Ophthalmic manifestations of nasopharyngeal carcinoma (NPC): A systematic review of reported cases with and without prior history of nasopharyngeal carcinoma

ABSTRACT Purpose: We aimed to study reported cases of nasopharyngeal carcinoma presenting with ophthalmic manifestations with and without a prior diagnosis of nasopharyngeal carcinoma. Methods: We conducted a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). A literature search was conducted using the MEDLINE database in PubMed and Google Scholar. We included patients with a previous diagnosis of nasopharyngeal carcinoma in Group I and those without a prior diagnosis of nasopharyngeal carcinoma in Group II. Data included demographics, clinical presentation, history of nasopharyngeal carcinoma, treatment, histopathological description, World Health Organization classification, and outcome. Results: Fifty-eight patients (26 in Group I and 32 in Group II) were included. The male-to-female ratio was 3:1. The mean age of the patients (53.3 ± 11.7 years and 54.8 ± 16.2 years, respectively) and gender did not differ significantly between the two groups. The most common ocular presentations were diplopia and proptosis in the first group (each in 34.6%), whereas visual disturbance was most common in the second group (46.9%). Treatment options and World Health Organization grading were comparable. The outcome in 38 patients (after a comparable follow-up period) was significantly better in group II (p=0.003). There was no statistically significant difference in the outcome of 23 patients in correlation with World Health Organization grades II versus III irrespective of group (p=0.094). Conclusions: The demographics of patients with nasopharyngeal carcinoma presenting with ophthalmic manifestations were similar between the two study groups, with a wide age range and male predominance. Patients presenting initially to ophthalmologists with no history of nasopharyngeal carcinoma have a more favorable outcome. World Health Organization grading may have less value as a prognostic indicator.

Quality of life in patients with Graves' orbitopathy submitted to orbital decompression: comparison between balanced and inferomedial techniques

ABSTRACT Purpose: To compare inferomedial wall orbital decompression to balanced medial plus lateral wall orbital decompression in patients with Graves' orbitopathy in the inactive phase with regard to exophthalmos reduction and the effects on quality of life. Methods: Forty-two patients with inactive Graves' orbitopathy were randomly divided into two groups and submitted to one of two orbital decompression techniques: inferomedial wall orbital decompression or medial plus lateral wall orbital decompression. Preoperative and postoperative assessments included Hertel's exophthalmometry and a validated Graves' orbitopathy quality of life questionnaire. The results of the two groups were compared. Results: Compared to preoperative measurement, exophthalmos reduction was statistically significant in both groups (p<0.001) but more so in patients undergoing medial plus lateral wall orbital decompression (p=0.010). Neither orbital decompression techniques increased the visual functioning subscale score on the Graves' orbitopathy quality of life questionnaire (inferomedial wall orbital decompression p=0.362 and medial plus lateral wall orbital decompression p=0.727), but a statistically significant difference was observed in the score of the appearance subscale in patients submitted to medial plus lateral wall orbital decompression (p=0.006). Conclusions: Inferomedial wall orbital decompression is a good alternative for patients who do not require large exophthalmos reduction. However, medial plus lateral wall orbital decompression offers greater exophthalmos reduction and greater improvement in appearance (higher Graves' orbitopathy quality of life questionnaire scores), making it a suitable option for esthetic-functional rehabilitation.

Treatment of orbital lymphatic malformation with oral sirolimus: a case report / Malformação linfática orbital tratada com sirolimo oral: relato de caso

ABSTRACT It is estimated that lymphatic malformations in children account for 6% of all benign vascular malformations. New medical therapies have been developed for the management of lymphatic orbital disease. The purpose of this article was to describe a clinical case of orbital venolymphatic malformation in a 10-year-old boy, causing proptosis and palpebral edema. The lesion was initially treated with local sclerotherapy. However, the lesion relapsed, and was successfully treated with oral sirolimus. Prospective studies are warranted to determine the appropriate dose and extend the indications of sirolimus in these patients.

RESUMO A incidência de malformações linfáticas em crianças é estimada em 6% de todas as malformações vasculares benignas. Têm sido desenvolvidos novos tratamento para doenças linfáticas orbitárias. Nosso objetivo é descrever um caso clínico de malformação venolinfática orbitária em um menino de 10 anos de idade, causando proptose e edema palpebral. A lesão foi tratada inicialmente com escleroterapia local. No entanto, a lesão teve recidiva e foi tratada com sucesso com sirolimo oral. Ainda são necessários estudos prospectivos para estabelecer a dose apropriada e a duração do tratamento com sirolimo nesses pacientes.

Systemic sarcoidosis presenting as acute rapidly progressive proptosis / Sarcoidose sistêmica apresentando-se como proptose aguda rapidamente progressiva

ABSTRACT Sarcoidosis is a generalized systemic chronic inflammation that rarely involves the orbit. As a chronic inflammation, sarcoidosis typically manifests with an insidious onset and slowly progressive course. We report a case of acute-onset proptosis resulting from a rapidly growing diffuse orbital mass that simulated malignant growth, which was biopsy proven to be the first manifestation of systemic sarcoidosis. The patient demonstrated complete resolution of proptosis and systemic involvement with long-term corticosteroid treatment.

RESUMO A sarcoidose é uma inflamação sistêmica generalizada que raramente envolve a órbita. Sendo uma inflamação crônica, a sarcoidose geralmente se manifesta com um início insidioso e uma evolução lentamente progressiva. Neste artigo, é relatado um caso de proptose de início agudo, resultante de uma massa orbitária difusa de rápido crescimento, simulando um crescimento maligno, mas cuja biópsia comprovou ser a primeira manifestação de uma sarcoidose sistêmica. O paciente apresentou resolução completa da proptose e do envolvimento sistêmico com tratamento de longo prazo com corticosteroides.

Exoftalmos bilateral por compromiso orbitario debido a enfermedad relacionada con IgG4 / Bilateral exophthalmos due to orbital involvement due to IgG4-related disease

La enfermedad relacionada con IgG4 (ER-IgG4) se caracteriza por el compromiso fibrótico e infiltrado linfoplasmocitario con predominio de células IgG4 en uno o varios órganos. El fenotipo limitado a la cabeza y el cuello abarca el compromiso de las glándulas lagrimales y salivares, enfermedad orbitaria, paquimeningitis, hipofisitis, tiroiditis, mastoiditis y compromiso de senos paranasales. Las manifestaciones orbitarias pueden incluir proptosis uni o bilateral, miopatía cicatrizal de los músculos extraoculares, inflamación orbitaria y dolor que pueden simular a la oftalmopatía de Graves. La enfermedad oftálmica relacionada con IgG4 puede presentarse con compromiso de músculos extraoculares y de grasa orbitaria, y usualmente se confunde con afección orbitaria por enfermedad de Graves. Se presenta el caso de un varón de 58 años, con historia de exoftalmos bilateral de más de 15 años de evolución, en quien se arriba al diagnóstico de ER-IgG4 y cuya resonancia magnética de órbitas evidencia un patrón característico de compromiso de músculos extraoculares.

IgG4-related disease (IgG4-ER) is characterized by fibrotic involvement and lymphoplasmacytic infiltrate with IgG4 cells predominance in one or several tissues. The head and neck phenotype includes lacrimal and salivary gland involvement, orbital disease, pachymeningitis, hypophysitis, thyroiditis, mastoiditis, and sinus involvement. Orbital manifestations may include unilateral or bilateral proptosis, extraocular muscles scarring, orbital inflammation, and pain that may mimic Graves' ophthalmopathy. IgG4-related ophthalmic disease may present with extraocular muscles and orbital fat involvement and is often confused with orbital Graves' disease. We present the case of a 58-year-old man, with a history of bilateral exophthalmos of more than 15 years, in whom the diagnosis of IgG4-ER was reached and whose orbits magnetic resonance showed a characteristic pattern of extraocular muscle involvement.

Spheno-orbital meningiomas: Is orbit reconstruction mandatory? Long-term outcomes and exophthalmos improvement.

Background: Meningiomas correspond to one-third of all primary central nervous system tumors. Approximately 9% of them are spheno-orbital meningiomas (SOMs), presenting significant clinical symptoms as visual impairment and orbital esthetics. This article aims to evaluate exophthalmos' improvement in a surgical series without orbital reconstruction. Methods: We consecutively included all patients diagnosed with SOM, admitted to a single institution for 10 years. Surgical resection was the standard of care, associated or not with adjuvant radiation therapy. The radiological investigation included preoperative and postoperative head CT or MRI. We quantified proptosis through imaging. Results: Forty patients composed this series, 87.5% were female. Proptosis was the most common presentation (90%), followed by decreased visual acuity (65%), motility deficit (20%), and headache (20%). Gross total resection was achieved in 65% of the procedures. In late outcomes, 78% of the patients maintained or improved visual acuity and 85% maintained or improved headache. Proptosis significantly improved after surgery and along with the follow-up (P < 0.001). Ten patients were submitted to adjuvant RT, six of them after a subtotal resection. All patients of this subgroup had proptosis. It was observed a higher frequency of worse in visual acuity in patients submitted to RT (71% vs. 28%, P = 0.038). Conclusion: Resection of SOM was sufficient to stop the evolution of visual deficit and allowed the improvement of proptosis. Orbital reconstruction does not seem to be an essential step in reducing enophthalmos.

Computed tomography findings of Crouzon syndrome: A case report.

Crouzon syndrome is a genetic condition characterized by a premature fusion of skull sutures resulting in head and facial deformities. Crouzon syndrome is usually suspected at birth through physical examination or in the antenatal period via ultrasonographic assessment. Once Crouzon syndrome is suspected, advanced imaging methods such as three-dimensional computed tomography must be requested, showing early signs of cranial sutures fusion. In this paper, we present a case of a six-year-old girl who was taken to a pediatrician control appointment due to abnormal facies. During the physical examination, a suspicion of Crouzon syndrome was raised. Therefore, a head computed tomography was requested, showing asymmetrical calvarium thickening, diffuse indentation of the inner table of the skull, and moderate hydrocephalus with a big cyst in the posterior fossa. Due to these findings, the patient was remitted to maxillofacial surgery for further evaluation; however, the medical appointment could not be achieved as a consequence of the poor medical insurance of the girl. This paper aims to describe and discuss the computed tomography findings of Crouzon syndrome.

Oral selenium improved the disease activity in patients with mild Graves' orbitopathy.

BACKGROUND: Graves' orbitopathy (GO) is the most common extrathyroidal manifestation of Graves' disease (GD), an autoimmune disorder arising from the activity of T lymphocytes against antigens that infiltrate thyroid tissue, orbital tissue and extraocular muscles. An increase in oxidative stress has been discovered in autoimmune thyroid disease, encouraging investigation into new forms of treatment. Selenium has been described as a treatment option given its antioxidant properties. The present study evaluates the decrease of progression and inflammatory signs in patients with mild GO with oral selenium supplementation. METHODS: Controlled, randomized, single center trial at an ophthalmology referral center in Mexico City. Patients at least 18years of age with mild GO according to the CAS classification were included; exclusion criteria in addition to corticosteroid treatment included smokers or selenium allergy. Each patient was randomized into one of two groups. Group A took placebo tablets which consisted of 100µg of starch twice a day for 6months, and group B took a 100µg selenium tablet twice a day for 6months. The patients from both groups were examined and evaluated using a CAS score before and after the first, third and sixth month of treatment. RESULTS: Thirty eyes of 30 patients were studied. The pretreatment values showed no statistically significant differences between groups (P>0.05). Intergroup analysis showed statistically significant differences in palpebral fissure and CAS score between the pretreatment values and six months after treatment in the selenium group (P<0.05). No differences were found in any variables in the placebo group during the study period (P>0.05). No adverse events were reported. CONCLUSIONS: This is the first study in a Mexican population demonstrating that oral selenium decreases clinical activity and stops progression in patients with mild GO.

Características de orbitopatía asociada al tiroides y tasa de requerimiento de cirugía / Thyroid-associated orbitopathy characteristics and surgery requirement rate

RESUMO Objetivo: Conheça as características demográficas e clínicas da Órbita Associada da Tiroide (OAT), bem como a taxa de exigência da cirurgia orbital em pacientes do Centro Médico Nacional do Oeste. Métodos. Estudo observacional, transversal, descritivo e retrospetivo realizado analisando os registos de pacientes diagnosticados com OAT tratados num centro de cuidados de terceiro nível de janeiro de 2005 a julho de 2016. Os resultados. Um total de 236 órbitas de 118 pacientes foram avaliados, com uma idade média de 47,3 (13,2 anos, 74,6% eram do sexo feminino e 25,4% masculinos. 4,2% dos doentes foram tratados com hipotiroidismo, 94,1% com hipertireoidismo e 1,7% com goiter tóxico difuso. 44,9% dos doentes estudados com restrição de movimento ocular,10,2% com queratopatia de exposição e 51,7% com hipertensão intraocular. 34,7% dos doentes avaliados no serviço necessitaram de descompressão orbital, 16,1% de cirurgia palpebral e 8,5% de correção do hatrabisma. Na gestão conservadora destes doentes, 48,3% exigiam o uso de lubrificantes tópicos dos olhos, enquanto 52,5% dos pacientes necessitavam do uso de hipotensivos oculares em número variável. As conclusões. A OAT foi associada principalmente ao hipertiroidismo, sendo mais comum em pacientes do sexo feminino entre os 40 e os 59 anos; mais de 50% dos pacientes necessitaram do uso de hipotensivos oculares. Da mesma forma, a gestão cirúrgica foi realizada em mais de 50% dos pacientes, sendo a descompressão orbital a intervenção mais frequente.

ABSTRACT Objective. To know the demographic and clinical characteristics of Thyroid Associated Orbitopathy (TAO), as well as the requirement rate of orbital surgery in patients of the Orbit Service in the National Medical Center of the West, IMSS. Methods. Observational, cross-cutting, descriptive and retrospective study carried out analyzing the records of patients diagnosed with TAO and treated at a third-level care center from January 2005 to July 2016. Results. A total of 236 orbits of 118 patients were valued, with an average age of 47.3 ± 13.2 years, 74.6% were female and 25.4% male. 4.2% of patients were treated with hypothyroidism, 94.1% with hyperthyroidism and 1.7% with diffuse toxic goiter. 44.9% of patients studied had eye movement restriction,10.2% exposure keratopathy and 51.7% intraocular hypertension. 34.7% of patients valued in the service required orbital decompression, 16.1% palpebral surgery and 8.5% strabism correction. In the conservative management of these patients 48.3% required the use of topical eye lubricants, while 52.5% required the use of eye hypotensives in variable numbers. Conclusions. TAO was mainly associated with hyperthyroidism, being more common in female patients between the age of 40 and 59; more than 50% of patients required the use of eye hypotensives. Likewise, surgical management was performed in more than 50% of patients, with orbital decompression being the most frequent intervention.

Ocular motility changes after inferomedial wall and balanced medial plus lateral wall orbital decompression in Graves' orbitopathy: a randomized prospective comparative study

OBJECTIVES: To compare the surgical outcomes of inferomedial wall orbital decompression (IM-OD) and balanced medial plus lateral wall orbital decompression (ML-OD) in patients with inactive Graves' orbitopathy (GO) with regard to exophthalmos reduction and ocular motility abnormalities. METHODS: Forty-two patients with inactive GO eligible for OD were randomly assigned to either the IM-OD or ML-OD groups. Pre and postoperative evaluations included Hertel exophthalmometry, sensory, and motor extraocular motility assessment, standardized photographs in the nine gaze positions, and computed tomography (CT) of the orbits. ClinicalTrials.gov: NCT03278964. RESULTS: Exophthalmometry reduction was statistically significant in both groups (p<0.001), but was greater in the ML-OD group (p=0.010). New-onset esotropia occurred in 11.1% and 23.5% of patients who underwent IM-OD and ML-OD, respectively, with no statistically significant difference in the frequency of pre and postoperative strabismus in either group. The mean increase in preoperative esotropia was 24±6.9 and 12±8.8 prism diopters in patients who underwent IM-OD and ML-OD, respectively. In the IM-OD group, abduction and elevation worsened at the first (p<0.05) and third (p<0.05) postoperative visits but were restored at 6 months. The versions did not change postoperatively with ML-OD. The preoperative CT-measured medial rectus muscle area predicted new-onset strabismus (p=0.023). Significant postoperative medial rectus muscle enlargement occurred in both groups (p<0.001). Restriction in elevation and abduction was significantly associated with enlarged inferior (p=0.007) and medial rectus muscle areas (p=0.002). CONCLUSIONS: IM-OD is as safe as ML-OD with regard to new-onset strabismus, and represents a good alternative for patients who do not require significant exophthalmos reduction. ML-OD offers greater exophthalmos reduction and smoother postoperative recovery. Patients with preoperative enlarged medial rectus muscle on CT are at risk for new-onset esotropia, and preoperative esotropia is likely to increase after OD.

Hematoma subperiosteal orbitário secundário a barotrauma em aviação civil / Orbital subperiosteal hematoma secondary to barotrauma in civil aviation

RESUMO Os diagnósticos diferenciais que compõem as proptoses agudas são, muitas vezes, desafiadores. A anamnese e o exame clínico exigem do oftalmologista atenção especial aos detalhes que permitem diferenciar quadros relativamente benignos e autolimitados de quadros que evoluirão com incapacidades permanentes. Relatamos o caso de uma paciente de 49 anos que, durante viagem de avião, apresentou dor ocular, hematoma periorbitário e proptose do olho esquerdo súbitos. Referia diplopia aguda incapacitante. Exames de tomografia e angiorressonância magnética confirmaram diagnóstico de sinusopatia do seio etmoidal esquerdo e hematoma subperiosteal da órbita esquerda, associado ao barotrauma. Apesar de raro, o diagnóstico de hematoma subperiosteal não traumático deve ser considerado diferencial em relação a proptoses agudas, sendo a anamnese fundamental para essa elucidação diagnóstica.

ABSTRACT Differential diagnoses of acute proptosis are often challenging. History and clinical examination require from ophthalmologists special attention to details, which make it possible to differentiate relatively benign and self-limited conditions from those that will progress to permanent disabilities. We report a 49-year-old female patient who had sudden eye pain, periorbital hematoma and proptosis of the left eye during a commercial flight. She also complained of disabling acute diplopia. Computed tomography and magnetic resonance angiography imaging confirmed the diagnosis of subperiosteal hematoma of the left orbit, associated with left ethmoid sinus disease. Although rare, non-traumatic subperiosteal hematoma should be considered in differential diagnoses of acute proptosis, and history taking is fundamental to elucidate the picture.

Meningioma primario intraóseo esfenotemporal: presentación de caso / Primary spheno-temporal intra-osseous meningioma: a case report

RESUMEN Fundamento: los meningiomas ectópicos, definidos como aquellos que no tienen ninguna conexión con la duramadre, poco frecuentes. Son una variante rara y representan cerca del uno por ciento de todos los meningiomas intracraneales. Objetivo: presentar una variante poco frecuente de un meningioma ectópico como causa de proptosis y oftalmoparesia encontrado en una paciente joven. Presentación del caso: paciente de 40 años de edad, que hace seis meses comenzó con dolor en el ojo derecho de moderada intensidad, aumento de volumen de la región frontorbitaria, disminución de la agudeza visual y visión doble. Los estudios de tomografía axial computarizada y resonancia magnética de cráneo y órbita mostraron lesión extraaxial a nivel de la pared lateral de la órbita con extensión extra e intraorbitaria con compresión de estructuras adyacentes que provocó desplazamiento anterior del globo ocular. Se realizó tratamiento quirúrgico con excéresis y el estudio histológico concluyó un meningioma meningotelial ectópico del hueso grado I. Conclusiones: los meningiomas ectópicos resultan poco frecuentes, el tratamiento quirúrgico con la resección total de la lesión es la elección para evitar recurrencias y pueden tener indicación de tratamiento oncológico complementario.

ABSTRACT Background: ectopic meningioma, defined as those that have no connection with the dura mater, are rare. They are a rare variant and represent approximately 1 % of all intracranial meningioma. Objective: to present a rare variant of an ectopic meningioma as a cause of proptosis and ophthalmoparesis found in a young patient. Case report: patient of 40 years of age, who 6 months ago began with pain in the right eye of moderate intensity, increased volume of the front-orbital region, decreased visual acuity and double vision. Computed tomography and MRI of the skull and orbit showed extra-axial lesion at the level of the lateral wall of the orbit with extra and intra-orbital extension with compression of adjacent structures that caused anterior displacement of the eyeball. Surgical treatment was performed with resection and the histological study concluded an ectopic meningotial meningioma of bone grade I. Conclusions: ectopic meningioma are infrequent, surgical treatment with total resection of the lesion is the choice to avoid recurrences and may have an indication of complementary oncological treatment.

Histiocitosis de células de Langerhans en su presentación focalizada en órbita. Revisión del tema a propósito de un caso / Langerhans cell histiocytosis in their orbitfocused presentation. Review of the topic regarding a case

Resumen Objetivo: Describir un caso de histocitosis de células de Langerhans en un paciente pediátrico. Método: Paciente de 4 años con proptosis, diplopía, fiebre y pérdida de agudeza visual, con imagen en tomografía computarizada evidente de masa orbitaria con erosión ósea. Se realizó biopsia excisional por craneotomía coronal, el resultado histopatológico fue histiocitosis de células de Langerhans. Resultados: El estudio anatomopatológico transoperatorio mostró una neoplasia de células pequeñas redondas y azules, compatible con rabdomiosarcoma. Las pruebas de inmunohistoquímica concluyeron el diagnóstico de histiocitosis de células de Langerhans. Conclusión: La histiocitosis de células de Langerhans es una enfermedad poco frecuente que requiere de una sospecha clínica y diagnóstico oportuno, adecuar el tratamiento y mejorar la sobrevida de los pacientes.

Abstract Objective: To describe a case of Langerhans cell histiocytosis in a pediatric patient. Method: A 4-year-old patient with proptosis, diplopia, fever and loss of visual acuity, with evident image in a computed tomography of orbital mass with bone erosion. An excisional biopsy was performed by coronal craniotomy. The histopathological result was Langerhans cell histiocytosis. Results: The anatomopathological study showed a neoplasm of small round and blue cells, compatible with Rhabdomyosarcoma. Immunohistochemical tests concluded the diagnosis of Langerhans cell histiocytosis. Conclusion: Langerhans cell histiocytosis is a rare disease that requires clinical suspicion and a timely diagnosis to adjust the treatment and improve the survival of patients.

A case of orbital extra-adrenal paraganglioma in a quarter horse

Paragangliomas are tumors originating from paraganglia cells which represent a considerable constituent of the dispersed neuroendocrine system. Rarely these tumors are found within the orbital region. This report documents a case of primary extra-adrenal paraganglioma in a 15-year-old female quarter horse, diagnosed on the basis of clinical signs, gross anatomopathological, histopathological and immunohistochemical investigations. Clinical diagnostics led to full appreciation of the severe tumor invasiveness and to an informed decision by the owner for euthanasia. Gross inspection confirmed the previous clinical assessments of exophthalmos consequential to pressure from the orbital neoplasia, revealing a mass infiltrating into surrounding tissues. Histopathology and immunohistochemistry allowed for the final diagnosis of paraganglioma. Histology sections showed a partially encapsulated infiltrative mass with multifocal angiotropic growth, and composed of polygonal cells organized in nests, packets, and bundles supported by a fibrovascular stroma. The lobules were lined peripherally by spindle cells. The tumor was highly vascular with blood-filled lacunae and multifocal to coalescing areas of liquefactive necrosis. The polygonal cells were characterized by indistinct borders, lightly eosinophilic cytoplasm with moderate amount of granules, round or oval central nuclei with stippled chromatin (“salt and pepper” appearance) and inconspicuous nucleoli. Immunohistochemistry aided in the diagnostic discrimination of paraganglioma from neuroendocrine carcinoma and in the assessment of tumor differentiation. Orbital paraganglioma holds low likelihood for animal survival, but may have good prognosis when timely addressed.(AU)

A case of orbital extra-adrenal paraganglioma in a quarter horse

Paragangliomas are tumors originating from paraganglia cells which represent a considerable constituent of the dispersed neuroendocrine system. Rarely these tumors are found within the orbital region. This report documents a case of primary extra-adrenal paraganglioma in a 15-year-old female quarter horse, diagnosed on the basis of clinical signs, gross anatomopathological, histopathological and immunohistochemical investigations. Clinical diagnostics led to full appreciation of the severe tumor invasiveness and to an informed decision by the owner for euthanasia. Gross inspection confirmed the previous clinical assessments of exophthalmos consequential to pressure from the orbital neoplasia, revealing a mass infiltrating into surrounding tissues. Histopathology and immunohistochemistry allowed for the final diagnosis of paraganglioma. Histology sections showed a partially encapsulated infiltrative mass with multifocal angiotropic growth, and composed of polygonal cells organized in nests, packets, and bundles supported by a fibrovascular stroma. The lobules were lined peripherally by spindle cells. The tumor was highly vascular with blood-filled lacunae and multifocal to coalescing areas of liquefactive necrosis. The polygonal cells were characterized by indistinct borders, lightly eosinophilic cytoplasm with moderate amount of granules, round or oval central nuclei with stippled chromatin (“salt and pepper” appearance) and inconspicuous nucleoli. Immunohistochemistry aided in the diagnostic discrimination of paraganglioma from neuroendocrine carcinoma and in the assessment of tumor differentiation. Orbital paraganglioma holds low likelihood for animal survival, but may have good prognosis when timely addressed.

Paquimeningitis y glomerulonefritis crescéntica en vasculitis p-ANCA / Pachymeningitis and crescentic glomerulonephritis in p-ANCA vasculitis

Resumen Las vasculitis asociadas a anticuerpos contra el citoplasma del neutrófilo configuran patologías multisistémicas caracterizadas por afectar pequeños vasos. Se ha descrito compromiso de sistema nervioso central en 2-8% de los casos, rara vez al inicio del cuadro clínico. Este corresponde al caso de un hombre de 67 años de edad con vasculitis p-ANCA, quien debutó con proptosis, paquimeningitis y disfagia severa. Posteriormente deterioro renal, necesidad de hemodiálisis, ciclofosfamida y recambio plasmático terapéutico con lo cual alcanza criterios de remisión parcial y egresa de diálisis. Las vasculitis ANCA se relacionan con una amplia gama de signos y síntomas inespecíficos. La edad de aparición, creatinina inicial y requerimiento de diálisis, son factores pronósticos. El inicio oportuno del tratamiento es fundamental, requiriendo por tanto un alto índice de sospecha. (Acta Med Colomb 2018; 43: 107-110).

Abstract Vasculitis associated with antibodies against the neutrophil cytoplasm they form multisystemic pathologies characterized by affecting small vessels. Central nervous system involvement has been described in 2 - 8% of cases, rarely at the beginning of the clinical picture. This corresponds to the case of a 67-year-old man with p-ANCA vasculitis, who debuted with proptosis, pachymeningitis and severe dysphagia. Subsequently, renal deterioration, the need for hemodialysis, cyclophosphamide and therapeutic plasma exchange, with which it reaches criteria of partial remission and dialysis discharge. ANCA vasculitis is associated with a wide range of nonspecific signs and symptoms. The age of onset, initial creatinine and dialysis requirement are prognostic factors. The timely initiation of treatment is fundamental, requiring therefore a high index of suspicion. (Acta Med Colomb 2018; 43: 107-110).

Presentación atípica de un prolactinoma gigante en un adolescente de 15 años / Atypical presentation of a giant prolactinoma in a 15-year-old boy

El prolactinoma gigante es un adenoma pituitario poco frecuente caracterizado por su gran invasión local. Se reporta el caso de un varón de 15 años de edad con dolor retroocular izquierdo y exoftalmos ipsilateral de 4 meses de evolución secundario a un tumor en la base del cráneo que invadía la órbita. Los estudios hormonales revelaron prolactina sérica de 6913,7 ng/ml (valor normal < 20), que confirmó el diagnóstico de prolactinoma gigante. El paciente inició un tratamiento con el agonista dopaminérgico cabergolina en dosis crecientes. Luego de 7 meses de seguimiento, la prolactina había descendido a 349,8 ng/ml y el volumen del tumor se redujo un 70%, sin efectos adversos al tratamiento. El paciente se encontraba asintomático y había reiniciado la pubertad. La rápida remisión de los síntomas sin necesidad de tratamientos invasores subraya la importancia de considerar el diagnóstico de prolactinoma entre los posibles diagnósticos diferenciales de tumor de la base del cráneo.

Giant prolactinomas are rare pituitary adenomas characterized by their great local invasion. In this paper, we report a 15-year-old male with left retro-ocular pain and ipsilateral exophthalmos of 4 months of evolution, secondary to a tumour in the base of the skull that invaded the orbit. Hormonal studies revealed serum prolactin of 6913,7 ng/ml (normal value < 20), confirming the diagnosis of giant prolactinoma. The patient started treatment with the dopaminergic agonist cabergoline in increasing doses. After 7 months of follow-up the prolactin had decreased to 349.8 ng/ml and the tumor volume was reduced by 70%, without presenting adverse effects to the treatment. The patient was asymptomatic and had restarted puberty. The rapid remission of symptoms without the need for invasive treatments underlines the importance of considering the diagnosis of prolactinoma among the possible differential diagnoses of tumor of the skull base.

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.

[Atypical presentation of a giant prolactinoma in a 15-year-old boy]. / Presentación atípica de un prolactinoma gigante en un adolescente de 15 años.

Giant prolactinomas are rare pituitary adenomas characterized by their great local invasion. In this paper, we report a 15-year-old male with left retro-ocular pain and ipsilateral exophthalmos of 4 months of evolution, secondary to a tumour in the base of the skull that invaded the orbit. Hormonal studies revealed serum prolactin of 6913,7 ng/ml (normal value < 20), confirming the diagnosis of giant prolactinoma. The patient started treatment with the dopaminergic agonist cabergoline in increasing doses. After 7 months of follow-up the prolactin had decreased to 349.8 ng/ml and the tumor volume was reduced by 70%, without presenting adverse effects to the treatment. The patient was asymptomatic and had restarted puberty. The rapid remission of symptoms without the need for invasive treatments underlines the importance of considering the diagnosis of prolactinoma among the possible differential diagnoses of tumor of the skull base.

El prolactinoma gigante es un adenoma pituitario poco frecuente caracterizado por su gran invasión local. Se reporta el caso de un varón de 15 años de edad con dolor retroocular izquierdo y exoftalmos ipsilateral de 4 meses de evolución secundario a un tumor en la base del cráneo que invadía la órbita. Los estudios hormonales revelaron prolactina sérica de 6913,7 ng/ml (valor normal < 20), que confirmó el diagnóstico de prolactinoma gigante. El paciente inició un tratamiento con el agonista dopaminérgico cabergolina en dosis crecientes. Luego de 7 meses de seguimiento, la prolactina había descendido a 349,8 ng/ml y el volumen del tumor se redujo un 70%, sin efectos adversos al tratamiento. El paciente se encontraba asintomático y había reiniciado la pubertad. La rápida remisión de los síntomas sin necesidad de tratamientos invasores subraya la importancia de considerar el diagnóstico de prolactinoma entre los posibles diagnósticos diferenciales de tumor de la base del cráneo.

Utility of rituximab treatment for exophthalmos, myxedema, and osteoarthropathy syndrome resistant to corticosteroids due to Graves' disease: a case report.

BACKGROUND: Exophthalmos, myxedema, and osteoarthropathy syndrome is a very rare condition that is associated with Graves' disease. The presence of dermopathy and the involvement of joint/bone tissues indicate that it seems to be related with the severity of the autoimmune process. Owing to its low incidence, there is a lack of information regarding its treatment and clinical follow-up. Some cases improved after use of high doses of steroids; however, some patients do not respond to this treatment. Recently, the effectiveness of rituximab for treatment of Graves' ophthalmopathy resistant to corticosteroids has been demonstrated. However, it has never been used for the treatment of exophthalmos, myxedema, and osteoarthropathy syndrome (particularly for the treatment of osteoarticular manifestations). CASE PRESENTATION: We present the case of a 54-year-old Mexican woman previously treated for Graves' disease who developed post-iodine hypothyroidism and exophthalmos, myxedema, and osteoarthropathy that did not improve after high doses of steroids (intravenous and oral). Her exophthalmos, myxedema, and osteoarthropathy syndrome symptoms improved as early as 6 months after treatment with rituximab. CONCLUSION: Exophthalmos, myxedema, and osteoarthropathy syndrome is a non-classical presentation of Graves' disease, whose clinical manifestations could improve after treatment with rituximab, particularly in those patients with lack of response to high doses of corticosteroids.