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Orbita III - Tumores Orbitais: Epidemiologia, Infiltrações Linfó Ides E Tumores Da Glândula Lacrimal

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Orbita III - Tumores Orbitais: Epidemiologia, Infiltrações Linfó Ides E Tumores Da Glândula Lacrimal REVISÃO'IEMÁTICA ,, Orbita III - Tumores Orbitais: Epidemiologia, Infiltrações linfó ides e tumores da glândula lacrimal Fernando Chahud 111 Fabiano Hueb de Menezes 121 Antonio Augusto Velasco e Cruz 131 INTRODUÇÃO tinham se submetido à tomografia computadorizada de órbita, segundo protocolo do setor de radiologia do HCFMRP-USP, Várias afecções neoplásicas, primárias ou não, podem que consiste em cortes axiais e coronais (3x3 mm), com acometer a órbita. O diagnóstico preciso da natureza dos contraste e janela óssea. Esses exames foram revistos. Os tumores orbitários é de fundamental importância pois só as­ dados relativos às idades dos pacientes foram obtidos median­ sim é possível direcionar a abordagem clínica e cirúrgica te consulta dos prontuários. destas patologias, bem como definir o caráter de urgência do As lâminas das biópsias orbitárias, coradas rotineiramente tratamento. Na grande maioria dos casos, a biópsia da lesão é pela hematoxilina e eosina, foram todas revisadas. Para os a pedra angular do diagnóstico, sem a qual a chance de tumores linfóides, além da hematoxilina e eosina, marcadores indicação de uma cirurgia equivocada é grande. linfóides para determinação de linfomas B ou T tinham sido O tema é bastante complexo e pouco discutido na literatura previamente utilizados pelo Setor de Patologia Cirúrgica, na nacional e será dividido em algumas secções. Na nossa opi­ ocasião do diagnóstico. Para os tumores linfóides, além da nião, uma revisão extensa da literatura internacional sobre o revisão das lâminas para a classificação das lesões segundo a assunto corre o risco de não expressar a realidade de nossos Working Formulation, os pacientes foram encaminhados para serviços de oculoplástica, uma vez que a epidemiologia des­ o Setor de Hematologia para estadiamento clínico e tratamen­ ses tumores varia de acordo com o local de atendimento. to das lesões. Assim, inicialmente situaremos a freqüência relativa das Na avaliação geral, os tumores malignos foram mais fre­ neoplasias orbitais com base num estudo de 63 lesões docu­ qüentes que os benignos (Figura 1 ), e os primários mais mentadas com tomografia computadoriza e histopatologia. encontrados que os secundários (Figura 2). Convém ressaltar Em seguida, detalharemos dois grupos de tumores orbitais que os critérios de malignidade para esta classificação foram importantes e que podem aparecer imbricados: as infiltrações puramente histopatológicos. linfóides e os tumores de glândula lacrimal . Quando as freqüências relativas dos tumores com relação à linhagem tecidual dos mesmos foram analisadas, os tumores epiteliais e linfóides foram os mais comuns, seguidos pelos PREVALÊNCIA RELATIVA DOS TUMORES ORBITAIS tumores neurais e vasculares (Figura 3). A figura 4 detalha a distribuição dos tumores de acordo com o diagnóstico Todos os pacientes computados neste estudo foram atendi­ histopatológico. Fica evidente a importância das infiltrações dos no setor de óculo-plástica do Hospital das Clínicas de linfóides (linfomas e hiperplasias linfóides) e carcinomas no Ribeirão Preto (HCFMRP-USP), no período de 01/92 a 03/99. contexto das massas orbitais. Com efeito, os linfomas As biópsias obtidas das lesões foram examinadas no setor de orbitários foram os tumores mais freqüentes, seguidos pelos patologia cirúrgica do HCFMRP-USP. Foram analisadas nesse carcinomas, tumores vasculares e hiperplasias linfóides. período um total de 63 biópsias orbitárias. Todos os pacientes Para uma adequada comparação entre as séries das fre­ qüências relativas das neoplasias orbitárias, é necessário levar em consideração as diferentes classificações utilizadas pelos 1 1 1 Departamento de Oftalmologia e Otorrinolaringologia, Faculdade de Medicina de diversos autores ao longo do tempo, bem como os viéses dos Ribeirão Preto, Pós-graduando. serviços de referências. "' Faculdade de Medicina do Triângulo Mineiro, Professor adjunto da disciplina de A freqüência de distribuição de tumores orbitais, com oftalmologia, responsável pelo setor de órbita e óculo-plástica. '" Depa:1amento de Oftalmologia e Otorrinolaringologia, Faculdade de Medicina de relação ao diagnóstico histopatológico, no Registro de Pato­ Ribeirão Preto, responsável pelo setor de órbita e óculo-plástica. logia Ocular do Instituto de Patologia das Forças Armadas dos Endereço para correspondência: Prof. Dr. Antonio Augusto V. Cruz, Departamento de Oftalmologia, Hospital das Clínicas - Campus, Faculdade de Medicina de Ribeirão Estados Unidos (AFIP) (McLean et al., 1994) é semelhante à Preto - USP. Av. Bandeirantes. 3900. Ribeirão Preto (SP). CEP 14048-900. E-mail: freqüência das principais séries publicadas na literatura [email protected] anglo-saxônica (Shields et al., 1984). No registro da AFIP, http://dx.doi.org/10.5935/0004-2749.19990023 ARQ. BRAS. OFTAL. 62(3), JUNHO - 351 Órbita Ili - Tumores Orbitais: Ep idemiologia, Infiltrações linfóides e tumores da glândula lacrimal linfoma, fibro-histiocitoma e hiperplasia linfóide foram os 50 tumores mais comuns. Já para Henderson, os mais comuns foram os carcinomas secundários, linfomas e hemangiomas (Henderson, 1980). Por outro lado, segundo Kennedy, os tumores mais freqüentes são os linfomas, os cistos dermóides 40 e os hemangiomas (Kennedy, 1984). Finalmente, Shi�lds en­ controu como mais freqüentes os cistos dermóides, as fR o hiperplasias linfóides e linfomas (Shields et al., 1984). A nossa série é mais próxima da de Henderson, ou seja, ou 30 tumores mais comuns foram os linfomas (em primeiro lugar) e 5CD os carcinomas (em segundo), seguidos pelos hemangiomase "g hiperplasias linfóides em terceiro lugar. Fica evidente a im­ 2 portância dos tumores linfóides no contexto geral das patolo­ CD 20 gias orbitárias uma vez que nas quatro séries citadas, além da E nossa, os linfomas estão entre as três principais lesõe� em três •:S delas, e as hiperplasias linfóides aparecem também entre as z três lesões mais freqüentesem duas das grandes séries. 10 Dentre os tumores benignos, similarmente à série de Henderson, encontramos como mais freqüentesos he:nangio­ mas que, no nosso caso, igualaram-se em número às hiper­ plasias linfóides. o Interessante notar que não encontramos em nmsa série casos de fibro-histiocitoma, tumor mesenquimal orbitc'.rio mais Maligno Benigno comum do adulto, segundo dados da AFIP (McLean et al., 1994). Para alguns autores, a órbita é provavelmente um sítio de Fig. 1 - Distribuição detumores orbitais segundo critérios histopatológicos. predileção para esse tumor (Enzinger, Weiss, 1983; Font, Hidayat, 1982). Quando falamos de neoplasias malignas ou benignas, con­ vém ressaltar que os critérios de malignidade adotados para a classificação foram puramente histopatológicos. Algms tumo­ 50 res, como por exemplo os hemangiopericitomas, apresentam muitas vezes quadro histopatológico benigno contrastandocom comportamento biológico mais agressivo, com ocorrência de metástases (Henderson, Farrow, 1978). Em outros, como nas 40 hiperplasias linfóides, há possibilidade de associação com linfoma sistêmico pregresso, concomitante ao diagnéistico ou futuro, em aproximadamente 27% dos casos (Knowles et al., 1990). Deste modo, especialmente nesse último grupo, é neces­ 30 sário um adequado seguimento dos pacientes, de preferência com um acompanhamento por um hematologista, para trata­ mento da lesão orbitária e reavaliação periódica, buscando evidências de possível acometimento sistêmico. 20 INFILTRAÇÕES LINFÓIDES 10 Faixa Etária A idade dos portadores de tumores linfóides vari JU de 6 a 85 anos (média = 48,82 e mediana = 53 anos) sendo que 52,95% dos pacientes tinham 50 anos ou mais. o O detalhamento da faixa etária no grupo das infiltrações Prlmãrlos Secundãrlos linfóides, mostra que os linfomas de baixo grau tendem a ocorrer numa faixa etária mais alta, enquanto que cs de alto Fig. 2-Distribuição dos tumores orbitais segundo o sítio original da lesão. grau, ao contrário, aparecem mais precocemente (Figura 5). 352 - ARQ. ERAS. OFTAL. 62(3), JUNH0/1999 Órbita III - Tumores Orbitais: Ep idemiologia, Infiltrações linfóides e tumores da glândula lacrimal 25 casos em nossa série e 69 casos na série deles); em segundo lugar, mesmo com uma série reduzida, tivemos a oportunida­ de de observar dois casos de comprometimento orbitário por 20 linfomas sistêmicos de alto grau (linfomas linfoblásticos), o • o que não foi observado na série maior, pois nessa última, todos • Ili u 15 os linfomas eram de baixo grau ou de grau intermediário de CD 'ti malignidade. Os dois casos de pacientes jovens representaram e 11,76% de nossa casuística e, portanto, acabaram por dimi­ CD 10 E nuir o valor médio e da mediana da faixa etária. •::li z Alguns autores afirmam que o linfoma linfoblástico quan­ 5 do envolve a órbita é quase sempre como parte de doença sistêmica (McLean et ai., 1994 ). Os casos que observamos vêm de encontro aos dados da literatura. De maneira geral, o o linfoma linfoblástico é mais comummente visto em crianças e adolescentes, podendo também ser encontrado em adultos. Em aproximadamente metade dos casos há massa mediastinal na região tímica. O curso clínico da doença não tratada é Fig. 3-Distribuição dos tumores orbitais segundo a linhagem tecidual. extremamente agressivo, com rápida disseminação sistêmica, leucemização do sangue periférico e morte em alguns meses (Nathwani et ai., 1976). Os dois pacientes portadores deste Com relação à faixa etária dos pacientes portadores de tipo de linfoma foram os dois casos mais jovens de nossa tumores linfóides orbitais, observamos, assim como outros
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