Intravascular papillary endotelial hiperplasia, the unusual diagnosis of an orbital mass.

Intravascular papillary endotelial hyperplasia is a bening vascular lesion that rarely involves the periocular region, nevertheless, it should be considered in the differential diagnosis of a periorbital mass. It is histopatologically characterized by intravascular fibrous papillary fronds covered by endothelial cells. Histopathological differentiation from angiosarcoma can be challenging but is very important to avoid aggressive treatments. Complete surgical excision is frequently curative. Uncompleted excision can lead to recurrences. We describe the clinical and histological findings of intravascular papillary endotelial hyperplasia in the left lower lid of a 72-year-old woman who had a painless mass since 15 years ago, that caused hypertropia of her left eye. Histopathological examination revealed intralesional calcification, which is not an usual feature in this entity. The patient underwent complete surgical resection of the mass and there was no evidence of recurrence after 5 months of follow-up after surgery.

Hiperplasia endotelial papilar intravascular, el inusual diagnóstico de una masa orbitaria / Intravascular papillary endotelial hiperplasia, the unusual diagnosis of an orbital mass

La hiperplasia endotelial papilar intravascular es una lesión vascular benigna, infrecuente en la región periocular, pero que debe ser tenida en cuenta en el diagnóstico diferencial de una masa periorbitaria.Histopatológicamente se caracteriza por la proliferación intravascular de proyecciones papilares de tejido conectivo recubiertas por células endoteliales. La diferenciación anatomopatológica con el angiosarcoma puede ser difícil, pero es muy importante para evitar tratamientos agresivos. Generalmente, la escisión quirúrgica completa es curativa, mientras que una escisión incompleta puede causar recurrencias.Describimos un caso clínico de hiperplasia endotelial papilar intravascular en una mujer de 72 años que presentaba una masa palpebral inferior izquierda de 15 años de evolución que le provocaba hiperglobo del ojo izquierdo. El estudio histopatológico mostró la presencia de calcificación intralesional, característica infrecuente en esta patología.La masa fue extirpada completamente y tras 5 meses de seguimiento no mostró signos de recurrencia.

Intravascular papillary endotelial hyperplasia is a bening vascular lesion that rarely involves the periocular region, nevertheless, it should be considered in the differential diagnosis of a periorbital mass.It is histopatologically characterized by intravascular fibrous papillary fronds covered by endothelial cells. Histopathological differentiation from angiosarcoma can be challenging but is very important to avoid aggressive treatments. Complete surgical excision is frequently curative. Uncompleted excision can lead to recurrences.We describe the clinical and histological findings of intravascular papillary endotelial hyperplasia in the left lower lid of a 72-year-old woman who had a painless mass since 15 years ago, that caused hypertropia of her left eye. Histopathological examination revealed intralesional calcification, which is not an usual feature in this entity.The patient underwent complete surgical resection of the mass and there was no evidence of recurrence after 5 months of follow-up after surgery.

Intravascular papillary endotelial hiperplasia, the unusual diagnosis of an orbital mass. / Hiperplasia endotelial papilar intravascular, el inusual diagnóstico de una masa orbitaria.

Intravascular papillary endotelial hyperplasia is a bening vascular lesion that rarely involves the periocular region, nevertheless, it should be considered in the differential diagnosis of a periorbital mass. It is histopatologically characterized by intravascular fibrous papillary fronds covered by endothelial cells. Histopathological differentiation from angiosarcoma can be challenging but is very important to avoid aggressive treatments. Complete surgical excision is frequently curative. Uncompleted excision can lead to recurrences. We describe the clinical and histological findings of intravascular papillary endotelial hyperplasia in the left lower lid of a 72-year-old woman who had a painless mass since 15 years ago, that caused hypertropia of her left eye. Histopathological examination revealed intralesional calcification, which is not an usual feature in this entity. The patient underwent complete surgical resection of the mass and there was no evidence of recurrence after 5 months of follow-up after surgery.

[Creutzfeldt - Jakob presenting with isolated visual symptoms: the Heidenhain variant]. / Creutzfeldt - Jakob con debut oftalmológico: la variante Heidenhain.

CLINICAL CASE: We report the case of a 67 year old female complaining of decreased vision and diagnosed with the Heidenhain variant of sporadic Creutzfeldt-Jakob disease. Her past medical history was unremarkable. She died less than three months after the onset. DISCUSSION: The Heidenhain variant of sporadic Creutfeld-Jakob disease should be suspected in patients suffering from early visual disturbances, unremarkable ophthalmic examination, and subsequent rapid decline of their cognitive function. A complete neurological exam including electroencephalogram recordings and magnetic resonance is mandatory. These patients share a common genotype (PRNP codon 129 MM) associated with a clinically typical disease course.

Creutzfeldt - Jakob con debut oftalmológico: la variante Heidenhain / Creutzfeldt - Jakob presenting with isolated visual symptoms: the Heidenhain variant

Caso clínicoPresentamos el caso de una mujer de 67 años con disminución de agudeza visual bilateral que fue diagnosticada de enfermedad de Creutzfeldt-Jakob esporádico en su variante Heidenhain. No presentaba antecedentes de interés. La paciente falleció tres meses después del inicio del cuadro.DiscusiónLa variante Heidenhain de la enfermedad de Creutzfeldt-Jakob esporádico debe sospecharse en todo paciente aquejado de alteraciones visuales y examen oftalmológico inespecífíco, que experimente deterioro cognitivo posterior. Es necesario un examen neurológico completo que incluya electroencefalograma y resonancia magnética. Estos pacientes comparten un mismo genotipo (codón 129 MM en gen PRNP) asociado a un curso clínico característico(AU)

Clinical caseWe report the case of a 67 year old female complaining of decreased vision and diagnosed with the Heidenhain variant of sporadic Creutzfeldt-Jakob disease. Her past medical history was unremarkable. She died less than three months after the onset.DiscussionThe Heidenhain variant of sporadic Creutfeld-Jakob disease should be suspected in patients suffering from early visual disturbances, unremarkable ophthalmic examination, and subsequent rapid decline of their cognitive function. A complete neurological exam including electroencephalogram recordings and magnetic resonance is mandatory. These patients share a common genotype (PRNP codon 129 MM) associated with a clinically typical disease course(AU)