Diagnosis Of Epithelioid Hemangioendothelioma Research Articles

TFE3 rearranged epithelioid hemangioendothelioma of the lymph node: A rare primary presentation of vascular tumor.

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor that has been described in numerous sites such as soft tissue, liver, and lungs. However, primary lymph nodal presentation of hemangioendothelioma is extremely rare. We present a 49-year-old male patient who presented with an inguinal mass but was otherwise asymptomatic. Clinical and radiological workup failed to reveal any other primary lesion. The lymph node was excised and the histomorphological examination showed an epithelioid and spindle neoplasm with chondromyxoid stroma. The diagnosis of epithelioid hemangioendothelioma was suspected based on histomorphology and immunohistochemistry, which showed diffuse positivity for CD31, CD34, and TFE3 stains. This was further confirmed by the fluorescent in-situ hybridization (FISH) technique that showed TFE3 gene rearrangement.

Epithelioid haemangioendothelioma: case report and brief revision of a rare vascular tumour

We describe the case of a 71-year-old man who presented with a single painful soft tissue mass of the right thigh. Following imaging and subsequent excision, histological and immunohistochemical assessment of the tumour was performed, and the diagnosis of epithelioid haemangioendothelioma (EHE) was made. EHE is a rare translocation-related vascular sarcoma with variable clinical behaviour ranging from a low grade malignancy with ability to recur locally, to that of a high grade sarcoma, with propensity for systemic involvement.

Rar liver tumour — epithelioid haemangioendothelioma: a case report

Abstract Epithelioid haemangioendothelioma (EHE) is a rare vascular soft tissue malignant tumour with unknown etiology; the estimated prevalence of EHE is less than one in 1 million. A 56-year-old man was admitted in our department due to pain in the right side of the abdomen lasting for two years and weight loss up to 10 kg. Since 2012, the patient underwent lung and abdominal CT scanning as well as biopsy, however the diagnosis was challenging. In 2015, repeated abdominal CT scanning and a liver core biopsy was performed. The epithelioid haemangioendothelioma was diagnosed based by histopathological examination with subsequent radiological and clinical correlation. Therefore, accurate histopathological examination with radiological and clinical correlation is essential in the diagnosis of epithelioid haemangioendothelioma.

Multifocal cutaneous epithelioid hemangiodothelioma on the same lower limb

<p class="abstract">Epithelioid hemangioendothelioma (EHE) is a rare low-grade vascular tumour mainly affecting bones, deep soft tissues and exceptionally the skin. Multifocal cutaneous involvement is uncommon and should raise suspicion for metastasizing extracutaneous epithelioid hemangiodothelioma. Hereby we report the case of a young patient with multifocal cutaneous EHE involving the same lower limb. We emphasize the necessity for dermatologists to evoke the diagnosis of EHE in patients presenting with nonspecific tumours of the extremities. Prompt management and diagnosis are essential in this potential aggressive tumour.</p>

Diagnosis of epithelioid hemangioendothelioma of the liver. Lecture and analysis of own cases

Epithelioid hemangioendothelioma of the liver (EHEL) is a rare (1 case per 1 000,000 people) primary malignant neoplasm from the group of mesenchymal tumors. EHEL is the most aggressive representative of the hemangioendothelioma range with metastatic response and relapse rate, and therein is close to angiosarcoma.The purpose of this publication is to summarize our own experience in comparison with published data and to describe a disease symptom that was not investigated before.Materials and methods. We studied five cases of epithelioid hemangioendothelioma of the liver, confirmed by histological examination. All patients underwent standard computer tomography with bolus contrast agent.Results. The analysis of the images revealed typical for this pathology symptoms such as local retraction of the liver contour, a specific contrast pattern – “the lollipop sign”. We have revealed a new symptom: “the beading sign”, which was detected in four of five cases. In three of five cases, this symptom allowed us to determine the nature of the disease before histological examination.Conclusion. EHEL is a difficult disease to diagnose due to its rare frequency and similarity to other focal liver lesions. In aspect of radiology, it is advisable to focus on specific symptoms of this pathology: retraction of the contour, “lollipop-sign” and “beading-sign”.

Pulmonary Epithelioid Haemangioendothelioma Involving Pleura: A Case Report

Abstract Introduction/Objective Pulmonary epithelioid haemangioendothelioma is a rare, low to intermediate grade tumor of endothelial origin. It is more common in middle aged women. In lung, more than 60 % of the cases present as bilateral multiple perivascular nodules on imaging raising the differential of metastasis or inflammatory cause. WWTR1- CAMTA1 fusion gene is characteristic for this entity. Evidence of pleural involvement carries a poor prognosis. Methods Here, we present a case of epithelioid haemangioendothelioma involving pleura in a 52-year-old woman with a long standing history of rheumatoid arthritis. Patient presented with recurrent pleural effusions and shortness of breath. Results A diagnosis of epithelioid haemangioendothelioma was made on her pleural decortication specimen that showed atypical vascular proliferation and confirmed by immunohistochemical staining. Subsequent right lung wedge resection performed later confirmed the presence of epithelioid haemangioendothelioma in lung parenchyma. Conclusion Pathologists should be aware of this rare condition that can clinically present in a very deceptive manner of recurrent effusion.

Modulation of YAP/ TAZ by statins to improve survival in epithelioid hemangioendothelioma (EHE).

e23527 Background: EHE is a rare soft tissue tumor of endothelial origin. It is distinguished by the pathognomonic WWTR1-CAMTA1 fusion (WWTR1 is the gene symbol for TAZ) seen in 90% of the tumors. YAP1-TFE3 fusion is less common and seen in 10% of the tumors. YAP and TAZ are critical downstream effectors of the Hippo pathway that regulate tumor development, progression, invasion and metastasis by modulating the expression of many Hippo pathway targets. Recent studies have shown that inhibition of HMG-CoA reductase, a key enzyme of the mevalonate pathway, can regulate YAP/ TAZ by preventing their nuclear accumulation and inhibiting their transcriptional activity. This has led to interest in the role of statins, which inhibit HMG-CoA reductase, as a modulator of YAP/ TAZ that could benefit patients with sarcoma, particularly EHE. Methods: A retrospective analysis was performed on patients with a diagnosis of EHE at M D Anderson Cancer Center. Patients were identified using the electronic database system and screened for statin use using EMRs. Demographic and clinical characteristics were tabulated. KM method was used to assess overall survival and log rank test was used to test survival differences between the statin use and non- statin use groups. All statistical analysis was performed using STATA 14. Results: 226 patients with EHE were identified. 27 of them had recorded statin use during the course of their disease. The median OS for the statin use group was not reached and the mean OS was 221 months. The median OS for the non- statin use group was 123.9 months, while the mean OS was 160 months. The difference in OS was not statistically significant between the two groups. The median follow-up time for our cohort was 36.6 months. Conclusions: Our findings indicate a trend towards improved survival for patients with EHE who have received statins over the course of their disease. Our study is limited by a small number of patients who received statins. Prospective studies are required to assess the therapeutic benefit of statins in EHE. [Table: see text]

EPITHELIOID HEMANGIOENDOTHELIOMA OCCURRING IN THE PAROTID GLAND: A CASE REPORT AND LITERATURE REVIEW

Epithelioid hemangioendothelioma (EH) is an intermediate grade vascular malignancy. EH often exhibits aggressive biologic behavior, frequently metastasizes to regional lymph nodes and rarely, to distant sites. EH most commonly occurs in deep soft tissue, viscera, and bone. Several cases of EH have been reported in the head and neck region; however, development of EH within the parotid gland is extremely rare. To our knowledge, only four cases of EH in the parotid have been reported in the English literature. We present a case of EH of the left parotid gland in a 45-year-old Caucasian woman. The patient had a history of a painless swelling on the left side of her face for several years and imaging studies indicated a neoplasm originated from the left parotid gland. A percutaneous biopsy demonstrated a concern for sarcoma. Therefore, the patient underwent a left parotidectomy with facial nerve preservation and left neck dissection. Histologic examination revealed a well-circumscribed proliferation of epithelioid tumor cells in a hyalinized stroma. Intracytoplasmic vacuoles were noted in some cells. Lymphovascular invasion was present, and a small metastatic tumor focus was identified in one regional lymph node in the ipsilateral neck. Immunohistochemical studies were performed. CD31 and Fli-1 were diffusely positive in tumor cells, while they were negative for AE1/AE3, S-100, SMA and p63. The Ki-67 proliferative index was estimated at 2%. A diagnosis of EH was established based on histological and immunohistochemical findings. No recurrence of the patient's disease has been noted in the 6 months following her surgery.

Primary pleural epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor exceptionally involving the pleura with less than 30 cases reported in literature. We herein describe another case of pleural EHE in a 79-year-old man with medical history of chronic obstructive pulmonary disease and high blood pressure. He presented rightsided pleural effusion. Computerized tomography revealed multifocal pleural thickening and effusion. Pleural biopsy was performed. Microscopically, the tumor showed a biphasic pattern with cords and nests of epithelioid cells showing mild atypia and rare mitosis with intracytoplasmic lumina containing red blood cells. The second pattern is composed of spindle-shaped cells with occasional necrosis. The tumor cells were positive for CD34 and focally with CK7. The diagnosis of EHE was made.

A Rarely Seen Hepatic Tumor: Epithelioid Hemangioendothelioma

Introduction: Epithelioid Hemangioendothelioma (EHE) is a rarely seen tumor most frequently originating from vascular structures of different regions including liver, followed by stomach, spleen, lung, bone, soft tissue, and skin. Case Presentation: In this paper, a patient with multiple mass lesions in the liver with a diagnosis of EHE who underwent liver transplantation is presented. Conclusion: It has an intermediate degree of malignancy between benign hemangioma, and malignant hemangiosarcoma. It can manifest with nonspecific symptoms or hepatic failure. Generally it is seen as a diffuse hepatic disease. The most optimal treatment alternatives are liver resection, and transplantation.

Hepatic Epithelioid Hemangioendothelioma with Cecal Metastasis in a Natural Course: A Case Report

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of low malignant potential that occurs mostly in soft tissue. So far, only two cases of EHE involving the intestine have been recorded. Here, we describe a rare case of cecal EHE due to a subsequent metastasis from the primary liver tumor three years after initial diagnosis. A 74- year-old man had sudden onset of epigastralgia for 24 hours. The abdominal CT revealed an ileocecal mass with a small bowel obstruction, and extensive tumor involvement in the liver was noted. He received an emergent right hemicolectomy for relief of the ileus. Unfortunately, he expired four days later due to septic shock. The pathologic diagnosis of EHE prompted a molecular study for a WWTR1-CAMTA1 fusion. The Sanger sequencing results showed the fusion involved exon 4 of WWTR1 with exon 8 of CAMTA1. There is no standard treatment for hepatic EHE because of its rarity and variable clinical outcome. The decision on a treatment strategy should be individualized for each patient. Since the patient received supportive care only for the liver tumor, this case demonstrated a natural course of hepatic EHE with a survival of more than 3.3 years.

CAMTA1 is a useful immunohistochemical marker for diagnosing epithelioid haemangioendothelioma.

The diagnosis of epithelioid haemangioendothelioma (EHE) is usually straightforward, based on characteristic histological features. However, it is sometimes difficult to differentiate EHE from a variety of other tumours with epithelioid morphology. The WW domain-containing transcription regulator 1-calmodulin-binding transcription activator 1 (WWTR1-CAMTA1) fusion gene, resulting in the overexpression of CAMTA1, is demonstrated in approximately 90% of EHEs, and the yes-associated protein 1-transcription factor E3 (YAP1-TFE3) fusion gene, associated with the strong and diffuse nuclear expression of TFE3, is present in another small subset of EHEs. The aim of our study was to examine CAMTA1 expression in EHEs and a variety of other tumours to evaluate its diagnostic utility, and to analyse TFE3 expression status in EHEs. Immunohistochemistry was performed on 16 EHEs, including five cases with CAMTA1 rearrangement and 276 non-EHE tumours. Fourteen of 16 EHEs and only one case of ductal carcinoma of the breast were positive for CAMTA1 and its expression was focal and weak in the latter (sensitivity 87.5%, specificity 99.6%). TFE3 expression was expressed focally and weakly in three (19%) EHEs (two with the CAMTA1 rearrangement). Nuclear CAMTA1 expression is sensitive and highly specific for EHE and can be applied to diagnostic immunohistochemistry in epithelioid tumours.

Epithelioid Hemangioendothelioma of the Rib: A Rare Tumour with a Confusing Localization

Background: Epithelioid hemangioendothelioma is a rare tumour whose prognosis ranges from benign form similar to hemangioma to aggressive tumors like angiosarcoma. Its localization in the rib is very rare and less than 10 cases of costal localized forms have been reported in the literature. The authors describe another case of costal epithelioid hemangioendothelioma illustrated by microscopic findings. Case presentation: We present the case of a 80-year-old man who reported a pain in the shoulder. Radiologic findings showed a hypervascularized tumour and microscopic findings diagnosed an epithelioid hemangioendothelioma. The treatment was the en-bloc resection of second to fourth posterior left ribs and the wedge resection of the left lung nodule and the patient didn’t present complications after a 6-year- follow-up period. Conclusion: This case emphasizes on the challenging diagnosis of epithelioid hemangioendothelioma and the importance of surgical resection in localized forms.

Successful Multimodality Treatment and Long- Term Follow-Up of a Primary Spinal Epithelioid Hemangioendothelioma

Successful Multimodality Treatment and Long- Term Follow-Up of a Primary Spinal Epithelioid Hemangioendothelioma Introduction: Epithelioid hemangioendothelioma (EHE) is an unusual vascular tumor which is rarely found in the spine. It has metastatic potential and unpredictable behavior. The choice of treatment remains controversial with the role of radiotherapy and chemotherapy still not clearly established. Case presentation: The case of a 63-year-old female patient with a spinal EHE that presented with gradually deteriorating paraparesis and sensory deficits is reported. Radiologic work-up revealed the presence of a mid-thoracic vertebral mass which extended epidurally and into the thoracic cavity. She underwent laminectomy and removal of the epidural mass. Postoperatively, she was further treated with intravascular embolization and stereotactic radiosurgery. These resulted in significant reduction of the tumor. Histopathologic analysis confirmed the diagnosis of EHE. Seven years later, she remained symptom-free with no evidence of tumor recurrence on Magnetic Resonance Imaging. Conclusion: Selective surgical excision combined with embolization and stereotactic radiosurgery may be effective in treating spinal EHEs obviating the need for an extensive surgery. To our knowledge, this is the first such case reported in the literature.

Malignant Epithelioid Hemangioendothelioma: A Case Report

Malignant epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin. We report a case of a woman who was found to have multiple hepatic masses in the right lobe of the liver on radiologic investigations, initially misdiagnosed as a metastatic carcinoma. The diagnosis of EH was made on histopathological study and confirmed by immunohistochemistry, which showed diffuse response for CD34 marker and no response to tissue CEA, HMB-45 or S-100 protein. Partial hepatectomy was made with good results.

Hemangioendotelioma epitelioide y fusiforme de ganglio linfático: Caso clínico

Primary vascular tumors of lymph nodes are extremely rare with the exception of AlDS-related Kaposi's sarcoma. The diagnosis of epithelioid hemangio-endothelioma (EH) is difficult to make without ancillary studies, since it is devoid of morphological features indicating its vascular nature and it may be overlooked when it appears as a primary tumor of lymph nodes. Spindle and epithelioid hemangio-endothelioma (SEH) is considered to be a variant of EH, which has been reported to occur exclusively in lymph nodes and the spleen. We report a 70-year-old male with chronic lymphocytic leukemia (CLL) and left cervical lymphadenopathy. An excisional biopsy was performed, and microscopically the lymph node showed effacement of nodal architecture by a tumor composed of spindle cells disposed in intersecting fascicles, and characterized by abundant eosinophilic cytoplasm, elongated nuclei and conspicuous nucleoli. A second population of cells had an epithelioid appearance with intracyto-plasmic vacuoles containing red blood cells. lmmunohistochemically, the tumor cells were positive for CD31 and CD34. The final diagnosis was SEH of the lymph node.

Pulmonary Artery Pseudoaneurysm Caused by a Rare Vascular Tumor: Epithelioid Hemangioendothelioma

We present a case of a 58-year-old female with a rare vascular tumor of intermediate malignancy. The initial manifestation was a pseudoaneurysm caused by the rupture of the right pulmonary artery after tumor invasion. The diagnosis of epithelioid hemangioendothelioma was confirmed by the morphologic and immunocytochemical features after surgery. The patient recovered smoothly and there has been no evidence of local recurrence or metastasis during the 2 years of follow-up.

ORIGINAL ARTICLE: Epithelioid hemangioendothelioma in the right auricle of an adult, male Rhesus macaque (Macaca mulatta)

Abstract Background A 9‐year‐old, male castrate, Rhesus macaque was euthanized following a prolonged history of chronic renal failure.Results Necropsy revealed a proliferative lesion within the right cardiac auricle composed of neoplastic epithelioid cells which infiltrated the myocardium and frequently exhibited intracytoplasmic luminae. Cells multifocally exhibited strong cytoplasmic immunoreactivity for Factor VIII‐related protein (von Willebrand’s factor).Conclusions The histological characteristics of this tumor are consistent with a diagnosis of epithelioid hemangioendothelioma, an intermediate‐grade vasoformative neoplasm which has to our knowledge not previously been reported in the heart of a non‐human species.

Meningel intracranial epithelioid hemangioendothelioma: case report and literature review

Intracranial epithelioid hemangioendothelioma (EH) is a very rare vascular neoplasm. We describe a case of meningeal intracranial tumor in a 58 year old man, located in the greater wing of the sphenoid bone. The tumor was completely excised via pterional approach and the pahological analysis showed a hemorrhagic lesion with capillary-sized vessels lined by atypical-appearing endothelial cells that expressed CD31, CD34 and Factor VIII antigens. A diagnosis of EH was established, and one year after surgery the patient is symptom-free, without tumor recurrence or residual tumor. A review of the literature disclosed other 34 cases of intracranial EH. In these tumors, total resection is mandatory if possible, otherwise radiotherapy is recommended. Outcome is favorable in the majority of cases.

Primary Peritoneal Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma is an uncommon malignant vascular tumor of intermediate grade that occurs in a variety of organs and soft tissues. Diagnosis of epithelioid hemangioendothelioma is often complicated by the rarity of the tumor, and because the tumor shares many morphologic features with other peritoneal neoplasms. This report presents 3 cases of epithelioid hemangioendotheliomas arising as primary tumors of the peritoneum and reviews 7 previously reported cases to establish the demographic, clinical, morphologic, immunohistochemical, and ultrastructural features of this neoplasm. These cases demonstrate that the light microscopic features are very similar to epithelioid hemangioendothelioma arising at more conventional sites, such as the liver, that immunohistochemical analysis provides a reliable approach for confirming or establishing the diagnosis, and that at least one endothelial marker (either CD31, CD34, or factor VIII) should be positive for a definitive diagnosis. This series identifies the characteristics of peritoneal epithelioid hemangioendothelioma that can be reliably used for diagnosis.