Anteroposterior Combined Surgery of a Rare Massive Epithelioid Hemangioendothelioma at the Cervicothoracic Junction.

Epithelioid hemangioendothelioma is a rare mesenchymal tumor of vascular endothelial origin. Non-soft tissue epithelioid hemangioendothelioma can also be seen in different organs. Although chemotherapy has been used in some patients, complete surgical removal of the tumor tissue has proven to be the most durable solution. A 15-year-old female patient was admitted to our institution with right arm and neck pain. The patient complained of numbness and weakness in the right hand. Computerized tomography indicated an expansile lesion exhibiting osteolytic features located predominantly on the right side of the corpus, pedicle, lamina, and lateral processes of the C7-T1 vertebra. The patient underwent a surgical procedure involving the application of a bilateral C4-5-6 lateral mass screw, left C7-T1 pedicle screw, and bilateral T2-3 pedicle screw and fusion. The complete residual neoplasm was surgically removed during the procedure. Due to the rarity of epithelioid hemangioendothelioma, the existing literature on this topic is confined to case reports, supplemented by a small number of retrospective descriptive case series that aimed to improve our understanding of the clinical, pathological, and molecular features of the condition, as well as to guide potential treatment strategies.

Report of a Rare Case of Epitheliod Hemangioendothelioma in Tongue.

Epitheloid hemangioendothelioma (EHE) is a rare vascular neoplasm of intermediate malignant potential. EHE commonly involves lungs, liver soft tissue, and bone. EHE is extremely rare in tongue and up to our best knowledge only nine cases of EHE of tongue reported in the literature. Clinicallyو EHE usually presents as an asymptomatic mass. Microscopically, EHE exhibits proliferation of epitheloid cells and spindle shaped endothelial cells. Epitheloid cells show cytoplasmic vacuoles with few cells containing RBCs. A 34-year-old male presented to our institution with the chief complain of swelling on the base of the tongue from eight months. Surgical excision was done. An extensive work up of immunohistochemistry was done using different markers including CD 31, CD 34, Ki 67, Factor VIII, and BCL2. Correlation of histopathology and immunohistochemistry confirmed the diagnosis of EHE. The follow up period of 2 years was uneventful.

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.

Effective Treatment of Pleural Epitheloid Hemangio-endothelioma with Pazopanib: A Case Report.

A patient with a pleural epitheloid hemangio-endothelioma (EHE) who failed to respond to six cycles of initial chemotherapy with iphosphamide and epirubicine was treated with pazopanib in second-line. A significant subjective and objective metabolic response on (18)F-fluoro-deoxyglucose positron-emission tomography-computed tomography was noted. Based on this observation, the role of vasculoendothelial growth factor receptor inhibitors such as pazopanib (or other tyrosine kinase inhibitors), in the treatment of pleural EHE should be established through prospective collaborative studies as upfront medication and in combination with chemotherapy.

An unusual presentation of pulmonary epitheloid hemangioendothelioma.

Pulmonary epitheloid hemangioendothelioma (PEHE) is a rare, often incidentally diagnosed, endothelial tumor of the lung. We present a case of a young adult who presented with acute hypoxemic respiratory failure and severe pulmonary hypertension with subsequent imaging and tissue biopsy confirming a diagnosis of PEHE. We briefly highlight the unique clinical, radiographic and histopathologic aspects of this rare disease. We propose that PEHE should be considered in the differential diagnosis for acute hypoxemic respiratory failure associated with bilateral pulmonary cavitary nodules and bronchoscopy should be considered as an initial diagnostic test.

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>