Análise do perfil clínico de pacientes portadores de neoplasia ocular escamosa atendidos em hospital referência de oculoplástica / Analysis of the clinical profile of patients with squamous ocular neoplasms attended at a reference hospital of oculoplastics

RESUMO Objetivo Analisar o perfil clínico de pacientes portadores de neoplasias escamosas da superfície ocular. Métodos Foram avaliados os principais fatores de risco envolvidos na gênese das neoplasias escamosas da superfície ocular, as características clínicas dos pacientes e os hábitos comportamentais associados. Foram incluídos neste trabalho de coorte histórica 80 pacientes com diagnóstico anatomopatológico de neoplasia escamosa da superfície ocular atendidos entre os anos de 2010 e 2020 em um hospital referência em oculoplástica e segmento anterior de Santa Catarina. Os dados clínicos e desfechos foram avaliados por meio da análise de prontuário e entrevista, sendo posteriormente tabulados no Excel e submetidos à analise estatística por meio do software Statistical Pakage for the Social Sciences, versão 16. Resultados Foi observado que 73,8% (n=59) eram do sexo masculino. A média de idade da amostra foi de 62 anos. Quanto ao fototipo de pele, de acordo com a escala de Fitzpatrick, constatou-se que a maioria apresentou os fototipos 1 e 2 (22; 27,5% e 44; 55%, respectivamente). Em relação à exposição ocupacional ao sol/radiação, 48% (n=60) apresentaram história de exposição ocupacional, sendo que, destes, 28 pacientes trabalhavam no setor de agricultura. Dos pacientes da amostra, 33 (41,2%) apresentavam histórico pessoal de neoplasias de pele, sendo que, destes, três apresentavam diagnóstico de xeroderma pigmentoso. Quanto ao hábito de uso de fatores de proteção solar, 61% (n=49) da amostra negou o hábito. Foi evidenciada associação estatisticamente significativa entre o hábito de usar fatores de proteção solar e histórico pessoal de neoplasias de pele. Em relação ao tipo de neoplasia escamosa, a maioria dos pacientes (72; 90%) apresentou diagnóstico anatomopatológico de carcinoma espinocelular ocular. Conclusão O perfil clínico epidemiológico dos pacientes portadores de neoplasias escamosas da superfície ocular neste estudo, predominantemente de carcinoma espinoceular ocular, foi de homens, idosos, de pele clara (fototipo 2) e com histórico importante de exposição aos raios solares ultravioleta A e B. Comorbidades imunodepressoras (HIV e transplante de órgão sólido) e doenças dermatológicas (albinismo e xeroderma pigmentoso) associaram-se ao aparecimento das neoplasias escamosas da superfície ocular em idade mais precoce. Em pacientes com histórico pessoal prévio de neoplasias de pele, foi evidenciado o hábito de uso de fatores de protetor solar mais presente em relação aos demais.

ABSTRACT Objective To analyze the clinical profile of patients with ocular surface squamous neoplasms (OSSN). Methods The main risk factors involved in the genesis of the ocular surface squamous neoplasms, the clinical features, and the behavioral habits associated were evaluated. This historical cohort study included 80 patients with anatomopathological diagnosis of OSSN who were treated between 2010-2020 at a reference hospital in oculoplastic and anterior segment in Santa Catarina. The clinical data and outcomes were evalated through the analysis of medical records and interviews, being later tabulated in Excel and analyzed using the SPSS 16 software. Results Regarding the clinical profile of the patients in the sample, 73.8% (n = 59) were male. The mean age of the sample was 62 years old. As for the skin phototype, according to the Fitzpatrick scale, most of the sample presented the phototype 1 and 2 (27.5% n = 22; and 55% n = 44 respectively). Regarding occupational exposure to the sun / radiation, 48% (n = 60) had history of occupational exposure, and of these, 28 patients worked in the agricultural area. Of the patients of the sample, 33 (41.2%) had a personal history of skin neoplasms, and of these, 3 had diagnosis of xeroderma pigmentosum. As for the habit of using sun protection factors, 61% (n = 49) of the sample denied the habit. A statistically significant association was evidenced between the habit of using sun protection factors and people's history of skin cancer. Regarding the type of squamous neoplasia, most patients in the 90% sample (n = 72) had an anatomopathological diagnosis of ocular squamous cell carcinoma. Conclusion The clinical epidemiological profile of patients with OSSN in this study, predominantly ocular squamous cell carcinoma, was men, elderly, fair-skinned (phototype 2) and with an important history of exposure to UVA and UVB rays. Immunosuppressive comorbidities (HIV, solid organ transplant) and dermatological diseases (albinism, xeroderma pigmentosum) are associated with the appearance of OSSN at an early age. In patients with a previous personal history of skin neoplasms, the habit of using sunscreen factors was more present than in the other patients.

Oncocitoma em carúncula: relato de caso / Oncocytoma of the caruncle: a case report

RESUMO O oncocitoma é um tipo de neoplasia rara na prática clínica e descrito na literatura, principalmente quando localizado no olho. Quando localizado nos anexos oculares, é mais frequentemente na carúncula. Analisou-se o caso de uma paciente de 74 anos, caucasiana, que relatou desconforto visual no olho esquerdo, e cujo exame físico mostrou lesão tumoral na carúncula esquerda, com volume moderado, presença de neovascularização e secreção excessiva. A excisão cirúrgica da lesão foi realizada sob sedação, e a peça foi enviada para avaliação anatomopatológica. A lesão foi diagnosticada histologicamente como oncocitoma, sem malignidade, e a paciente não apresentou recidiva após o procedimento. Embora raro, esse tumor deve ser reconhecido pelos oftalmologistas, devido ao risco já relatado de desenvolvimento de adenocarcinoma.

ABSTRACT Oncocytoma is a neoplasm rarely observed in clinical practice and reported in the literature, especially when located in the eye. When described in the ocular adnexa, it is most often located in the caruncle. The case of a 74-year-old Caucasian female patient is reported. She complained of visual discomfort in the left eye, and physical examination showed a tumoral lesion in the left caruncle, of moderate volume, presence of neovascularization, and excessive secretion. Surgical excision of the lesion was performed under sedation, and the specimen was sent for pathological examination. The lesion was histologically diagnosed as oncocytoma with no malignancy, and the patient presented no recurrence after the procedure. Although rare, this tumor must be recognized by ophthalmologists due to the risk of developing adenocarcinoma, as already reported.

Lentigo maligno na face: um desafio na conduta / Lentigo maligna on the face: a challenging conduct

Resumo O lentigo maligno é um melanoma in situ, de crescimento radial e lento, que acomete áreas fotoexpostas principalmente em idosos. Quando acomete a pálpebra, devido à proximidade a um órgão nobre, a conduta é controversa, porém a cirurgia é o método mais usado, com margens que variam de acordo com a referência utilizada. Terapias conservadoras são descritas, como o imiquimode 5% e a radioterapia. O presente relato tem como objetivo demonstrar a escassez de estudos sobre a margem cirúrgica e citar opções de tratamentos não cirúrgicos para o lentigo maligno da face.

Abstract Lentigo maligna is a melanoma in situ, of slow radial growth, which affects sun-exposed areas, especially in the elderly. When it affects the eyelid, due to the proximity to a noble organ, the conduct is controversial, but surgery is the method most commonly used, with with margins varying according to the reference used. Conservative treatments are described, such as imiquimod 5% and radiotherapy. This report aims to demonstrate the lack of studies on the surgical margin, and to name nonsurgical treatment options for lentigo maligna of the face.

Oncocytoma of the lacrimal gland: a case report / Oncocitoma de glândula lacrimal: relato de caso

ABSTRACT Here we describe a rare case of a benign tumor in the lacrimal gland of a healthy 4-year-old girl. Mild proptosis was the only abnormality observed on clinical examination. Magnetic resonance imaging of the right orbit revealed an oval, solid, well-circumscribed, homogeneous mass extending from the lacrimal gland and measuring 2.5 × 2.3 × 1.7 cm without any evidence of invasion into adjacent bones. The lesion was surgically excised and histological analyses defined the diagnosis of oncocytoma of the lacrimal gland. Although rare, oncocytoma should be included in the differential diagnosis of lacrimal gland tumors.

Resumo Nós descrevemos um raro caso de tumor benigno na glândula lacrimal em uma criança sadia de 4 anos de idade. Clinicamente, a paciente apresentava apenas uma discreta proptose. A ressonância nuclear magnética (RNM) de órbita direita revelou a presença de uma massa oval, sólida, bem-circunscrita, homogênea, se extendendo a partir da glândula lacrimal, medindo 2,5 cm x 2,3 cm x 1,7 cm, sem nenhum sinal evidente de invasão a estrutura óssea adjacente. A lesão foi cirurgicamente removida e analizada histopatologicamente, sendo estabelecido o diagnóstico de oncocitoma de glândula lacrimal. Apesar de raro, o oncocitoma deve ser incluído no diagnóstico diferencial de qualquer tumor originado da glândula lacrimal.

Adenoid cystic carcinoma of the lacrimal sac: case report / Carcinoma adenóide cístico do saco lacrimal: relato de caso

ABSTRACT Lacrimal sac tumors are rare with a clinical presentation that typically includes obstruction of the lacrimal drainage system and epiphora as the most frequent symptom. Cribriform adenoid cystic carcinoma (ACC) is the most common malignant epithelial tumor of the lacrimal gland and minor salivary glands; however, its occurrence in the lacrimal drainage apparatus is extremely rare. Given the rarity of ACC, definitive diagnosis is almost invariably late conferring a poor prognosis. Herein we report the case of a 41-year-old woman with primary ACC of the lacrimal sac and describe the ophthalmological examination, diagnosis, and multidisciplinary treatment of this rare type of tumor.

RESUMO Tumores do saco lacrimal são raros. A apresentação clínica muitas vezes mostra uma obstrução no sistema de drenagem lacrimal sendo a epífora o sinal mais frequente. Carcinoma adenóide cístico cribriforme (ACC) é o tumor epitelial maligno mais comum da glândula lacrimal e glândulas salivares menores, mas a sua ocorrência no aparelho de drenagem lacrimal é extremamente rara. Infelizmente, devido a raridade destes tumores, o diagnóstico preciso é quase sempre atrasado, o que por sua vez leva a um pior prognóstico. Nós relatamos o caso de uma mulher de 41 anos de idade, com ACC primário do saco lacrimal e analisamos o exame oftalmológico, diagnóstico e tratamento multidisciplinar deste tipo de tumor.

Ocular masquerade syndrome associated with extranodal nasal natural killer/T-cell lymphoma: case report / Síndrome ocular mascarada associada ao linfoma extranodal nasal natural killer/células T: relato de caso

A 33-year-old woman complained of unilateral eyelid edema and blurred vision. Initial ophthalmic examination disclosed anterior chamber reaction with keratic precipitates on the cornea, without posterior abnormalities. Anterior uveitis was treated. Despite that, patient showed rapidly progressive unilateral vision loss with optic nerve swelling. Systemic workup was inconclusive, as well as cranial magnetic resonance imaging and cerebrospinal fluid examination. Based on the hypothesis of optic neuritis, intravenous methylprednisolone pulse was performed with no success. During the following days, the patient presented pericardial effusion and cardiac tamponade, progressing to death. Necropsy was performed and diagnosis of extranodal natural killers/T-cell lymphoma, nasal type with ocular involvement was confirmed by immunohistochemistry.

Paciente feminina de 33 anos apresentando edema palpebral unilateral com baixa acuidade visual. Ao exame oftalmológico inicial apresentava reação de câmara anterior com precipitados ceráticos corneano, sem alterações no fundo de olho. Foi instituído tratamento para uveíte anterior e solicitado exames sistêmicos para investigação. Após alguns dias, paciente apresentou piora da acuidade visual, com edema de disco óptico unilateral. Sendo então solicitada ressonância nuclear magnética de crânio. Ambos exames de imagem e investigação sistêmica foram inconclusivos. Em vista da piora progressiva da acuidade visual e sob hipótese diagnóstica de neurite óptica, foi iniciado pulso intravenoso de metilprednisolona por 3 dias sem sucesso. A paciente apresentou dos dias seguintes, derrame pericárdico e tamponamento cardíaco, evoluindo para óbito. A necrópsia confirmou o diagnóstico de linfoma extranodal de células T e natural killers do tipo nasal com envolvimento ocular, através de imunoistoquímica.

Embryonal rhabdomyosarcoma of the caruncle in a 4 year-old boy: case report / Rabdomiossarcoma embrionário da carúncula em um menino de 4 anos: relato de caso

Rhabdomyosarcoma is a rare tumor, with an annual incidence of 4.3 cases per million children. Even thought, it is the most common soft tissue sarcoma in childhood, with a mean age of 6 to 8 years at diagnosis. A 4 year-old boy presented with a history of a fast growing (1-month) nodular lesion in the caruncle of his left eye. Slit lamp examination showed a vascularized solid nodular lesion in the semilunar fold. The lesion was surgically removed obtaining infiltrated edges with tumoral cells. A second surgery was performed with free tumour edges. The diagnosis of embryonal rhabdomyosarcoma, botryoid type, of intermediate differentiation was made. The treatment for botryoid rhabdomyosarcoma is basically surgical with the combined use of adjuvant polychemotherapy. In adolescent or adult patients (not in infants where growth bone disturbances can occur) external beam radiotherapy can be combined with chemotherapy. Rhabdomyosarcomas of the conjunctiva without orbital extension are rarely reported. We presented a case of a child with a rare tumor which we had a high suspicion of malignancy and early diagnosis and treatment and child is free of systemic disease 6 and half years later. Our research group believes that the key in these tumors is the high index of suspicion and early treatment.

Rabdomiossarcoma é um tumor raro, com uma incidência anual de 4,3 casos por milhão de crianças. É o sarcoma de partes moles mais comum na infância, com uma idade média de 6 a 8 anos no momento do diagnóstico. Um menino de 4 anos apresentou-se com uma história de um crescimento rápido (1 mês) lesão nodular em carúncula de seu olho esquerdo. O exame biomicroscópico mostrou uma lesão vascularizada sólida nodular na prega semilunar. A lesão foi removida cirurgicamente com obtenção de bordas infiltradas com células tumorais, segunda cirurgia foi feita com bordas tumorais livres. O diagnóstico de rabdomiossarcoma embrionário, tipo botrióide, de diferenciação intermediária foi feita. O tratamento para a rabdomiossarcoma botrióide é basicamente cirúrgico com o uso combinado de poliquimioterapia adjuvante. Em pacientes adolescentes e adultos (e não em lactentes onde os distúrbios do crescimento ósseo pode ocorrer) radioterapia externa pode ser combinada com a quimioterapia. Rabdomiossarcomas da conjuntiva sem extensão orbital são raramente relatadas. Apresentamos um caso de uma criança com um tumor raro que tivemos uma alta suspeita de malignidade e diagnóstico precoce e tratamento e criança é livre de doença sistêmica 6 anos e meio depois. Nosso grupo de pesquisa acredita que a chave para esses tumores é o alto índice de suspeita e tratamento precoce.

Primary ductal adenocarcinoma of the lacrimal gland: case report / Adenocarcinoma ductal primário da glândula lacrimal: relato de caso

A 78-year-old male patient presented with double vision, painless palpable mass under the right superolateral orbital rim, downward displacement and restricted adduction of the right eye. His visual acuity was 20/50 OD and 20/20 OS. Hertel exophthalmometry was 21 mm OD and 17 mm OS. Computed tomographic scans showed an infiltrative orbital mass with ill-defined, irregular margins, involving the lacrimal gland and the lateral rectus muscle. The patient underwent an anterior transcutaneous transseptal orbitotomy with incisional biopsy and surgical debulking. Histopathologic evaluation revealed primary ductal adenocarcinoma of the lacrimal gland. Following the metastatic work up, he underwent an eyelid-sparing orbital exenteration. Microscopically, the tumor elements were characterized by large polygonal cells with vesicular nuclei, prominent nucleoli and amphophilic cytoplasm. The tumor components comprised duct-type structures with papillary and cribriform patterns, surrounded by prominent basement membrane. The tumor cells were positive for cytokeratin-7, matrix metalloproteinase (MMP)-2, MMP-9, MMP-13 and proto-oncogene Her-2/neu, but negative for cytokeratin-5, cytokeratin-20, p63, prostate-specific antigen, S-100 protein and thyroid transcription factor. These histopathologic findings were compatible with poorly differentiated ductal adenocarcinoma of the lacrimal gland, T3N0M0. Twenty-four months after orbital exenteration, the patient was diagnosed with ipsilateral parotid gland and cervical lymph node metastases and died of disease.

Paciente do sexo masculino e com 78 anos de idade apresentou diplopia, massa palpável abaixo da margem orbitária direita, deslocamento inferior do bulbo ocular direito e limitação da adução do olho direito. A acuidade visual foi 20/50 OD e 20/20 OE. A exoftalmometria de Hertel foi 21 mm OD e 17 mm OE. Tomografia computadorizada mostrou uma massa orbitária, infiltrativa e com margens irregulares, envolvendo a glândula lacrimal e o músculo reto lateral. O paciente foi submetido a uma orbitotomia anterior com biópsia incisional. O exame histopatológico revelou adenocarcinoma ductal primário da glândula lacrimal. Em seguida, o paciente foi submetido a uma exenteração orbitária com preservação das pálpebras. Microscopicamente, os elementos tumorais foram caracterizados por células poligonais grandes com citoplasma anfofílico, núcleo vesicular e nucléolo proeminente. Os componentes tumorais incluíram estruturas ductais com padrões cribriforme e papilífero e cercadas por membrana basal proeminente. As células tumorais foram positivas para citoqueratina 7, metaloproteinase 2 da matriz, metaloproteinase 9 da matriz, metaloproteinase 13 da matriz e Her-2/neu, mas negativas para citoqueratina 5, citoqueratina 20, p63, antígeno prostático específico, proteína S-100 e fator de transcrição da tiroide. Estes achados histopatológicos foram compatíveis com o diagnóstico de adenocarcinoma ductal pouco diferenciado da glândula lacrimal, T3N0M0. Vinte e quatro meses após a exenteração orbitária, o paciente foi diagnosticado com metástases nos linfonodos cervicais ipsilaterais e na glândula parótida ipsilateral e faleceu.

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.

Meningioma de la vaina del nervio óptico: colaterales optociliares y quiste aracnoideo perióptico / Meningioma of the optic nerve sheath: collateral optociliary veins and the perioptic arachnoidal cyst

En la década sesenta, antes del advenimiento de la tomografía computarizada de las órbitas, los meningiomas de la vaina del nervio óptico eran considerados de rara ocurrencia y su diagnóstico positivo solo era posible en aquellos casos en que por su tamaño producían proptosis ocular, la que conducía a su estirpación quirúrgica. El tumor de crecimiento tubular, comprime el nervio en forma progresiva hasta atrofiarlo por completo. Sus manifestaciones clínicas incluyen la pérdida gradual de la agudeza visual hasta alcanzar la ceguera, edema del nervio óptico y luego atrofia del mismo, y presencia de venas colaterales optociliares. Este complejo signológico se designa como triada de Hoyt-Spencer. Un hecho patológico distintivo de estos tumores objetivado mediante resonancia magnética de órbitas con administración de gadolinio y supresión de grasa, es la presencia de una dilatación quística del espacio subaracnoideo perióptico entre el limite distal del tumor y el polo posterior del ojo. Presentamos el caso de un paciente ilustrativo con demostración histopatológica de las colaterales venosas y del quiste aracnoideo

During the 1970s, before the advent of computerized tomography of orbits, optic nerve sheath meningiomas were considered of rare ocurrence and their positive diagnosis was only posible in those cases in which its size produced ocular proptosis, which led to its surgical removal. Tumor growths in a tubular, compreses progressively the optic nerve and in time produces its atrophic thinning. Clinical manifestation include gradual visual acuity loss until total blindness, optic nerve edema and then optic atrophy, and the presence of collateral optociliary veins. This complex clinical picture is designated as the triad of Hoyt-Spencer. A distinctive fact in these tumors is the presence of a cystic dilatation of the perioptic subarachnoid space between the distal adge of the tumor and the posterior pole, which can be defined using magnetic resonance imaging of the orbits with fat supression following intravenous gadolinium administration. We present the case of a patient with histopatological demostration of the collateral veins and the distal aracnoidal cyst

Adenocarcinoma metástasico a globo ocular: de primario desconocido: reporte de caso revisión de la literatura / Metastatic adenocarcinomas to ocular globe: of unknown primary: report of a case and literature review

El tejido ocular puede verse afectado por tumores metastásicos. La localización más frecuente de estas metástasis es en la coroides. Las lesiones primarias halladas con mayor frecuencia se localizan en la glándula mamaria en las mujeres (47 por ciento) y en el pulmón en los hombres (25 por ciento). Ocasionalmente, las metástasis oculares pueden constituir la primera manifestación de un tumor primario asintomático. Presentamos el caso de una mujer de 40 años que consulta a oftalmólogo refiriendo dolor y disminución progresiva de la visión del ojo derecho como consecuencia de una metástasis ocular derecha de un adenocarcinoma bien diferenciado de origen desconocido. Los síntomas oculares fueron la primera manifectación de la enfermedad.

The ocular tissue can be involved and affected by metastatic tumors. The choroid is the most common localization site for this metastasis. The most common primary lesions found and involved were in the breast in the female (47 %) and in the lung in male (25 %). Occasionally, ocular metastasis may be the first sign of an asymptomatic primary tumor. We report a case of a 40 year old woman which consults to ophthalmology doctor, she refer a progressive decreased visual acuity in her right eye due as consequence of a right choroid metastasis of an adenocarcinoma of unknown origin. The ocular symptoms were the initial manifestation of the 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.

A Case of Recurrent Schneiderian Papilloma of the Lacrimal Sac Invading the Nasal Cavity

A 44-year-old man presented with a history of chronic epiphora, discharge from the right eye, and a palpable mass in the medial canthal area. Irrigation of the lacrimal system revealed bloody discharge. Orbital magnetic resonance imaging (MRI) showed a well-defined heterogeneous enhanced mass filling the lacrimal sac and upper nasolacrimal duct (NLD). A wide excision and surgical biopsy were performed. Histopathology showed the tumor to be an exophytic Schneiderian papilloma with moderate to severe dysplasia. Three months later, the mass was found to be invading the nasal cavity through the NLD. Endoscopic histopathological evaluation confirmed that it was identical to the originally identified papilloma.

Primary Sebaceous Carcinoma of the Corneaoscleral Limbus With Pagetoid Recurrence

We report a sebaceous carcinoma confined to the corneoscleral limbus without involvement of the eyelid. A 60-year-old man, who showed multiple masses on the corneaoscleral limbus and limbal ulceration but with normal eyelids, underwent surgical en-bloc excision of the masses. Histopathologic examination revealed a sebaceous carcinoma. Three weeks after excision, multiple pagetoid recurrences were found along the bulbar conjunctiva 2 mm away from the limbus. After the application of topical mitomycin C, the pagetoid spread regressed completely. After a 2 year follow-up, no other local or systemic recurrences were observed. This report shows that the ulcerative mass which is confined to only the corneoscleral limbus may be a sebaceous carcinoma even without eyelid involvement. Topical mitomycin C may be effective for treating pagetoid spread of sebaceous carcinoma of limbal origin.

The Clinical Usefulness of 18F-FDG PET/CT for the Evaluation of Lymph Node Metastasis in Periorbital Malignancies

OBJECTIVE: The aim of this study was to assess the clinical role of 18F-FDG PET/CT for the evaluation of lymph node metastasis in periorbital malignancies, compared with CT alone. MATERIALS AND METHODS: We analyzed eighteen PET/CT and CT scans in 15 patients with biopsy-proven periorbital malignancies. We compared the diagnostic capabilities of PET/CT and CT with regard to nodal metastasis by level-by-level analysis and by N staging prediction. The reference standards were surgical pathology (n = 7) from dissected lymph node specimens and the results from radiological follow-up (n = 11, mean 20.5 months; range 10-52 months). Moreover, any changes in patient care as prompted by PET/CT were recorded and compared with treatment planning for CT alone. RESULTS: PET/CT had a sensitivity of 100%, while CT had a sensitivity of 57% (p = 0.03) for nodal metastasis by level-by-level analysis. PET/CT had a specificity of 97%, positive predictive value of 93%, negative predictive value of 100%, and diagnostic accuracy of 98%, while the CT values for these same parameters were 97%, 89%, 82%, and 84%, respectively. PET/CT correctly predicted N staging with an accuracy of 100%, while CT was only 83% accurate (p = 0.01). Regarding the impact on patient care, the extent of surgery for regional lymph nodes and the treatment decision were modified by PET/CT in 39% of patients. CONCLUSION: PET/CT could provide useful information in the management of regional lymph node metastases in patients with periorbital malignancies.

Granular Cell Tumor of The Inferior Rectus Muscle

A 53-year-old woman complaining of vertical diplopia, presented with a localized swelling in the right lower lid. Magnetic resonance imaging studies demonstrated a relatively well-defined mass in the inferior rectus with similar signal characteristics to the muscle. Excisional biopsy of the mass revealed granular cell tumor composed of S-100 positive cells with acidophilic granular cytoplasm and a peripheral lymphocytic infiltration. Granular cell tumor, which is very rare in the orbit, should be considered in the differential diagnosis of tumors adjacent to or within the extraocular muscles, particularly in the inferior orbit.

A clinicopathological study of malignant melanoma with special reference to atypical presentation.

Malignant melanoma is a tumor of melanocytic origin. Lymphatic and hematogenous metastases are common in this condition. Retrospective analysis was performed in 16 consecutive cases diagnosed histopathologically as malignant melanoma at the pathology department of a medial college in eastern India. 75% of the patients were male; majority of them was in their sixth decade. All (100%) the lesions were pigmented. The primary site was known in all cases, except two (12.5%). Out of the 14 cases with known primary site 11 (78.57%) were cutaneous melanomas, including one arising in labia minora, two (14.29%) were ocular and one (7.14%) was vaginal in origin. Among cutaneous melanomas, superficial spreading type was the commonest variety and mixed population of epithelioid and spindle cell was the commonest histopathological pattern. The commonest grade of invasion was grade III (Clark's). The clinical presentation of the case of vaginal melanoma and the two cases of secondary melanomas, including the one with obscure primary tumor, were bewildering and hence are discussed separately.

Goldenhar syndrome with unusual features.

We report here the case of a 17 year-old girl with the classic signs of Goldenhar syndrome in the form of multiple accessory tragi, bilateral ocular dermoids, mandibular hypoplasia (micrognathia) and cervical lordosis. She also had a high arched palate, gingival hypertrophy and malaligned teeth, features which are as yet unreported.

Neurofibromatose crânio-orbital: diagnóstico e abordagem cirúrgica da doença de von Recklinghausen / Cranio-orbital neurofibromatosis: diagnosis and surgical management of the Von Recklinghausen's disease

A neurofibromatose (NF1) é uma soença hereditária do grupo das neuromesoectodermatose, de acometimento sistêmico variável e progressivo, com transmissão autossômica dominante de expressão variável. Objetivo: Relatar a experiência do grupo de Cirurgia Craniomaxilofacial da Divisão de Cirurgia Plástica e Queimaduras do Hospital das Clínicas da Faculdade de Medicina da Universidade de São paulo na abordagem e tratamento cirúrgico da neurofibromatose. Método: Este é um trabalho retrospectivo de 20 pacientes tratados no período de 1985 a 2003. Dados clínicos e cirúrgicos foram revisados. Resultados: Nove pacientes eram do sexo feminino, com idade entre 4 e 25 anos. História familiar positiva foi encontrada em cinco casos. Os sinais clínicos comuns à doença foram encontrados em todos os pacientes, incluindo manchas café-com-leite e neuromas. A localização cranio-orbitária foi caracterizada por acometimento orbitário em 15 casos. Na tomografia, ausência parcial da asa maior do esfenóide foi encontrada, com alargamento da fissura orbitária. O tratamento cirúrgico incluiu a ressecção tumoral parcelada e reconstrução orbitária, cirurgia palpebral e do globo ocular, além de procedimentos secundários, como ritidoplastia. Conclusão: A NF1 é uma doença agressiva, que requer tratamento individualizado. O paciente deve estar consciente que são muitas as etapas operatórias, sendo as ressecções parciais o tratamento de escolha, enquanto as excisões radicais têm indicações específicas nos casos mais graves.