Bilateral metastases to the extraocular muscles from small cell lung carcinoma / Metástases bilaterais aos músculos extraoculares de um carcinoma de pequenas células do pulmão

ABSTRACT Bilateral orbital metastases restricted to the extraocular muscles (EOMs) are exceedingly rare. We report a case of bilateral extraocular muscle metastases from a small cell lung carcinoma and provide a review of the relevant literature. A 56-year-old smoker presented with proptosis, motility changes, and a relative afferent pupillary defect of the left eye, with a previous history of a small cell lung carcinoma. An orbital computerized tomography scan revealed a mass restricted to the left medial rectus. An incisional biopsy confirmed metastasis. Visual acuity of the left eye decreased rapidly, and right globe proptosis became evident. Orbital magnetic resonance imaging at two months follow-up showed marked left orbital mass enlargement and a new right lateral rectus mass. The patient was maintained on palliative care and died from metastatic disease-related complications.

RESUMO As metástases orbitárias bilaterais restritas aos mús­culos extraoculares são extremamente raras. Os autores apresentam um caso de metástases bilaterais, localizadas aos musculares extraoculares com base num carcinoma de pequenas células do pulmão e revisão da literatura relevante. Um homem, fumador, de 56 anos recorreu ao serviço de urgência por proptose, alterações de motilidade ocular extrínseca e um defeito pupilar aferente relativo do olho esquerdo, com história pessoal de carcinoma de pequenas células do pulmão. A tomografia computadorizada orbitária revelou uma massa restrita ao reto medial esquerdo. Uma biópsia incisional confirmou o diagnóstico de metástase. A acuidade visual do olho esquerdo diminuiu rapidamente e surgiu uma proptose do globo ocular direito. A ressonância magnética orbitária aos dois meses de seguimento revelou um aumento da massa orbitária esquerda e uma nova massa no reto lateral direito. O paciente foi mantido em cuidados paliativos e faleceu devido a complicações relacionadas com doença metastática.

Role of new magnetic resonance imaging modalities in diagnosis of orbital masses: a clinicopathologic correlation

To evaluate the role of diffusion-weighted magnetic resonance imaging [MRI] and proton magnetic resonance spectroscopy [MRS] in the diagnosis of different orbital masses and their advantages over conventional MRI. The study included 20 patients presenting with proptosis. Every patient was subjected to thorough clinical examination, conventional MRI [Tl weighted, T2 weighted, and postcontrast Tl weighted if needed,] diffusion-weighted MRI, and proton MRS. Orbitotomy was performed, the orbital mass was excised, and histopathological examination was performed. Diffusion-weighted MRI could differentiate between benign lesions and malignant tumors in 70% of cases; however, overlap occurred in 30% of cases with benign tumors showing restricted diffusion whereas proton MRS could differentiate between benign and malignant tumors in 90% of cases. Diffusion-weighted MRI and proton MRS can potentially increase the accuracy of diagnosis of orbital masses through in vivo tissue characterization. Magnetic resonance spectroscopy seems to be the more accurate modality

Retinoblastoma unilateral estadio III en pre-escolar: a propósito de un caso Hospital Central Dr. José María Vargas San Cristobal marzo 2008 Edo. Táchira-Venezuela / Unilateral retinoblastoma stage III in pre-school: a purpose of case Hospital Central Dr. José María Vargas San Cristobal in march 2008 state Táchira-Venezuela

El retinoblastoma comprende el 4 por ciento de tumoraciones malignas en edad pediátrica con una incidencia de 1 por cada 20000 niños vivos y en USA se presenta por año entre 250-300 niños, 50-95 por ciento sobreviven a nivel mundial, no hay predisposición por sexo o raza y entre ellos 25-35 por ciento se presentan en forma bilateral, la edad aproximada para el diagnóstico varia en las formas unilaterales alrededor de los 12 meses hasta las formas bilaterales a los 24 meses. Preescolar femenino de 4 años de edad natural de Socopó, procedente de El Piñal quien inicia enfermedad actual de 2 años de evolución caracterizada por la presencia de masa tumoral en ojo izquierdo la cual se incrementa en los últimos 4 meses llegando hasta la protrusión del globo ocular y de 6 días limitación para la oclusión, rubor en párpado superior izquierdo y fiebre. Asimetría ocular, protrusión del globo ocular izquierdo, limitación para la apertura, hifema y no se visualiza córnea, pupilas ni el iris. Es evaluada por el servicio de Oftalmología: OI: Blefarospasmo reactivo, eritema bipalpebral y edema que limitan la apertura ocular. Biomicrooscopia: Quemosis conjuntival en 360 con salida de secreción purulenta a nivel temporal de la conjuntiva, pannus corneal periférico en banda cálcica, opacidad de medios transparentes. LDH 267 UI/I. Ultrasonido Ocular: globo ocular izquierdo: faquico, con lesión sólida localizada en pared ocular extendiéndose anteriormente, sin movilidad, con ecos de alta reflectividad en su interior con sombrado posterior de pared ocular. TAC de cráneo y órbita: imagen radiólucida que infiltra globo ocular izquierdo sin compromiso del nervio óptico, resto sin anormalidad, TAC de tórax y abdomen: normal, Ecosonograma Abdominal: normal, Gammagrama Óseo: normal. Se realizó exanteración de la órbita del globo ocular izquierdo. Biopsia que microscópicamente evidencia tumor infiltra todas las capas con microscopia donde evidencia pseudoresetas en masa tumoral.

Proptosis de ojo izquierdo / Left eye proptosis

Se presenta el caso clínico de una paciente joven que acude al Servicio de Oftalmología del Hospital Nacional con leve disminución de la agudeza visual y proptosis del ojo izquierdo, sin antecedentes de traumatismo, cefalea, ni diplopía...(GG)

Acometimento intra-ocular na doença de Erdheim-Chester - primeiro relato na literatura: relato de caso / Intraocular involvement in Erdheim-Chester disease - first report in the literature: case report

A doença de Erdheim-Chester (DEC) tem causa desconhecida e se caracteriza por ser granulomatosa e infiltrativa, com proliferação de histiócitos contendo colesterol e particular acometimento ósseo. À histologia, é semelhante à histiocitose de células Langerhans, salvo na análise imuno-histoquímica. Pela primeira vez é descrito o acometimento intra-ocular nesta doença. MPSG, 46 anos, sexo feminino, apresentou-se com proptose do olho direto. Referia úlceras no palato duro, osteoesclerose bilateral e simétrica na diáfise tibial e fibular e nódulo na mama direita (biópsia: infiltrado rico em histiócitos xantomatosos e perfil imuno-histoquímico com CD68 +, S-100 e CD1a negativos, compatível com DEC). À RNM, tumoração extraconal justa-bulbar temporal superior na órbita direita, hiperintensa em T1 e próxima da glândula lacrimal. Em cada olho, visão 20/20, com numerosas drusas nas arcadas e na região macular similares às drusas laminares basais, além de duas regiões placóides elevadas com infiltrado alaranjado sub-retiniano e hiperfluorescentes na angiografia na região peridiscal inferior e justamacular temporal do olho direito. Dois anos depois, surgiram membranas neovasculares sub-retinianas em ambos os olhos. O OD manteve visão 20/20 com pequenas alterações campimétricas e o OE evoluiu com grande cicatriz disciforme e visão de vultos. Esta descrição pioneira demonstra características in vivo dos granulomas histiocíticos da DEC, e alerta para possíveis complicações intra-oculares.

Erdheim-Chester disease (ECD) is a granulomatous and infiltrative disorder of unknown etiology with proliferation of cholesterol-containing histiocytes and peculiar bone involvement. It is very similar to Langerhans cell histiocytosis (LCH) on histology but with a different immunohistochemical profile. This is the first report of intraocular involvement in this disease. MPSG, a 46 y.o. woman, presented with proptosis of the OD. She referred ulcerated lesions on the hard palate, symmetrical and bilateral osteosclerosis of the fibulae and tibiae and a nodule in the right breast (biopsy: xantomatous histiocytic infiltrate CD68+, S-100 and CD1a negative on immunohistochemistry compatible with ECD). MRI studies demonstrated an extraconal tumor in the juxta-bulbar temporal portion of the right orbit close to the lacrimal gland and hyperintense on T1. Vision was 20/20 OU, with numerous drusen in the posterior pole, similar to basal laminar drusen. Two regions of orange subretinal infiltrates that showed progressive staining on the angiogram were seen in the peripapillary region and also close to the fovea in the OD. Choroidal neovascular membranes were seen 2 years later in OU leading to severe visual loss in the OS and to a slight visual field loss in the OD, which retained 20/20 vision. This pioneer report depicts in vivo characteristics of histiocytic granulomas in ECD. Caution should be taken with patients with ECD as potentially blinding intraocular complications may arise.

Oftalmopatía distiroidea / Dysthiroid Ophtalmopathy

La oftalmopatía distiroidea es la causa más frecuente de proptosis uni o bilateral en el adulto. Afecta con más frecuencia a las mujeres entre los 25 y los 50 años, aunque el curso es más agresivo en los hombres. Se relaciona con hipertiroidismo (91%), tiroiditis de Hashimoto (3%) y eutiroidismo (6%). Hisotpatológicamente se observa un aumento de glucosaminoglicanos en el tejido conectivo de grasa orbitaria y músculos extraoculares. Las manifestaciones clínicas son, en orden de frecuencia, la retracción palpebral, la proptosis, la diplopia y la neuropatía óptica por compresión.

Weber-Christian disease presenting with proptosis: a case report

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.

Weber-Christian disease presenting with proptosis: a case report

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.

Solitary intracranial plasmacytoma of the skull base.

Two cases of extradural solitary plasmacytoma (SIP) with infiltration of the dura, destruction of the skull base, multiple cranial nerve palsies and proptosis are presented. The cases were treated aggressively with surgery and radiotherapy and showed no signs of progressing to multiple myeloma after 15 months and four and a half years respectively.