempre debe considerarse el diagnóstico de NB ante un exoftalmos en un paciente previamente sano. El tratamiento de los pacientes con neuroblastoma de alto riesgo es multimodal, habiendo mejorado el pronóstico el uso combinado de inmunoterapia, ácido 13 cis-retinoico y trasplante autólogo de médula ósea

Neuroblastoma (NB) is an extracranial solid brain tumor found most frequently during childhood. It represents 7% of the pediatric cancers. It originates in the neural crest and can appear in any part of the anatomy along the ganglionic sympathetic chain, as well as in the adrenal gland. 90% of cases occur en children under the age of 5. It presents a broad spectrum of behavior. Studies have shown orbital metastases in 10-20% of cases. Proptosis and ecchymosis periorbitary are considered two classic signs of neuroblastoma in children. We are presenting a case of a 4 year old female patient with metastatic neuroblastoma that manifested like a proptosis of short evolution to its diagnosis, without any other type of symtomatololgy or findings in the physical exploration. After complementary tests she was diagnosed with stage IV neuroblastoma in the INSS (The International Neuroblastoma Staying System). The primary tumor was not located. Complete remission was obtained using induction chemotherapy, conditioning therapy and autologous hematopoietic progenitors transplant. After immunotherapy negative residual disease was maintained. We would like to highlight the importance of early diagnosis in this pathology facing the patient’s survival and must always consider the NB diagnosis when an exophthalmos presents in a previously healthy patient. Treatment in patients with high risk neuroblastoma is multimodal, having improved the prognosis by using a combination of immunotherapy, 13-cis-retinoic acid and the autologous bone marrow transplant

The management of an orbital diffuse large B-cell lymphoma.

BACKGROUND: The orbit represents a rare site of presentation of non-Hodgkin lymphoma. The diagnosis and management of orbital lymphomas may be challenging because these neoplasms present few specific features. METHODS: A 69-year-old woman presented with painless swelling of the left lower eyelid of 5 years' duration. Magnetic resonance imaging and incisional biopsy were necessary to establish a diagnosis of orbital diffuse large B-cell lymphoma. Staging was completed, thanks to a computed tomographic study of the chest and abdomen. RESULTS: The patient underwent systemic chemotherapy with 1 regimen (doxorubicin, vincristine, prednisone, 6-mercaptopurine, and methotrexate), followed by 1 R-COMP-14 regimen (rituximab, cyclophosphamide, nonpegylated liposome-encapsulated doxorubicin, vincristine, and prednisone). Complete resolution of the disease was obtained. CONCLUSIONS: Although not typically performed by the head and neck surgeon, an understanding of the staging process is crucial for multidisciplinary management of orbital lymphomas.

Orbital lymphoma: diagnostic approach and treatment outcome.

BACKGROUND: Lymphomas of the orbit and orbital adnexae are rare tumors, comprising only 1% of all non-Hodgkin's lymphoma. The majority of non-Hodgkin's lymphomas of the orbit are extranodal marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue type. Because of nonspecific clinical signs and symptoms, some diagnostic delay may occur. The purpose of the study was to evaluate the diagnostic approach in orbital lymphomas and to analyze their treatment outcome. METHODS: In the period from 2005 to 2012, from a group of 135 patients with tumors of the orbit, we identified 11 patients diagnosed with orbital lymphoma. This patient cohort was reviewed retrospectively. RESULTS: The patient group consisted of 11 patients (seven females, male males) with a median age of 57.7 years (range 42 to 88 years). Orbital swelling, pain and motility impairment were the leading clinical symptoms. Diagnosis was confirmed by surgical biopsy. Depending on the anatomic location of the tumor, a surgical biopsy was taken using a blepharoplasty incision, a lateral orbitotomy or a navigation-guided biopsy. The predominant histology was extranodal non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue type (82%). All patients underwent complete clinical staging. These were clinical stage IEA in seven patients, and stages IIEA (n = 2) and IIIEA (n = 2) in four patients . Patients in stage IEA were treated with radiation therapy alone, with radiation doses between 25 and 40 Gy, and patients with stage IIEA received systemic chemotherapy with bendamustin/rituximab. Those two patients diagnosed with diffuse large B-cell lymphoma and mantle cell lymphoma received systemic chemotherapy according to the R-CHOP protocol. CONCLUSIONS: Owing to unspecific clinical symptoms, some diagnostic delay may occur in orbital lymphoma. If unspecific orbital symptoms are present, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis. Once diagnosis is established and staging is complete, radiation therapy is the recommended treatment for stage IEA patients. Systemic chemotherapy is indicated in selected stage IIEA patients and in patients with stage IIIEA disease.

Orbital metastasis associated with primary breast carcinoma in a man detected during peribulbar anesthesia for cataract surgery.

PURPOSE: A case of orbital infiltration by breast carcinoma in a male patient causing mild enophthalmos and subcutaneous mass detected accidentally during peribulbar anesthesia for cataract surgery is discussed. METHODS: The authors report a case of a 65-year-old man who came to the Ophthalmology Department for cataract surgery. During the presurgery peribulbar injection, a hard palpable mass located under the inferior left eyelid was noted, together with mild enophthalmos. A historical clinical screening revealed that 5 years previously the patient had undergone a right radical mastectomy to treat a ductal carcinoma of the breast. RESULTS: The patient underwent an incisional biopsy of the orbital mass that confirmed the clinical hypothesis of a metastasis. DISCUSSION: This case highlights the importance of the collection and screening of detailed clinical information on the patient before every ophthalmic operation including cataract surgery. The occurrence of progressive enophthalmos can represent an unusual symptom of orbital metastasis, commonly presenting with proptosis and diplopia.

Ocular adnexal MALT lymphoma: an intriguing model for antigen-driven lymphomagenesis and microbial-targeted therapy.

Non-Hodgkin's lymphomas constitute one half of malignancies arising in the orbit and the ocular adnexae. Mucosa-associated lymphoid tissue (MALT)-type lymphoma is the most common histological category in this anatomic region. The incidence of ocular adnexal lymphoma of mucosa-associated lymphoid tissue-type (OAML) is increasing and recent studies offered new relevant insights in molecular, pathogenetic and therapeutic issues on these neoplasms. A pathogenetic model of antigen-driven lymphoproliferation similar to that reported for Helicobacter pylori-related gastric MALT lymphomas has been hypothesized for OAML. This notion is supported by the association between OAML and Chlamydophila psittaci infection, an association that is of likely pathogenetic relevance and may influence both the biological behavior and the therapeutic management of these neoplasms. However, this association displays evident geographical variability indicating that other etiopathogenic agents could be involved. These recent acquisitions coupled with the occurrence of chromosomal translocations and other genetic alterations, as well as additional risk factors like autoimmune disorders have contributed to render OAML an exciting challenge for a broad group of physicians and scientists. OAML is an indolent and rarely lethal malignancy that, in selected patients, can be managed with observation alone. Lymphomatous lesions are frequently responsible for symptoms affecting patient's quality of life, requiring, therefore, immediate treatment. Several therapeutic strategies are available, often associated with relevant side-effects. However, the therapeutic choice in OAML is not supported by consolidated evidence due to the lack of prospective trials. In this review, we analyze the most relevant biological, molecular, pathological and clinical features of OAML and propose some therapeutic guidelines for patients affected by this malignancy.

Frecuencia del linfoma No Hodgkin orbitario en el Hospital "Dr. Luis Sánchez Bulnes" de la APEC: revisión de 40 años / Frequency of the NonHodgking Lymphoma of the orbit in the \"Luis Sánchez Bulnes\" Hospital (APEC): aforty-years review

Objetivo. Conocer la frecuencia, distribución topografía y presentación clínica de los linfomas No-Hodgkin (LNH) de la órbita, así como reportar la clasificación histopatológica y pronóstico de estas neoplasias de localización orbitaria utilizando la denominada "fórmula de trabajo" (WF). Método. Se recopilaron 31 casos con diagnóstico histopatológico de LNH del archivo de Anatomía patológica entre 1957 y 1996. Se reevaluaron por microscopia de luz: patrón arquitectónico, tamaño y morfología celular, características nucleares y grado de malignidad de acuerdo a la WF. Clínicamente se evaluó: edad, sexo, lateralidad, tiempo de evolución, presentación clínica y diagnóstico clínico. Resultados. Se incluyeron 20 mujeres (64.5 por ciento) y 11 hombres (35.5 por ciento). Timepo de evolución promedio de 12.2 meses. La proptosis y limitación de movimientos oculares fueron las manifestaciones clínicas más frecuentes. El grado de malignidad de acuerdo a la clasificaicón fue: bajo 29 por ciento, intermedio 55 por ciento y alto 16 por ciento. Conclusiones. Los LNH por su presentación clínica variable deben considerarse en el diagnóstico diferencial en aquellos casos de proptosis en pacientes mayores de 60 años. El diagnóstico definitivo de LNH es histopatológico con el apoyo del cuadro clínico y estudios de gabinetes; su manejo es multidisciplinario

Implant brachytherapy: a novel treatment for recurrent orbital rhabdomyosarcoma.

BACKGROUND: Orbital rhabdomyosarcoma is the most common primary malignancy of the orbit in childhood. Tumor resection and exenteration were the preferred treatment modalities for rhabdomyosarcoma. In the past 20 years, however, combined local radiation and systemic chemotherapy have shown excellent survival results. Tumor recurrence after any of the aforementioned therapies is almost always fatal. We have developed a novel treatment for recurrent disease that has resulted in long-term survival for three patients. METHODS: Three patients with recurrent orbital rhabdomyosarcoma were previously treated with primary radiation and chemotherapy. At the time of recurrence, exenteration and localized brachytherapy were performed. An individually molded poly(methylmethacrylate) (Lucite; E. I. du Pont de Nemours & Co., Wilmington, Del.) device loaded with radioactive iodine seeds delivered localized high-dose radiation, 6000 cGy over 6 days, to the orbit without irradiating the brain. RESULTS: All patients are alive and free of disease with follow-up ranging from 4 years and 4 months to 8 years and 4 months. CONCLUSION: A novel technique of delivering localized radiation to the orbit of three children with recurrent orbital rhabdomyosarcoma appears curative.

Tumores primarios de glándula lagrimal / Primary lacrimal gland tumors

Se presentan los hallazgos clínicos e histopalógicos de 16 pacientes portadores de tumores epiteliales primarios de glándula lagrimal, consultantes entre los años 1960 y 1994 en el Instituto Nacional de Enfermedades Neoplásicas (INEN), con el objeto de conocer la distribución de frecuencias de los distintos tipos histológicos, su tratamiento y evolución. De éstos, 12 presentaron un tumor maligno de la glándula lagrimal principal, uno un tumor maligno de glándulas lagrimales accesorias y tres tumores mixtos benignos de glándula lagrimal principal. En la literatura se indica que los tumores de origen epitelial constituyen entre el 22 por ciento y el 48 por ciento de las lesiones ubicadas en las glándulas lagrimales y que de ellos la mayoría son benignos. Sin embargo, por ser el Instituto Nacional de Enfermedades Neoplásicas un centro de referencia, los porcentajes de lesiones malignas y benignas difieren significativamente de las reportadas en centros generales. Se discute el diagnóstico, tratamiento y evolución de estas patologías y se hace una revisión de la literatura reciente sobre el tema