Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma

Hemangioendothelioma is a rare vascular tumor of intermediate malignant potential. Though epithelioid hemangioendothelioma (EH) is commonly found in soft tissues, it has been known to be present in skeletal tissues. The authors present a case of a 50-year-old woman diagnosed with EH of the iliac bone and acetabulum, who experienced pathological fracture at presentation. This report describes a multidisciplinary approach to the management that includes initial incisional biopsy, curettage, and bone grafting, followed by Intensity Modulated Radiation Therapy. The patient finally underwent hemipelvic resection with allograft reconstruction after recurrence. Histopathological study revealed osseous EH of low mitotic activity that stained positively for CD31, CD34, vimentin, and Factor VIII. Herein, the authors discuss the imaging characteristics, histopathological aspects, cytogenetic findings, and the radiobiological behavior of osseous EH. After an aggressive multidisciplinary intervention, the patient is able to achieve local control with no evidence of distal metastatic disease.

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Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

Diagnostic Pathology ◽

10.1186/s13000-020-01039-2 ◽

2020 ◽

Vol 15 (1) ◽

Author(s):

Nasir Ud Din ◽

Shabina Rahim ◽

Tamana Asghari ◽

Jamshid Abdul-Ghafar ◽

Zubair Ahmad

Keyword(s):

Surgical Resection ◽

Case Series ◽

Liver Function Tests ◽

Vascular Tumor ◽

Metastatic Carcinoma ◽

Epithelioid Hemangioendothelioma ◽

Clinicopathological Features ◽

University Hospital ◽

Detailed Review

Abstract Background Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature. Methods All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained. Results Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon. Conclusion Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.

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Metastatic cutaneous epithelioid hemangioendothelioma involving right atrium - a case report

Case Reports in Internal Medicine ◽

10.5430/crim.v3n3p47 ◽

2016 ◽

Vol 3 (3) ◽

pp. 47

Author(s):

Sandy Mpho Mosenye ◽

Rahul Daimari ◽

Neha Sharma ◽

Princess Lelani Chikanda ◽

Feng Zhou ◽

...

Keyword(s):

Right Atrium ◽

Cardiac Involvement ◽

Soft Tissues ◽

Vascular Tumor ◽

Epithelioid Hemangioendothelioma ◽

Malignant Potential ◽

Vascular Endothelial ◽

Rare Occurrence ◽

Scalp Lesion ◽

The Common

Epithelioid hemangioendothelioma (EHE) is a rare vascular endothelial tumour with unpredictable malignant potential. The common sites involved are the lung, liver and soft tissues; with cardiac involvement being a very rare occurrence. The diagnosis of this rare vascular tumor is made by typical histopathological findings and confirmed by specific immunohistochemistry. We present a case of a patient with a long standing painless scalp lesion who presented with pulmonary symptoms and ultimately diagnosed to have metastatic cutaneous EHE involving the lungs, pericardium and right atrium.

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Epithelioid hemangioendothelioma: A rare vascular tumor

Indian Journal of Dermatology ◽

10.4103/0019-5154.92681 ◽

2012 ◽

Vol 57 (1) ◽

pp. 53 ◽

Cited By ~ 4

Author(s):

SVidya Lakshmi ◽

D Prabhavathy ◽

S Jayakumar ◽

C Janaki ◽

GK Tharini

Keyword(s):

Vascular Tumor ◽

Epithelioid Hemangioendothelioma

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HepaticYAP1-TFE3Rearranged Epithelioid Hemangioendothelioma

Case Reports in Gastrointestinal Medicine ◽

10.1155/2019/7530845 ◽

2019 ◽

Vol 2019 ◽

pp. 1-5

Author(s):

Mira M. Lotfalla ◽

Andrew L. Folpe ◽

Karen J. Fritchie ◽

Patricia T. Greipp ◽

Gretchen G. Galliano ◽

...

Keyword(s):

Fusion Gene ◽

Vascular Tumor ◽

Epithelioid Hemangioendothelioma ◽

Low Grade ◽

Diagnostic Pitfall ◽

Vascular Neoplasms ◽

Nodular Hyperplasia ◽

Genetic Features ◽

Diagnostic Confusion

Epithelioid hemangioendothelioma (EHE) is an uncommon low-grade malignant vascular tumor that may arise in soft tissue/bone or visceral sites such as the liver and lung. As this tumor exhibits epithelioid morphology, it frequently causes diagnostic confusion with other epithelioid vascular neoplasms as well as carcinoma. While 90% of classic EHE are driven by aWWTR1-CAMTA1fusion gene, a histologically distinctive subset of EHE has been recently shown to harbor a different fusion gene,YAP1-TFE3. This variant is likely underrecognized given its rarity and only recent description. Notably, EHE frequently involves the liver but only one case of hepaticYAP1-TFE3rearranged EHE has been reported to date. We present the second case ofYAP1-TFE3rearranged EHE affecting a 65-year-old woman and presenting as multiple liver masses, with characterization of the fusion gene at the transcriptomic and genomic levels. There are several educational points noted from this case.YAP1-TFE3rearranged EHE shows distinctly vasoformative foci, unlike classic EHE and mimicking angiosarcoma or epithelioid hemangioma. The tumors cells show a histiocytoid appearance with voluminous cytoplasm, similar to otherTFE3-rearranged tumors. Finally, in the liver, this tumor may in part mimic focal nodular hyperplasia of the liver which is an underrecognized diagnostic pitfall. This report highlights the key diagnostic and genetic features of this newly recognized variant of hepatic EHE to aid pathologists in appropriately classifying these tumors.

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Maxillary epithelioid hemangioendothelioma: an especially rare malignant tumor mimicking periodontal disease

BMC Oral Health ◽

10.1186/s12903-020-01291-4 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Gintaras Januzis ◽

Dovydas Sakalys ◽

Martynas Mantas Krukis ◽

Dmitrij Seinin

Keyword(s):

Correct Diagnosis ◽

Vascular Tumor ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors ◽

Low Grade ◽

X Rays ◽

Adequate Treatment ◽

Clinical Appearance ◽

Allogenic Bone ◽

Bony Destruction

Abstract Background Epithelioid hemangioendothelioma (EHE) is an especially rare, low-grade malignant vascular tumor that, according to WHO classification, is described as locally aggressive tumor with possible metastasis and makes up 1% of all vascular tumors. EHE is characterized by the accumulation of round, eosinophil-infiltrated endothelium cells; with vacuolation of their cytoplasm; frequent angiocentric inflammation; and myxohyaline stroma. This tumor is usually found in the liver, lungs, and bones and is especially rare in the mouth. Case presentation We present an 18-year-old Caucasian female whose oral cavity lesion had been misdiagnosed as marginal periodontitis. The patient was treated improperly for 2 years until she was referred to a maxillofacial surgeon. The patient complained only about gingival recession in the palatal area of her upper-right-side 13th, 14th, and 15th teeth. The lesion’s clinical appearance was of locally ulcerated painless lesion that affect the underlying bone as seen in X-rays in the palatal side of the right canine and the first and second premolars. Patient underwent surgery for her present defect and reconstruction using allogenic bone transplant. The diagnosis of EHE was based on the bony destruction as seen in x-rays and in the accumulation of tumor cells that were 100% positive to CD31; CD34 and ERG to endothelial markers. During the 31-month follow-up period, the patient exhibited no clinical and radiographic complications. Conclusions With this clinical case, we demonstrate that this rare tumor must be included in differential diagnoses of periodontal pathologies to perform histomorphological examination in a timely manner, which could lead to correct diagnosis and adequate treatment.

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Epithelioid hemangioendothelioma of the retroperitoneum – a rare vascular tumor

VASA ◽

10.1024/0301-1526/a000898 ◽

2020 ◽

pp. 1-5

Author(s):

Fragiska Sigala ◽

George Galyfos ◽

Efthimios Koniaris ◽

Christos Barkolias ◽

Sotirios Artsitas ◽

...

Keyword(s):

Rare Case ◽

Clinical Presentation ◽

Vascular Tumor ◽

Epithelioid Hemangioendothelioma ◽

Optimal Treatment ◽

Proper Management

Summary: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, affecting the liver, the lungs and the bones most frequently. It has a heterogenous clinical presentation and there is no consensus on optimal treatment. This report aims to present a rare case of a retroperitoneal EHE and to discuss on proper management.

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