Epitheloid Hemangioendothelioma Research Articles

Hepatic epithelioid hemangioendothelioma: a diagnostic challenge of vascular tumors.

Epitheloid hemangioendothelioma is a very rare tumor, with a variable presentation and unpredictable clinical behavior. The etiology and the triggering risk factors have not been specified. Unlike other primary liver tumors, it does not arise in the background of chronic liver disease. The approach is challenging due to the spectrum of possibilities and the need for immunohistochemistry to establish the definitive diagnosis. The information available so far is limited due to the few published cases, this favors that the therapeutic options are few or that there is insufficient evidence to standardize them when the lesion is not resectable.

PLEURAL EPITHELIOID HEMANGIOENDOTHELIOMA

TOPIC: Disorders of the Pleura TYPE: Medical Student/Resident Case Reports INTRODUCTION: Epitheloid Hemangioendothelioma (EHE) is a rare malignant vascular tumor with extremely variable presentations and organ involvement. Here, we present a case of pleural EHE in a 40-year-old male who presented with spontaneous non-traumatic hemothorax. CASE PRESENTATION: A 40-year-old African American male, never smoker, with past medical history of pulmonary nodules was being evaluated for new multiple pulmonary nodules. When he presented for CT-guided biopsy of one of the nodules, he was noted to have right hemithorax opacification and was referred to the emergency room for further evaluation.He was minimally symptomatic with right pleuritic chest pain and exertional dyspnea. CT chest showed complete opacification of right hemithorax with evidence of mass effect and mediastinal shift to the left. Point of care ultrasound showed heterogenous densities consistent with the CT findings. Bedside thoracentesis was attempted and small amount of dark blood was aspirated. He then underwent diagnostic/therapeutic VATS with pleural biopsy. Intraoperatively, approximately 2500 cc of old blood was evacuated from the right pleural cavity. Other findings included thickened, hard, calcified pleura stuck to the lung and unexpandable right lung. He was discharged home with CT surgery follow up. Pleural biopsy was positive for neoplastic cells and immunohistochemical staining was consistent with a vascular tumor. Due to the extensive pleural involvement, surgical excision was not possible. He was referred to oncology for discussion regarding chemotherapy. DISCUSSION: EHE has heterogenous presentations involving various organs, more commonly the lung and liver. Pleural involvement can be primary or metastatic. While PEH seems to be more common in women, pleural EHE appears to be more common in men with the average age at diagnosis being 45.7 years and mean survival of 4.6 years. Radiobiologic characteristics aid in the diagnosis. Presence of multi-organ involvement, pulmonary lesions, male sex, age greater than 55 years confers poor prognosis. There is no standard treatment for pleural EHE although surgical intervention, chemotherapy, and radiation, alone or in various combinations, have been reported. CONCLUSIONS: Pleural epithelioid hemangioendothelioma, with an incidence of less than one in a million, can present as spontaneous hemothorax in a middle-aged male; one of its myriad presentations. REFERENCE #1: Sardaro, Angela et al. "Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor." Oncology reviews vol. 8,2 259. 13 Oct. 2014, doi:10.4081/oncol.2014.259 REFERENCE #2: Salijevska, Julita et al. "Pleural epithelioid hemangioendothelioma: literature summary and novel case report." Journal of clinical medicine research vol. 7,7 (2015): 566-70. doi:10.14740/jocmr2174w REFERENCE #3: Wu, Xin et al. "Clinical characteristics of epithelioid hemangioendothelioma: a single-center retrospective study." European journal of medical research vol. 24,1 16. 28 Feb. 2019, doi:10.1186/s40001-019-0375-8 DISCLOSURES: No relevant relationships by Yashubhrika Bharani, source=Web Response No relevant relationships by Andrew Holt, source=Web Response No relevant relationships by Qasim Mirza, source=Web Response No relevant relationships by Mary Thomas, source=Web Response No relevant relationships by Snigdha Yeramareddy, source=Web Response

Liver Transplantation for Epithelioid Hemangioendothelioma (EHE) - Short and Long-term Outcomes from 30 Years' Experience

Aim: We report our experience of liver transplantation (LT) for Epitheloid hemangioendothelioma (EHE) which is an uncommon vascular tumour of liver with intermediate malignant potential. Single centre experience remains limited.

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.

Coexistence of Pulmonary and Mediastinal Epitheloid Hemangioendothelioma- A Rare Tumor with A Rare Presentation

Epitheloid hemangioendothelioma (EHE) is a very rare,vascular, low to intermediate grade malignant tumor. It has a predilection for the lungs and liver but may arise from many organs, simultaneously or sequentially. Mediastinal location is extremely rare with no more than 25 cases in the literature. In the present case, we report synchronic lung and mediastinal EHEs which have different histopathologic grades and FDG uptakes. A 49-year-old male patient was admitted to our hospital with respiratory complaints. Computed tomography revealed a left mediastinal tumor and bilateral multipl lung nodules. Positron Emission Tomography scan showed a significant fluorodeoxyglucose (FDG) uptake in the mediastinal mass (SUVmax:9.7) but no significant uptakes in pulmonary nodules. Histopathologic and immunohistochemical analysis were consistent with a low grade EHE for lung nodules and intermediate grade EHE for mediastinal tumor. The patient had chemotherapy postoperatively but died 12 months after the initial diagnosis due to the respiratuary failure. Epitheloid hemangioendothelioma should be kept in mind in the differential diagnosis of synchronic tumors involving lung and mediastinum. It is very difficult in some cases to confirm whether the tumor is a primary, multicentric or metastatic lesion. Clinical behaviour is variable among different cases but prognosis is poor for intermediate grade tumors.

A RARE CASE OF A GIANT HEMANGIOENDOTHELIOMA OF NECK

Introduction
 Hemangioendothelioma is a rare variety of vascular soft tissue neoplasm, classified as a borderline tumour with diverse modality of clinical presentation. It is seen mostly at sites like the liver, lungs and bones while very few head and neck cases have been reported. 
 Case Report
 A rare case of an unusually large tumour mass presenting as an anterolateral neck mass with extensions to right parapharyngeal space, posterior triangle and prevertebral space is presented here. The entire tumour was removed en-masse surgically. Histopathological examination confirmed the mass to be an epitheloid hemangioendothelioma, the enormous size of which in the neck makes it unique and unusual.

Epitheloid hemangio-endothelioma of right aryepiglottic fold: a rare case report with review of literature

<p class="abstract"><span lang="EN-US">Epitheloid hemangio-endothelioma is a very rare tumour of intermittent malignancy of vascular origin, having a tendency to recur with rare incidence of metastasis. The tumour is intermediate between haemangioma and angiosarcoma, mainly affecting liver, lung as well as bones, skin, penis, ovary, scalp, or any part of the body. Internet search was made with the key words epitheloid hemangio-endothelioma and epitheloid hemangio-endothelioma of Larynx, since now only single case has been reported from larynx involving subglottis. Hence we report this rare entity with involvement of the larynx (Sub site: Rt. Aryepiglottic fold) describing clinical and histopathological characteristic. This is perhaps the first case of epitheloid hemangio-endothelioma involving aryepiglottic fold.</span></p>

A Rare Pathology of the Parotid Gland: Epitheloid Hemangioendothelioma

Epitheloid hemangioendothelioma is a tumor of vascular origin with the proliferation of endothelial cells in Epitheloid morphology which is reported very rarely in parotid gland. Clinically it has a borderline acting pattern between hemangioma and angio sarcoma with its local aggressive and metastatic features. World Health Organization put the Epitheloid subtype of hemangioendothelioma to the malign category in soft tissue tumors classification in 2013. Herein we report a case of Epitheloid hemangioendothelioma who had experienced total parathyriodectomy due to a parotid mass in our clinic. In 2 years of follow up of this case any recurrences is not determined.

Epitheloid Hemangioendothelioma of the Submandibular Region

Objective: To present an uncommon cause for a submandibular mass and review of the literature.
 Methods:
 Design: Case Report
 Setting: Tertiary Government Hospital
 Patient: One
 Results: 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.
 Conclusion: 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.
 Keywords: epitheloid hemangioendothelioma, malignant vascular tumor, submandibular mass

Primary hepatic malignant epitheloid Hemangioendothelioma represent a genetically homogenous tumor entity

Epitheloid Hemangioendothelioma (EHE) is a rare malignant vascular tumors of unknown etiology and molecular pathogenesis. It may arise at many different sites, but liver is among the more frequent manifestations. Course of the disease is highly variable and so far only limited therapeutic options are available such as resection and liver transplantation. Our aim is to molecularly characterize this tumor entity in order to find new potential therapeutical targets.

Epitheloid hemangioendothelioma of the subcutaneous tissues of a finger.

Epithelioid hemangioendotheliomas (EHEs) are known to have a variable malignant potential. EHEs are rarely seen in the hand and there is no consensus about their management. The options include excision, excision followed by adjuvant radiotherapy and amputation. In this paper, we report a case of EHE of a finger that was treated by excision. Although the tumor had ill-defined borders and there was histological evidence of tumor extension to all resection margins, no local recurrence or metastasis were noted during the 3 years of followup. The literature is reviewed and an argument is made that EHEs of the hand may have a more benign behavior compared with EHEs of the lower limbs and viscera.

Spindle Cell Hemangioma of the Nasal Cavity: A Rare Tumor with Unusual Presentation

ABSTRACT Vascular tumors as such are rare in the nasal cavity. Few cases of lobular capillary hemangioma, cavernous hemangioma and epitheloid hemangioendothelioma have been reported in the nasal cavity. But, there are no reports of spindle cell hemangioma occurring within the nasal cavity. They usually present as subcutaneous nodules in the extremities. A case of recurrent bleeding polypoid lesion in the left nasal cavity, arising from the middle turbinate which showed features of spindle cell hemangioma is reported for its rarity and unusual presentation. The tumor was excised by minimally invasive transnasal endoscopic approach. How to cite this article Suranagi V, Harugop AS, Bannur HB, Pilli GS, Mudhol RS. Spindle Cell Hemangioma of the Nasal Cavity: A Rare Tumor with Unusual Presentation. Clin Rhinol An Int J 2013;6(3):149-151.

A review of transplantation for tumors other than hepatocellular carcinoma

Introduction: Debates are ongoing in many centers for the indications of liver transplantation for non-hepatocellular carcinoma tumors due to a number of factors. Standardized protocols are lacking and clinical outcomes vary. Objective: A review was performed of the numerous published articles, with focus on the management and clinical outcome of tumors besides hepatocellular carcinoma (HCC) which were treated by liver transplantation, namely, cholangiocarcinoma, neuroendocrine tumors and hepatic epitheloid hemangioendothelioma in an attempt to promote a more standardized guideline. Discussion: Liver transplantation for cholangiocarcinoma reveals promising results in selected group of patients who underwent neoadjuvant chemoradiation prior to transplantation. For neuroendocrine tumors with liver metastases a number of selection criteria have been proposed in different centers, however the results of which are still variable. Transplantation is a viable option for unresectable hepatic epitheloid hemangioepithelioma with results at par with resection. Conclusion: Over time as patient selection, techniques, peri-operative care and the understanding of the different diseases improves, the results of liver transplantation for these tumors likewise will became favourable. However, standardization of the management and guidelines are still needed.

Metastatic lung angiosarcoma from a femoral subperiosteal origin

Abstract Primary malignant vascular bone tumors are unusual and include hemangioendothelioma, epitheloid hemangioendothelioma and angiosarcoma. Although few cases of primary bone angiosarcomas have been reported, those of femoral origin are even more infrequent. Such tumors diagnosis may be challenging due to their radiographic and histologic variety. We present a case of a 24-yearold woman with a subperiosteal diaphyseal angiosarcoma originating from the femoral bone and metastatic to the lung at the time of diagnosis. The clinical, histological and radiological features of this extremely rare lesion are presented.

Epitheloid Hemangioendothelioma and Liver Transplantation

Purpose: A 45 year old man presented with low grade fever, progressive weight loss and right upper abdominal discomfort of 3 wks duration was found to have a diffuse infiltrating mass involving the right and majority of the left lobe. The patient underwent a needle biopsy of the mass in an outside institution and was diagnosed as hepatocellular carcinoma. He underwent two sessions of Transarterial chemoembolization (TACE) with doxorubicin beads. Post TACE his symptoms worsened, he presented to our hospital with worsening fatigue, dyspnea, jaundice and rapid increase in abdominal girth. Computerized tomography (CT) of the abdomen revealed large ascites and hepatic hydrothorax. He underwent paracentesis and thoracocentesis draining 5L and 1L respectively. The fluid cytology was negative for malignancy. The laboratory findings included the following. Bilirubin: 27 mg/dL, alkaline phosphates: 449 U/L, alanine aminotransferase: 77 U/L, aspartate aminotransferase: 123 U/L, albumin: 1.8 g/dL, all hepatitis, viral and autoimmune panels were negative. INR: 1.54, AFP: 2.3 ng/ml, CA 19-9: 4.2, CEA: <0.5 mcg/L. CT of the abdomen showed a nodular, shrunken liver with a large diffuse hypodense lesion involving the entire right lobe of the liver, and the medial lateral segment of the left lobe of the liver. The lesion in the right lobe of liver was causing mass effect on the intrahepatic portion of the inferior vena cava. No intrahepatic biliary dilatation. Review of the needle biopsy revealed epitheloid cells with immune positivity to CD31 and CD34. A diagnosis of epitheloid hemangioendothelioma was entertained and he underwent liver transplantation. Post transplant period was uneventful and, he is doing quite well and fully functional five months after his transplant. Discussion: Hepatic epithelioid hemangioendothelioma(EHE) is a rare, low-grade malignant vascular tumor. It is usually misdiagnosed as HCC. The EHE has a better prognosis compared to same grade of HCC. EHE presents histologically as small groups or isolated neoplastic cells surrounded by a generous amount of fibro-sclerotic stroma, which eventually obliterates the hepatic plates. All tumors should be positive for at least one endothelial marker, such as CD34, CD31, or factor VIII related antigen with immunohistochemical staining. Most common treatment is OLT followed by liver resection (LRx). LRx for HEHE is restricted to patients with limited involvement of the liver. Most patients with HEHE have large, multifocal tumors at the time of diagnosis rending OLT as the most feasible option. Patient survival rates after OLT at one and five years were 80% and 64%, respectively. Allograft survival rates were 70% and 55%, respectively.

Unresectable Epitheloid Hemangioendothelioma of the Neck Region Cured by Radiotherapy

Epitheloid hemangioendothelioma (EHE) is a rare vascular neoplasm which has a variable malignant potential. EHE cases have been reported in the head and neck region. This is the report of an unresectable neck EHE cured totally by radiotherapy. As EHE has a malignant potential, wide local excision is the accepted treatment of choice. If total resection is not possible, additional curative therapy modalities are needed. Invasive forms of EHE which can not be resected totally can be treated by radiotherapy. doi:10.4021/jmc216w

Metastatic epitheloid hemangioendothelioma (EHE): Role of systemic therapy and survival.

10079 Background: Data regarding the management of patients with metastatic EHE are almost nonexistent. Methods: From 1990 to 2010, 78 adult patients (pts) with a confirmed diagnosis of EHE were referred to one of the 9 participating institutions, and their medical records reviewed. Results: Forty-two (54%) patients had metastatic disease. Median age was 45 years. Systemic therapy was delivered in 34 cases (81%): single agent in 24 cases (71%). Twenty-two patients received cytotoxic chemotherapy: anthracycline-containing regimen (+/- ifosfamide): n=16; others: n=6. Twelve patients received an angiogenesis-targeting drug and/or a tyrosine kinase inhibitor: sorafenib n=6, thalidomide: n=2, metronomic cyclophosphamide: n=2, imatinib: n=2. No objective response was observed. Fourteen patients had stable disease > 6 months: cytotoxic chemotherapy n=8 (doxorubicin-containing regimen: n=4, other n=4); angiogenesis-targeting agent n=6 (sorafenib: n=3, metronomic cyclophosphamide: n=2, thalidomide n=1). 9/16 (56%) treated with doxorubicin-containing regimen had disease progression within 3 months of starting treatment. Median progression-free survival (PFS) was 4.8 months (mo) (95% CI: 2.6-7.1). Age <45 years was the sole factor significantly associated with PFS: 6.7 mo (95% CI: 4.4-9.0) vs 2.2 mo (95% CI: 1.9-2.6) (log-rank test: p=0.03). 16 patients received further lines of systemic treatments (median: 1, range 1-4). Median overall survival (OS) was 41 mo (95% CI: 0.1-90). Age <45 years and performance status ≤1 were the sole factors significantly associated with better OS. 6/8 pts who did not receive systemic treatment are still alive, after a median of 49 months (range 11-188) from the time of distant recurrence, consistent with presence of a subset of patients with indolent disease who do not require systemic treatment. Conclusions: OS of patients with metastatic EHE appears significantly longer than most other metastatic soft-tissue tumors. Anthracyclines and ifosfamide have modest activity while long-term stable disease is achievable with anti-angiogenic drugs which have a better safety profile. Systemic therapy should be avoided in patients with indolent, asymptomatic disease.

Epitheloid Haemangioendothelioma Liver Coexisting with Retroperitoneal Rosai Dorfman Disease– A Rare Case Report

Epitheloid hemangioendothelioma is a rare tumor of vascular origin which occurs at sites such as soft tissues liver or lung and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. Neoplastic cells express factor VIII related antigen CD31 and CD34. Hepatic epitheloid hemangioendothelioma is a rare vascular tumor. The clinical course is unpredictable and different treatment modalities are offered depending on the patientrsquos conditions. Rosai-Dorfman disease originally described as sinus histiocytosis with massive lymphadenopathy is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites mediastinal and inguinal are also frequently involved. The retro-peritoneum is an infrequent site of involvement.We report a case of a 41 year old man presented with recurrent pain abdomen diagnosed as epitheloid hemangioendothelioma liver coexisting with retroperitoneal Rosai Dorfman disease. He was managed symptomatically without any modalities of treatment with regular follow up every month since one year. This case highlights the diagnostic dilemma of retroperitoneal Rosai Dorfman disease which mimicked metastasis from hepatic neoplasm and its rare association with epitheloid hemangioendothelioma liver

Unresectable Epitheloid Hemangioendothelioma of the Neck Region Cured by Radiotherapy

Unresectable Epitheloid Hemangioendothelioma of the Neck Region Cured by Radiotherapy

Multifocal Epitheloid Hemangioendothelioma of Liver after Long-Term Oral Contraceptive Use—A Case Report and Discussion of Management Difficulties Encountered

A 43-year-old woman presented with right upper abdominal pain, on and off for 3-4 years. One year prior to her presentation, a space-occupying lesion was found in the right lobe of the liver, which was suspected to be hemangioma, as the patient had history of combined oral contraceptive pill use for the last 15 years. On examination, she was thinly built and had pallor; abdominal examination revealed tender hepatomegaly 3 cm below the costal margin, smooth surface, sharp regular edge with left lobe enlargement, and no bruit. All routine biochemical and hematologic investigations were normal, except hemoglobin of 7.6 gm/dl. On triple-phase CT scan, there were hypodense ill-defined lobulated area in right lobe of liver; similar lesions were also seen in segments V and VII of liver along with a few sub-diaphragmatic and para-aortic nodes. Liver biopsy showed features suggestive of hemangioendothelioma, and immunohistochemistry showed CD 31 and CD 34 positivity but negative for estrogen receptors. Laparoscopy revealed presence of multiple deposits on left lobe of liver and ascites. Tumor was unresectable, and the patient was found to be a candidate for liver transplant. Chemoembolization was tried as a palliation awaiting transplant, but the procedure was unsuccessful due to the hypovascular nature of the tumor. The patient died within 4 months of diagnosis due to liver failure, awaiting liver transplant, as a suitable donor was not available. A clinical suspicion based on demographics, risk factors and imaging, familiarity with the pathologic findings, and utilization of advanced imaging techniques may allow early diagnosis of these tumors and hence their appropriate management.