Metástasis intraorbitárias de cáncer de mama / Breast cancer orbital metastasis

Objetivo: Describir un caso raro de una paciente previamente diagnosticada con cáncer de mama que evolucionó después de algunos años con metástasis intraorbitaria. Métodos: Relato del caso y revisión de literatura. Resultados: La correlación del diagnóstico previo de neoplasia de mama y la presencia de múltiples lesiones en la órbita y regiones adjacentes ha permitido concluir que la paciente presentaba enfermedad metastásica. Las metástasis se presentan como manifestaciones cada vez más frecuentes en enfermos oncológicos. La órbita y estruturas próximas constituyen topografías poco usuales de diseminaciónde células neoplásicas y manifestaciones iniciales pueden mimetizar otras condiciones, retrasando el diagnóstico. Como se trataban de pequeñas lesiones, se ha optado por radioterapia como tratamiento único. Después del término la paciente evolucionó con regresión de sintomatología. Conclusiones: Aunque infrecuentes, manifestaciones oculares pueden ocurrir en enfermos oncológicos y la investigación de enfermedad metastático de la órbita es esencial para el seguimiento adecuado.

Objective: Description a rare case of a patient with previous diagnosis of breast cancer that evolved years after with orbital metastasis. Method: Case report and literature review. Results: The relation between the previous diagnosis of breast cancer and the presence of multiple orbital and adjacent lesions allowed concluding that the patient presented metastatic disease. Metastasis presented as a common manifestation in oncological patients. Orbit and neighboring structures constitute unusualsite of neoplastic cells dissemination and initial manifestations could mimic other conditions, which can slow diagnosis. As they were small lesions that are close to noble structures, it was opted by performing exclusive radiotherapy. After the end of treatment, the patient referred improvement of her symptoms. Conclusions: Although unusual, ocular manifestations could occur in oncological patients and orbit metastatic disease investigation is essential to the proper follow.

[Primary orbital squamous cell carcinoma]. / Carcinoma escamoso primario de órbita.

Primary orbital squamous cell carcinoma is a rare entity. There is little published literature. We report a case of primary squamous cell carcinoma of the orbital soft tissues. Surgical resection offered the best treatment for the patient. Complete resection of the lesion was achieved. The patient received adjuvant radiotherapy due to the proximity of the lesion to the surgical margins. Surgical treatment is feasible and should be considered as part of the surgeon's arsenal. However, therapeutic decisions must be made on a case-by-case basis.

Carcinoma escamoso primario de órbita / Primary orbital squamous cell carcinoma

El carcinoma escamoso primario de órbita es una entidad poco frecuente. Hay escasa literatura publicada al respecto. Presentamos un caso de carcinoma escamoso primario de los tejidos blandos de la órbita. La resección quirúrgica ofrecía el mejor tratamiento para esta paciente. Se realizó un abordaje cráneo-facial y se logró la resección completa de la lesión. La paciente recibió radioterapia adyuvante debido a la proximidad de la lesión a los márgenes quirúrgicos. El tratamiento quirúrgico es factible y debe ser considerado como parte del arsenal del cirujano. Sin embargo, las decisiones terapéuticas deben tomarse teniendo en cuenta las particularidades de cada caso.

Primary orbital squamous cell carcinoma is a rare entity. There is little published literature. We report a case of primary squamous cell carcinoma of the orbital soft tissues. Surgical resection offered the best treatment for the patient. Complete resection of the lesion was achieved. The patient received adjuvant radiotherapy due to the proximity of the lesion to the surgical margins. Surgical treatment is feasible and should be considered as part of the surgeon´s arsenal. However, therapeutic decisions must be made on a case-by-case basis.

Reactive post-radiotherapy bone formation in the maxilla.

OBJECTIVE: High-dose radiation therapy affects bone metabolism, and therefore post-radiotherapy bone formation is an uncommon finding. This case describes an unusual ossification in the maxillary region identified after head and neck radiotherapy. RESULTS: A 45-year-old female patient was submitted to maxillary surgical resection and orbital exenteration due to squamous cell carcinoma. Radiotherapy post-surgery was performed because of compromised surgical margins. After 4 months of the conclusion of radiotherapy, the patient presented a bone exposition at surgical site. Surprisingly, the oral examination and computed tomography revealed a new formation of the right palatine vault and bone formation filling the nasal and orbital cavity. The incisional biopsy discarded residual disease or osteoradionecrosis and showed normal tissue. CONCLUSIONS: To the best of the authors' knowledge, this is the first case of wide bone formation as an early effect of head and neck radiotherapy.

Plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma: case report.

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.

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.

Mesenchymal chondrosarcoma of the orbit.

PURPOSE: To describe a patient with mesenchymal chondrosarcoma of the orbit, review clinical and imaging findings, and refine the differential diagnosis from other tumors of the orbit. METHODS: Interventional case report and literature review. RESULTS: A 21-year-old woman with a 6-month history of progressive proptosis presented with left-sided visual loss of recent onset. CT revealed a clearly outlined heterogeneous mass with calcified foci, whereas MRI showed an isointense signal to gray matter on T1- and T2-weighted images. The patient was diagnosed with mesenchymal chondrosarcoma of the orbit only after orbitotomy and subsequent histopathologic study. After surgery, she received adjuvant therapy with irradiation of the orbit. CONCLUSIONS: Mesenchymal chondrosarcoma is a rare malignant tumor of the orbit capable of mimicking several other lesions. Early diagnosis requires a high level of suspicion, particularly with regard to internally calcified lesions. The treatment of choice is complete surgical resection, or, when the tumor is not resectable or residual mass is present after surgery, chemotherapy and radiotherapy. Prognosis for patients with orbital mesenchymal chondrosarcoma is tentative at best, in part because of the rarity of the lesion.

Aggressive management of orbital meningeal melanocytoma.

OBJECTIVE: Meningeal melanocytoma generally occurs in the posterior fossa. Orbital manifestation is rarely encountered. METHODS: A thirty-five year-old man presented with progressive proptosis of his right eye. Computed tomography (CT) and Magnetic Resonance Imaging (MRI) of the brain showed an expansive intraconal mass lesion occupying the superior orbital compartment, the entire orbital apex, and the optic foramen. Histological analysis and Immunohistochemical staining for S-100 and HMB-45 monoclonal antibodies confirmed melanocytoma. FINDINGS: Microsurgical removal was accomplished through a fronto-orbital craniotomy. Chemotherapy and irradiation followed the initial intervention. The patient returned for follow up two years after surgery, complaining of headache and right visual loss. A subfrontal tumor with massive edema was found on follow up CT scan. INTERPRETATION: Meningeal melanocytomas are rare benign pigmented tumors of the central nervous system. They are predominant in the posterior fossa and spinal cord and frequently mistaken for melanomas, especially on frozen sections. Orbital presentation is rare. The natural history is poorly defined.

Cáncer de mama metastásico en ojo y órbita: tratamiento radiante. Nuestra experiencia / Breast carcinoma metastatic to the eye and orbit: our experience

Los tumores malignos más frecuentes en ojo y órbita son las metástasis. El cáncer de mama es el tumor que con más frecuencia metastatiza en ojo y órbita (40 por ciento-50 por ciento de los casos según las distintas series). El tratamiento tiene como uno de sus pilares a la radioterapia. Objetivos: evaluar la eficacia del tratamiento radiante en pacientes (ptes.) con cáncer de mama metastásico en ojo y órbita. Materiales y métodos: entre los años 1996-2002 recibieron tratamiento radiante 17 ptes. con tumores metastásicos en ojo y órbita, de ellos 9 ptes. (53 por ciento) tenían metástasis óculo-orbitarias de cáncer de mama. La edad media fue 54 años (R: 43-84 años). Todas las ptes. son de sexo femenino. La localización de las metástasis fue: ojo derecho, 2 ptes.; ojo izquierdo, 5 ptes.; compromiso orbitario bilateral, 2 ptes. De las 9 ptes., 3 presentaron metástasis en coroides y 6 en órbita...(AU)

Radioterapia estereotáxica fraccionada como tratamiento radiante en la metástasis orbital y ocular (reporte de tres casos) / Fraccionated stereotactic radiation therapy as treatment in retro-ocular and orbital metastasis: report of 3 cases

Tres pacientes, uno con metástasis ocular y dos con metástasis en órbita, fueron referidos para su tratamiento al CMPDF, Córdoba, Argentina. Todos ellos recibieron Radioterapia Estereotáxica Fraccionada (REF). En dos pacientes el tumor primario fue de mama y el restante de pulmón. La técnica de la REF es descripta en éste artículo, la dosis entregada fue de 50 Gy en 2 pacientes y de 58 Gy en 1 paciente. Este estudio analiza las características y los resultados obtenidos en éstos pacientes. Dos pacientes se hallan vivos y libres de enfermedad clínica en la órbita, al cierre del presente estudio; el paciente con metástasis ocular falleció por diseminación sistémica, con enfermedad ocular controlada. No se han observado complicaciones inmediatas o mediatas, referidas al tratamiento. Concluímos que la REF es una terapéutica muy efectiva para el tratamiento de lesiones metastásicas en órbita y sin evidencia de complicaciones severas (AU)

Small choroidal melanoma with massive extraocular extension: invasion through posterior scleral emissary channels.

Massive orbital extension arising from a small posterior pole uveal melanoma is rare. A 75-year-old male followed for an asymptomatic choroidal nevus developed a two and a half month history of mild decrease in visual acuity. Upon clinical examination minimal enlargement of the tumor with surrounding serous retinal detachment was observed. Pre-operative magnetic resonance imaging disclosed massive extrascleral extension. Histopathology revealed that the tumor was composed of spindle B malignant melanoma cells. Multiple serial sections of the entire tumor area failed to demonstrate permeation through scleral lamellae. Instead, peripapillary perivascular short posterior ciliary vessel invasion was observed. This route of dissemination may explain the rapid growth and clinically undocumented behavior of small malignant melanomas at this location.

Retinoblastoma with low risk for extraocular relapse.

OBJECTIVE: To define a subgroup of patients with retino-blastoma and low risk of extraocular relapse through histopathological and clinical variables. PATIENTS AND METHODS: Inclusion criteria consisted of stage I (intraocular disease), stage IIb1 (without concomitant choroid and/or scleral invasion), and nonenucleated patients (according to the Grabowski-Abramson classification). A total of 112 consecutive patients admitted to Hospital JP Garrahan from 1987 to 1997 were evaluable. Treatment included enucleation or local therapy and no chemotherapy. RESULTS: Forty-one patients had stage Ia (intraretinal), 8 stage Ib (prelaminar optic nerve invasion), 40 stage Ic (uveal invasion), and 12 stage IIb1 (postlaminar optic nerve invasion and cut end free of tumor). Eleven patients had neither eye enucleated. Median follow-up was 60 months. Only two events occurred: one patient had progressive disease in the contralateral globe and died of CNS metastasis and another had an orbital relapse that was successfully treated. Both had choroidal invasion. Five-year pEFS and pOS were 0.97 and 0.98, respectively. Neither length of the optic nerve stump, tumor size, anterior chamber invasion, degree of differentiation, nor degree of ocular coat invasion correlated with increased risk of metastasis. CONCLUSIONS: A subset of patients with retinoblastoma with low risk of relapse can be determined using histopathological evaluation of the invasion of ocular coats. Adjuvant chemotherapy is not warranted for patients with intraretinal extension and prelaminar optic nerve invasion. It is also probable that those patients with isolated choroidal invasion and those with postlaminar optic nerve extension with surgical margins clear of tumor do not need chemotherapy.

Carcinoma mucoepideermoide nasal y de antro maxilar y su recuperación orbitaria / Nasal mucoepidermoid carcinoma and maxilar sinus and orbital repercussion

Dentro del grupo de los tumores malignos epiteliales de las fosas nasales y antro maxilar, el carcinoma mucoepidermoide es el más raro. La participación orbitaria es frecuente y se ilustra con dos casos, uno con participación de la cavidad nasal y antro maxilar y el otro exclusivamente del antro maxilar. Las manifestaciones clínicas dependen de las alteraciones mecánicas musculares y la proptosis; las más frecuentes son: exoftalmo no reductible, disminución de la capacidad visual hasta llegar a la ceguera y deformación facial. La tomografía y resonancia nuclear magnética son muy útiles en el diagnóstico y pronóstico. El pronóstico es malo tanto para la función como para la vida. El tratamiento es eminenetemente quirúrgico, la radioterapia y quimioterapia son paliativos y con severas complicaciones deletéreas

Tumores orbitarios en niños. Su abordaje quirúrgico transcraneal / Orbitary tumors in children. The transcranial surgical approach

De marzo de 1980 a diciembre de 1995 se trataron 23 pacientes con tumores orbitarios en el Departamento de Neurocirugía del Instituto Nacional de Pediatría (INP). En este trabajo se analiza edad, características clínicas, alteraciones radiológicas y de potenciales evocados; tipo de cirugía, diagnóstico histopatológico y resultados. En todos ellos se efectuó un abordaje craneoorbitario, tipo destechamiento simple, o bién, por fronto-orbitotomía. De estos pacientes, 11 tenían glioma del nervio óptico, tres displasia ósea fibrosa, dos meningioma, neurofibroma, histocitosis de células de Languerhans; uno astrocitoma anaplásico, teratoma, tumor neuroectodérmico primitivo y germinoma con áreas de coriocarcinoma. Se realizó, craneotomía con destechamiento orbitario (Nafziger) en 19 casos y fronto-orbitotomía en 4. Los resultados fueron clasificados de acuerdo a la escala de karnofsky; potenciales evocados postoperatorios, presencia de defecto estético y proptosis pulsátil. Buenos resultados fueron: Karnofsky mayor de 70, no recurrencia tumoral y no emperoramiento de potenciales Visuales; regulares: sin recurrencia tumoral y no empeoramiento de Potenciales Visuales; regulares: sin recurrencia tumoral, cambio en potenciales evocados, escala de Karnofsky entre 50 y 60; malos: recurrencia, lesión visual agregada en potenciales evocados y Karnofsky menor de 50. El reporte muestra la variedad de lesiones intraorbitarias que pueden ser abordadas por vía transcraneal

Biópsia percutänea orientada pela tomografia computadorizada em um caso de tumor orbitário / Percutaneous biopsy oriented by computed tomography in a case of orbitary tumor

É apresentado um caso de tumor orbitário que foi diagnosticado por biópsia de agulha percutânea orientada pela tomografia computadorizada. O paciente foi poupado de uma orbitotomia e tratado com radioterapia. A técnica e o resultado após o procedimento säo mostrados

Sarcomas de partes blandas de localizaciones parameníngeas en el niño: valoración del tratamiento radiante / Childhood soft tissue sarcoma in parameningeal sites: assessment of radiation therapy

En un grupo de 21 niños con sarcomas de partes blandas de localizaciones parameningeas (nasofaringe, senos perinasales, órbita, fosas nasales y oido medio) tratados con la asociación radioterapia-poliquimioterapia se alcanzó una supervivencia del 27,09

En 12 niños, donde la irradiación estuvo localizada en el área del tumor con una dosis en el rango de 56 a 60 Gy,la supervivencia alcanzada fue de 20.83

, estabilizada a partir de los 38 meses de evoluciòn. En 9 niños la irradiación incluyó, además del tumor primario con dosis entre 55 y 65 Guy, la irradiación de todo el cráneo con dosis entre 24 y 30 Guy, con una supervivencia del 40

, estabilizada en el mes 13 de la evolución. En ambos grupos se empleó la quimioterapia con la combinación vincristina,ciclofosfamida y actinomicin D (VAC) y además la adriamicina (VACA). La diferencia en la supervivencia alcanzada en ambos grupos es estadisticamente significativa a favor de la irradiación extendida al cráneo y demuestra la utilidad de esta en el tratamiento de estos tumores parameníngeos