Intravascular papillary endothelial hyperplasia (Masson's tumor) of the conjunctiva. Unusual location of a rare vascular tumor.

Intravascular papillary endothelial hyperplasia (IPEH) is characterized by proliferation of endothelial cells usually occurring within a long-standing vascular malformation or tumor. IPEH usually affects neck, head and lower extremities, and few orbital and eyelid cases have been reported. We present a unique case, a 48-years-old man with a purple, elevated, multilobular conjunctival lesion of three-weeks duration that underwent an excisional biopsy. Pathological features revealed intravascular papillary endothelial hyperplasia (Masson's tumor).

Rapidly progressive orbital intravascular papillary endothelial hyperplasia in a newborn.

Intravascular papillary endothelial hyperplasia (IPEH), also known as "vegetant intravascular haemangioendothelioma", is a rare benign proliferation of vascular endothelial cells secondary to intravascular thrombosis and thrombus organisation. It can develop from vascular lesions such as haemangiomas, pyogenic granulomas or varicose veins. This vascular tumour of the skin and subcutaneous tissue may exhibit rapid and progressive growth, and is usually located in the neck or head. Ocular presentation is unusual and orbital involvement is even rarer. We report a case of a preterm newborn male with a rapidly growing left orbital mass that was histologically diagnosed as intravascular papillary endothelial hyperplasia. He was successfully managed with total excision of the tumour and propranolol therapy and remains recurrence free after an eight-year follow-up.

Hiperplasia endotelial papilar intravascular (tumor de Masson) de la conjuntiva. Ubicación inusual de un tumor vascular endotelial raro / Intravascular papillary endothelial hyperplasia (Masson's tumor) of the conjunctiva. Unusual location of a rare vascular tumor

La hiperplasia endotelial papilar intravascular (IPEH) se caracteriza por la proliferación de células endoteliales generalmente producida dentro de una malformación vascular previa o un tumor. La IPEH suele afectar cuello, cabeza y extremidades inferiores, se han publicado pocos casos en la órbita ocular y párpados. Presentamos un caso único, un hombre de 48 años con una lesión conjuntival púrpura, elevada y multilobular de tres semanas de duración que se sometió a una biopsia excisional. Las características patológicas revelaron una hiperplasia endotelial papilar intravascular (tumor de Masson) (AU)

Intravascular papillary endothelial hyperplasia (IPEH) is characterized by proliferation of endothelial cells usually occurring within a long-standing vascular malformation or tumor. IPEH usually affects neck, head and lower extremities, and few orbital and eyelid cases have been reported. We present a unique case, a 48-year-old man with a purple, elevated, multilobular conjunctival lesion of three-weeks duration that underwent an excisional biopsy. Pathological features revealed intravascular papillary endothelial hyperplasia (Masson's tumor) (AU)

Hiperplasia endotelial papilar intravascular orbitaria rápidamente progresiva en un recién nacido / Rapidly progressive orbital intravascular papillary endothelial hyperplasia in a newborn

La hiperplasia endotelial papilar intravascular (IPEH), también conocida como «hemangioendotelioma vegetante intravascular», es una proliferación benigna y rara de células endoteliales vasculares, secundaria a trombosis intravascular u organización del trombo. Puede desarrollarse a partir de lesiones vasculares como hemangiomas, granulomas piógenos o várices. Este tumor vascular de la piel y del tejido subcutáneo puede presentarse como crecimiento rápido y progresivo, y es usualmente localizado en cuello o cabeza. La presentación ocular es inusual y la afectación orbitaria es todavía más rara. Presentamos un caso de un varón recién nacido pretérmino, el cual presentó una masa orbitaria izquierda de crecimiento rápidamente progresivo y se obtuvo el diagnóstico por histología de hiperplasia endotelial papilar intravascular. El paciente fue exitosamente tratado mediante una escisión total del tumor y terapia con propranolol. Actualmente, después de un seguimiento de ocho años, ha permanecido sin recurrencias (AU)

Intravascular papillary endothelial hyperplasia (IPEH), also known as “vegetant intravascular haemangioendothelioma”, is a rare benign proliferation of vascular endothelial cells secondary to intravascular thrombosis and thrombus organisation. It can develop from vascular lesions such as haemangiomas, pyogenic granulomas or varicose veins. This vascular tumour of the skin and subcutaneous tissue may exhibit rapid and progressive growth, and is usually located in the neck or head. Ocular presentation is unusual and orbital involvement is even rarer. We report a case of a preterm newborn male with a rapidly growing left orbital mass that was histologically diagnosed as intravascular papillary endothelial hyperplasia. He was successfully managed with total excision of the tumour and propranolol therapy and remains recurrence free after an eight-year follow-up (AU)

Hiperplasia endotelial papilar intravascular (tumor Masson) de localización ginecológica / Intravascular papillary endothelial hyperplasia (Masson's tumour) of gynaecological origin

La hiperplasia papilar endotelial intravascular o tumor de Masson es una lesión vascular no neoplásica de carácter reactivo. Se caracteriza por ser un tumor poco frecuente, clínicamente inespecífico y con localizaciones descritas muy diversas. Resulta fundamental tenerlo en consideración dentro del diagnóstico diferencial de los tumores vasculares malignos como el angiosarcoma, cuyo pronóstico es muy diferente. Para lograr su diagnóstico definitivo el estudio anatomopatológico resulta primordial. El tratamiento de elección es la exéresis quirúrgica asegurando márgenes libres de enfermedad. El presente caso reportado supone un hecho excepcional, al ser un tumor de Masson de localización pélvica, cuyo diagnóstico fue un hallazgo casual propiciado por el estudio de estadificación quirúrgica de un adenocarcinoma de ovario.

Intravascular papillary endothelial hyperplasia or Masson's tumour is a non-neoplastic vascular lesion of reactive character. It is a rare diagnosis, clinically non-specific and with diverse locations. It is essential to take it into consideration and make a differential diagnosis with malignant vascular tumours such as angiosarcoma. Pathological study is fundamental for diagnosis. Treatment consists of complete resection of the tumour, including sufficiently wide margins to avoid recurrence.The case reported is an exceptional event, because of the pelvic location of the Masson's tumour that was diagnosed as part of the surgical staging of an ovarian cancer.

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.