Epitheloid hemangioendothelioma of the urinary bladder: A case report of a rare entity

Epithelioid hemangioendothelioma (EHE) is a rare, often multifocal, intermediate-grade vascular sarcoma arising from the endothelial cells. Their behavior is intermediate between hemangioma and conventional angiosarcoma. Their rarity and unpredictable clinical behavior result in our still limited understanding and treatment options for this variety of tumors. Molecular diagnostic tools like the presence of WWTR1-CAMTA1 fusion have been developed to aid this challenging diagnosis. There is no definitive consensus on the management of patients with this disease and no commonly accepted treatment strategies have been found to be effective, particularly in the metastatic setting. When possible, surgery with clear margins should be considered in case of localized disease. Here, we not only present such a rare case of EHE of the urinary bladder but also provide a comprehensive discussion on the present treatment modalities offered, as found through a thorough search of the published studies. Finally, more studies are required to establish a standard of care for this rare entity

Hemangioendotelioma epitelioide de pulmón / Pulmonary epitheloid hemangioendothelioma

El hemangioendotelioma epitelioide es un tumor vascular raro, de bajo grado de malignidad, con origen en las células endoteliales, que se puede desarrollar en cualquier tejido. En el pulmón surge habitualmente como secundario, pero también puede aparecer como primario, lo que es extremadamente inusual. Normalmente aparece como múltiples nódulos bilaterales entre jóvenes del sexo femenino. Raramente se desenvuelve como un nódulo pulmonar solitario. Los nódulos únicos y unilaterales pueden ser removidos quirúrgicamente. Se relata el caso de una paciente de 11 años que ingresó con impresión diagnóstica de neumonía lobular, pero que con una evolución desfavorable, necesitó realizar biopsia de la lesión, con el resultado de hemangioendotelioma epitelioide. Se discute también el diagnóstico y el tratamiento de esta entidad patológica.

Epitheloid hemangioendothelioma is a rare vascular tumor of low level of malignancy that emerges in the endothelial cells and may develop in any other tissue. It is generally secondary tumor in the lung but it may also appear as primary one, being very unusual. Generally speaking, it appears in the form of multiple bilateral nodules in young women. It rarely develops as a single pulmonary nodule. The single unilateral nodules may be surgically removed. This is the case of a 11-years old girl who was hospitalized with a diagnostic suspicion of lobular pneumonia, but her unfavorable progression required biopsy of the lesion with resulting epitheloid hemangioendothelioma. The diagnosis and the treatment for this pathological entity were also discussed.

Epitheloid hemangioendothelioma of the submandibular region

@#<p style="text-align: justify;"><strong>OBJECTIVES:</strong> To  present  an  uncommon  cause  for  a  submandibular  mass  and  review  of  the literature.<br /><strong>METHODS:</strong><br /><strong>Design:</strong> Case Report<br /><strong>Setting:</strong> Tertiary Government Hospital<br /><strong>Patient:</strong> One<br /><strong>RESULTS:</strong> A  25-year-old  lady  presented  with  a  painless  chronic  submandibular  swelling. Ultrasound identified a solid mass following which an uncomplicated core biopsy was performed obtaining    an    accurate    pre-operative    histopathological    diagnosis.    Pre-operative    arterial embolization of this vascular mass led to a relatively bloodless wide local excision. Radiological imaging for distant metastases was negative.<br /><strong>CONCLUSION:</strong> Epitheloid  Hemangioendothelioma  is  an  uncommon  cause  for  a  submandibular mass.   A malignant   vascular   soft   tissue   tumor   with   morphologic   characteristics   similar   to carcinomas, melanomas and epitheloid sarcomas, it has a high rate of metastasis and morbidity when it affects the soft tissues and viscera. Immunohistochemistry provides clues to differentiation and recommended treatment consists of a surgical wide local excision with regional lymph node resection. As there are no established standard therapeutic protocols for this disease due to its rarity, an individual case-by-case approach and follow-up needs to be undertaken.</p>