Pérdida de agudeza visual progresiva en paciente con proptosis. Caso clínico y revisión de la literatura / Progressive visual impairment in a patient with proptosis. Case report and review of the literature.

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.

Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.

Undifferentiated pleomorphic sarcoma of the orbital region: a case report / Sarcoma pleomórfico indiferenciado da região orbitária: relato de caso

ABSTRACT Undifferentiated pleomorphic sarcoma (UPS) is an extremely rare tumor that occurs in the head and neck region. Here, we report a unique case of a primary undifferentiated pleomorphic sarcoma in the orbital region. A 35-year-old woman presented with a progressive proptosis and periocular edema for 1 year. She had no previous history of surgery, skin malignancy, or radiation. Imaging tests showed an extraconal mass, not involving the muscles. The tumor was surgically removed and adjuvant radiotherapy was required after histological examination, which showed an undifferentiated pleomorphic sarcoma of the orbit. There was no recurrence after 1 year of follow-up. Though rare, undifferentiated pleomorphic sarcoma should be included in the differential diagnoses of orbital tumors.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) é um tumor extremamente raro na região da cabeça e pescoço. Relatamos um caso de um sarcoma pleomórfico indiferenciado primário na região orbital. Uma mulher de 35 anos apresentou proptose progressiva e edema periocular há um ano. Ela não tinha histórico prévio de cirurgia, malignidade da pele ou radiação. Exames de imagem mostraram uma massa extraconal, poupando os músculos. O tumor foi removido cirurgicamente e foi necessária radioterapia adjuvante após o resultado histopatológico. O exame histológico demonstrou um sarcoma pleomórfico indiferenciado da órbita. Não houve recidiva após 1 ano de seguimento. Apesar de raro, o sarcoma pleomórfico indiferenciado deve ser incluído no diagnostico diferencial de qualquer tumor originado na órbita.

Plasmocitoma intraconal e infiltração uveal em paciente portadora de mieloma múltiplo / Intraconal plasmocytoma and uveal infiltration in a patient with multiple myeloma

Resumo Mieloma múltiplo (MM) é uma neoplasia que cursa com a proliferação desordenada de clones de plasmócitos, produzindo imunoglobulina monoclonal e normalmente se apresenta como lesões osteolíticas. Em alguns casos, porém, esta doença apresenta-se como massas, chamadas de plasmocitomas. O acometimento ocular e orbitário é incomum nesta patologia. Neste trabalho, descrevemos o caso de uma paciente de 63 anos com diagnóstico prévio de MM que evoluiu com um plasmocitoma intraconal em olho direito, bem como uma massa vascularizada câmara anterior proveniente de infiltração uveal. Essas lesões foram correlacionadas MM e culminaram com a perda visual no olho acometido. Não foi encontrado na literatura relatos de plasmocitoma intraconal.

Abstract Multiple myeloma (MM) leads to disorderly proliferation of plasma cells clones, producing monoclonal immunoglobulin and commonly presents osteolytic lesions. In some cases, however, masses called plasmocytomas are found. Ocular and orbital involvement is unusual in this pathology. In this paper, we describe a case of a 63 year-old patient with previous diagnostic of MM that evolved an intraconal plasmocytoma in the right eye, as well as a vascularized mass in the anterior chamber from uveal infiltration. These lesions were correlated to MM e lead to visual loss in the affected eye. Reports of intraconal plasmocytoma have not been found in literature.

Orbital apocrine hidrocystoma with immunohistochemical confirmation / Hidrocistoma apócrino orbitário com confirmação imunohistoquímica

ABSTRACT The authors report the first case in Brazilian literature of orbital apocrine hidrocystoma with immunohistochemistry confirmation. The tumor had been growing slowly and progressively, there was no history of impaired vision, diplopia, watering, discharge, or prior trauma. There was no proptosis and extraocular mobility was normal. The radiologic study by orbital computerized tomography revealed an extraconal nodule, with partially defined limits, with cystic and solid areas in the superomedial right orbit. An anterior orbitotomy, with full excision of the tumor was performed. A histopathology examination revealed apocrine hidrocystoma and immunohistochemistry confirmed the diagnosis. After surgery, the patient had total remission of symptoms.

RESUMO Os autores relatam o primeiro caso de hidrocistoma apócrino orbitário com confirmação imunohistoquímica. O tumor apresentou crescimento lento e progressivo, sem relato de baixa de acuidade visual, diplopia, epífora, secreção ou trauma prévio. Não houve proptose ou alteração da motilidade extraocular. O exame de imagem por tomografia computadorizada da órbita revelou uma lesão nodular, extraconal, de limites imprecisos, com áreas císticas e sólidas no seu interior, na região súpero-medial de órbita direita. O paciente foi submetido à orbitotomia por via anterior, com exérese integral da tumoração. O exame histopatológico revelou o diagnóstico de hidrocistoma apócrino e a imunohistoquímica confirmou o diagnóstico. Após a cirurgia, o paciente teve regressão total dos sintomas.

Successful Treatment of Orbital Lymphangioma with Intralesional Bleomycin and Application of Continuous Negative Pressure

No abstract available.

Tumor of orbit / Tumor orbitário

The cavernous hemangiomas are the most common intra orbital tumors found in adults of the middle age. Although histological benign, they can encroach on intra orbital or the adjacent structures (optic nerve) and be considered anatomically or positional malignant. We present a case report of orbital cavernous hemangioma of right orbit in young women after pregnancy, from Topola near Kragujevac (Central Serbia) with visual compromise and it's by trans-nasal endoscopic surgical management. Our patient was controlled and treated with the symptomatic therapy, topical therapy with artificial tears and surgical treatment. Our patient has optimal visual acuity of affected right eye after surgical treatment of orbital tumor. Surgical treatment of symptomatic orbital cavernous hemangioma is safe and effective, so that the cosmetic results are the important parameter to evaluate the clinical outcome.

Os hemangiomas cavernosos são os tumores intraorbitais mais comuns encontrados em adultos de meia-idade. Embora histológico benigno, eles podem invadir a área orbital ou intraorbital ou ainda as estruturas adjacentes (nervo óptico) e ser considerado anatomicamente ou posicional maligno. Apresentamos um relato de caso de hemangioma cavernoso orbital da órbita direita em mulheres jovens após a gravidez, a partir de Topola perto Kragujevac (Central Sérvia), com comprometimento visual e medicado por tratamento cirúrgico endoscópico transnasal. O paciente foi controlado e tratado com terapia, terapia tópica sintomático de lágrimas artificiais e tratamento cirúrgico. Nosso paciente tem acuidade visual ideal do olho direito afetada após o tratamento cirúrgico de tumor orbital. O tratamento cirúrgico do sintomático hemangioma cavernoso orbital é segura e eficaz, de modo que os resultados cosméticos são o parâmetro importante para avaliar o resultado clínico.

Extranodal Nasal NK/T-Cell Lymphoma: A Rare Oral Presentation and FASN, CD44 and GLUT-1 Expression

Extranodal natural killer (NK)/T-cell lymphoma is an aggressive malignant tumor with distinctive clinicopathological features, characterized by vascular invasion and destruction, prominent necrosis, cytotoxic lymphocyte phenotype and a strong association with Epstein-Barr virus. Here is reported an extranodal nasal NK/T-cell lymphoma case, involving the maxillary sinus, floor of the orbit, and interestingly extending to the oral cavity through the alveolar bone and buccal mucosa, preserving the palate, leading to a primary misdiagnosis of aggressive periodontal disease. Moreover, this work investigated for the first time the immunohistochemical expression of fatty acid synthase (FASN) and glucose transporter 1 (GLUT-1) proteins in this neoplasia. FASN showed strong cytoplasmatic expression in the neoplastic cells, whereas GLUT-1 and CD44 were negative. These findings suggest that the expression of FASN and the loss of CD44 might be involved in the pathogenesis of the extranodal nasal NK/T-cell lymphoma, and that GLUT-1 may not participate in the survival adaptation of the tumor cells to the hypoxic environment. Further studies with larger series are required to confirm these initial results.

O linfoma de células natural killers (NK)/T extranodal é um tumor maligno agressivo com características clinicopatológicas distintas, caracterizadas por invasão e destruição vasculares, necrose proeminente, fenótipo linfocítico citotóxico e uma forte associação com o vírus Epstein-Barr. Relatamos aqui um caso de linfoma de células NK/T nasal extranodal, envolvendo o seio maxilar, assoalho de órbita, e interessantemente estendendo-se para a cavidade oral através do osso alveolar e mucosa vestibular, preservando o palato, levando a um diagnóstico inicial equivocado de doença periodontal agressiva. Ainda, nós investigamos pela primeira vez a expressão imunoistoquímica das proteínas Fatty acid sinthase (FASN) e glucose transporter 1 (GLUT-1) nesta neoplasia. FASN revelou uma forte expressão citoplasmática nas células neoplásicas, enquanto GLUT-1 e CD44 foram negativas. Estes achados sugerem que a expressão de FASN e a perda de CD44 podem estar envolvidas na patogênese do linfoma de células NK/T nasal extranodal, e que GLUT-1 não deve participar da adaptação das células tumorais ao ambiente de hipóxia. Estudos adicionais com séries maiores são necessários para confirmar nossos resultados iniciais.

Les tumeurs orbitaires aspects épidémiologiques cliniques et thérapeutiques à propos de 27 cas colligés à l'hôpital

Objectifs : Les objectifs de ce travail sont : de déterminer les aspects épidémiologiques, cliniques et les résultats thérapeutiques d'une étude faite chez les patients présentant une tumeur de la région orbitaire, puis de proposer une stratégie adaptée aux réalités locales de prise en charge des patients en milieu hospitalier Nigérien. Méthodes : Il s'agit d'une étude rétrospective et prospective effectuée à l'Hôpital National de Niamey du 31 Juillet 1998 au 31 Juillet 2012 avec un suivi de 12 mois. Sont inclus dans notre étude les personnes de tous les sexes et âges présentant des troubles visuels et ou des signes d'hyperpression intra orbitaire et ayant pu bénéficier d'un bilan suffisant aboutissant au diagnostic certain de tumeur orbitaire et d'une intervention chirurgicale. Résultats : Dans cette étude durant ces quatorze (14) dernières années, les tumeurs orbitaires ont représenté 7,8% de tumeurs intracrâniennes opérées à Niamey. Le sexe féminin était le plus touché soit 55,56% avec un sex ratio homme/femme de 0,8. L'âge moyen est de 28,82 ans avec des extrêmes de 4 mois et de 75 ans. La tranche d'âge la plus représentée est celle allant de 16 à 45 ans soit 51,85%. Les méningiomes sphéno-orbitaires sont les tumeurs les plus fréquemment représentées soit 22,22% . L'exophtalmie est le motif de consultation le plus fréquent soit 96,26%. Le diagnostic clinique a été complété dans 77,77% des cas par un bilan ophtalmologique avec une baisse de l'acuité visuelle dans 62,96% des cas, une perte de la perception lumineuse dans 18,51% des cas et un fond d'œil anormal dans 48,14% des cas. Le diagnostic est fait par un examen radiologique (radiographie standard du crâne dans 7,4% des cas ; scanner crânio-orbitaire 77,77% des cas, l'IRM dans 3,7% des cas) et aussi par un examen anatomopathologique dans 62,96% des cas. Le traitement effectué dans la majorité des cas est chirurgical par voie transcraniale dans 100% des cas. Une exérèse de la tumeur dans 81,14% des cas. Le traitement adjuvant post opératoire par la chimiothérapie s'est effectué chez 22,22% des patients. Au bout de trois(3) mois post opératoire : Les patients qui étaient admis avec une cécité sont restés aveugles. Dans 41,17% des cas nous avons eu une amélioration de l'acuité visuelle avec un gain moyen de 4,5/10 .. Nous notons deux cas de complications. La première est infectieuse à trois mois post opératoire à type d'ostéite bulleuse fronto-ethmoïdale. La deuxième est une récidive d'un méningiome intra orbitaire avec une atteinte osseuse au bout de trois (3) ans postopératoire. Tous ces deux patients ont bénéficié avec succès d'une seconde intervention (second look) . Conclusion : Cliniquement identique, le diagnostic de toutes les tumeurs orbitaires se fait à Niamey par l'association d'un bilan ophtalmologique, d'un bilan radiologique ; d'un examen anatomopathologique pour confirmer le diagnostic

Metástasis enoftalmiantes de la órbita: una excepción a la regla / Enophthalmos due to orbit metastasis: an exception to the rule

De los tipos de cáncer que metastatizan la órbita, el de la mama, el pulmon y la próstata ocupa un lugar preeminente. En ocasiones el diagnóstico del tumor primario permanece elusivo a pesar de una evaluación exhaustiva. La mayoría de las veces producen proptosis ocular y son fácilmente detectados. En otras producen todo lo contrario, enoftalmía, haciendo entonces el problema menos aparente y el diagnóstico elusivo. La metástasis mamaria enoftalmiante de la órbita es un raro evento y debe ser considerada como un diagnóstico diferencial inicial en mujeres maduras con enoftalmía no traumática. En elpresente trabajo presentamos una serie de ocho pacientes con carcimona lobulillar infiltrante y escirroso de la órbita productor de enoftalmía, describimos sus características clínicas y hacemos notar su mal pronóstico vital

Of the types of cancer that metastatize the orbit, the breast, lung and prostate occupy a pre-eminet place. Sometimes the diagnosis of the primary tumor remains elusive despite a thorough assessment. Most of the times produce eye proptosis and are easily detected. In other cases they produce just the oppsite, enophthalmos, then doing the problem least apparent and the diagnosis elusive. Enopthalmos and breast metastasis are a rare event and should be considered as an initial differential diagnosis in especially in mature women with non-traumatic enophthalmos. In this paper we present a series of eight patients with infiltrating lobulillar and scirrous carcinoma of the orbit producing enophthalmos, we described their clinical features and do note their poor vital prognosis

Orbital lymphangioma in a child: a diagnostic dilemma / Linfangioma orbitaria en un niño: un diagnóstico dilema

We report a case of significant unilateral left proptosis after a fall in a 10-year old child. Magnetic resonance imaging showed an extraconal hyperintense orbital mass extending into the maxillary sinus which was opacified. After drainage the proptosis resolved. The cause of the acute proptosis was haemorrhage within an orbital lymphangioma.

Se reporta el caso de una proptosis izquierda unilateral significativa en un niño de diez años de edad después de una caída. La tomografía por resonancia magnética mostró una masa orbitaria hiperintensa extracónica que se extendía hasta adentro del seno maxilar, opacificándolo. Después del drenaje, la proptosis se resolvió. La causa de la proptosis aguda fue la hemorragia dentro de un linfangioma orbitario.

Clinical and histopathological features of orbital granular cell tumor: case report / Características clínicas e histopatológicas de tumor de células granulares da órbita: relato de caso

A 53 year-old woman presented with a slowly progressive, painless proptosis OS. Computed tomography disclosed a round, homogeneous, well-delimited lesion in the inferior-temporal orbit. The tumor was composed of round cells with eosinophilic granular cytoplasm. Some of the cells had larger eosinophilic granules surrounded by a clear halo; known as pustulo-ovoid bodies of Milian or Bangle bodies. The diagnosis of a granular cell tumor was then established and confirmed by immunohistochemistry. Granular cell tumors are uncommon benign soft tissue neoplasms that have a predilection for the head and neck region. Awareness of the typical histopathological features is crucial for the correct diagnosis.

Mulher de 53 anos apresentou proptose lentamente progressiva no olho esquerdo. Tomografia computadorizada mostrou uma lesão na região temporal inferior da órbita esquerda, bem delimitada, arredondada, homogênea. O tumor era composto de células com citoplasma granular eosinofilico. Algumas das células possuíam grandes grânulos eosinofílicos circundados por um halo claro, conhecidos como corpos ovoides-pustulares de Milian or corpos de Bangle. O diagnóstico de tumor de células granulares foi estabelecido, confirmado pela imuno-histoquímica. Tumor de células granulares são neoplasias incomuns com predileção da região da cabeça e pescoço. O conhecimento das características histopatológicas típicas são cruciais para o correto diagnóstico.

A Case of Histiocytoid Variant Eccrine Sweat Gland Carcinoma of the Orbit

A 79-year-old male presented with left ocular pain. Evisceration and silicone ball implantation were performed after a diagnosis of phthisis. He returned six weeks later because of left facial erythematous swelling, tenderness, mild fever, chills and cough. His condition was diagnosed as orbital cellulitis. Despite two weeks of empirical antibiotic therapy, the symptoms worsened. A subsequent orbital computed tomography scan revealed enhanced soft tissue infiltrations in his left orbit and eyelid. Biopsy showed a diffusely infiltrating tumor of signet ring cell cytology. A systemic evaluation revealed multiple bone metastases. Based on this evidence, the patient was diagnosed with a very rare case of histiocytoid variant eccrine sweat gland carcinoma with multiple bone metastases.

Rosai-Dorfman disease of the paranasal sinuses and orbit

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign indolent disorder, characterized by enlarged lymph nodes filled with histiocytes. Extranodal involvement is uncommon. The disease rarely affects the nose and paranasal sinuses. We report a case that presented with a right nasal mass, extending into all the paranasal sinuses and right orbit without any accompanying lymphadenopathy. Because of the absence of lymphadenopathy it posed a diagnostic challenge until the pathology was confirmed on histopathological examination

Tumor de la órbita como forma poco común de presentación de un linfoma no hodgkiniano extranodal de la zona marginal / Orbit tumor as an uncommon way of presentation of a no-Hodgkin extranodal lymphoma of marginal zone

Los linfomas no hodgkinianos (LNH) representan la mitad de los tumores de la órbita, sin embargo, su incidencia en nuestro medio es poco frecuente. Se presenta una paciente de 52 años de edad, femenina, blanca, con antecedentes patológicos personales de hipertensión arterial, sin otros datos relevantes al interrogatorio, que acude a consulta por epífora y discreta ptosis palpebral del ojo derecho acompañada de tumoración dura y fija en zona ínfero medial de la órbita derecha. Las principales pruebas de laboratorio no mostraron nada relevante. En la resonancia magnética se comprobó tumoración de 2 × 1 cm, de contornos irregulares en relación con la pared posteroinferior de la órbita derecha. Se realizó biopsia de la lesión que se concluyó como un LNH con inmunofenotipo compatible con proceso linfoproliferativo de linfocitos B kappa. En la biopsia de médula ósea se informó infiltración nodular por proceso linfoproliferativo de bajo grado. Se concluyó el caso como un LNH primario de la órbita tipo zona marginal con expresión leucémica de células B monocitoides en estadio IVA. Se realizaron 8 ciclos de CHOP y se obtuvo respuesta parcial. Posteriormente se pasó a un esquema de segunda línea consistente en rituximab-fludarabina-ciclofosfamida-dexametasona, que se mantiene.

The no-Hodgkin lymphomas (NHL) accounted for the half of orbit tumor; however, its incidence in our environment is very uncommon. This is the case of a white female patient aged 52 with personal pathological backgrounds of high blood pressure without other relevant data in questioning, referred to consultation due to epiphora and a discrete palpebral ptosis of right eye accompanied by hard and fixed tumor in inferior medial of right orbita. Main laboratory tests were normal. In magnetic resonance (MR) it was demonstrated the presence of a 2 x 1 cm tumor with irregular borders in relation to posteroinferior wall of right orbit. A lesion biopsy was carried out with diagnosed as a primary NHL con immunophenotype compatible with a lymphoproliferative process. We conclude that this case is a primary orbit NHL of principal zone with leukemic expression of monocytic B cells in IVA state. We performed 8 cycles of CHOP with a partial response. Subsequently, we passed to a second-line maintained scheme consistent in Rituximab-Fludarabine-Cyclophosfamide-Dexamethasone.

Linfangioma orbitário: relato de caso / Orbital lymphangioma: case report

Descrevemos um caso de linfangioma orbitário em uma paciente de nove anos de idade que apresentava proptose à direita (Hertel= 29 mm), acompanhada de restrição da motilidade ocular, dor e perda visual decorrente de neuropatia óptica compressiva. A ressonância magnética demonstrou a lesão expansiva, localizada na órbita direita, de aspecto cístico, não infiltrativa, extraconal e com sinais sugestivos de hemorragia intralesional. Não houve melhora com corticoterapia oral. Foi, então, realizada punção via transconjuntival, com aspiração de 35 ml delíquido "cor-de-chocolate" (confirmado como hemorrágico pela citologia). Ocorreram exacerbações do quadro clínico, manifestadas por dor e piora da proptose, devido à hemorragia intralesional, optando-sepela exérese dos cistos orbitários, usando o acesso orbitário lateral (cantólise e retirada de parede orbitária lateral, a qual não foi recolocada para efeito de descompressão) e inferior (transconjuntival inferior), com resolução do quadro. CONCLUSÃO: Foi relatado um caso de linfangioma, uma doença desafiadora, de difícil tratamento, com potenciais complicações visuais e estéticas, além da possibilidade de recidivas frequentes.

A case of orbital lymphangioma in a 9 year-old female, with proptosis (Hertel= 29 mm), ocular motility restriction, pain and visual loss due to compressive optic neuropathy is described. A magnetic resonance imaging (MRI) exam showed an expansive cystic lesion in the extraconal space of the right orbit, non-infiltrating with intralesional hemorrhage. The patient had no improvement with steroid treatment. A transconjunctival puncture with 35 ml aspiration of a "chocolate color" fluid (which was confirmed by cytologic study as a hemorrhagic fluid) was performed. Exacerbations occurred after an intralesional hemorrhage (pain and increase of proptosis). The lesion was excised via lateral cutaneous approach (a lateral canthotomy and removal of the right orbital wall was performed done for decompression) and lower transconjunctival approach. CONCLUSION: Lynphangioma is a challenging disease and difficult to treat, with potential visual and cosmetic complications and the possibility of frequent recurrences.

Metastatic Esophageal Squamous Cell Carcinoma to the Orbit and Periorbit Masquerading as Periorbital Abscess

A 61-year-old man with no history of malignancy presented with a rapidly expanding left periorbital mass, first noticed one month prior to presentation. The mass was firm, and a pus-like discharge drained spontaneously from the center of the lesion. A biopsy was performed, and histopathology confirmed squamous cell carcinoma. Systemic evaluation revealed that the patient had a primary esophageal squamous cell carcinoma with multiple metastases. The prognosis of orbital metastasis is generally poor, and this patient expired after one month of conservative treatment.

Orbital alveolar soft part sarcoma: histopathologic report of two cases

Alveolar soft part sarcoma is considered as a distinct histopathological entity with rare cases reported from the orbit area. Two cases of alveolar soft part sarcomas occurring in the orbit of two patients along with their histopathologic findings are reported herewith. In both cases, the patients presented with eyelid swelling and proptosis. The diagnosis was made by incisional biopsies and histopathology. The literature is reviewed regarding occurrence of this tumor, its diagnosis and management

Congenital orbital teratoma presenting as microphthalmos with cyst.

We report a rare case of non-communicating large orbital cyst with microphthalmos which was surgically separated from the globe and excised. Histopathology reported it to be a teratoma. Congenital cystic teratoma should be a part of the differential diagnosis in an infant presenting with a clinical picture of microphthalmos with orbital cyst, in view of the different management required.

Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit.

Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.