Primary Epithelioid Hemangioendothelioma Research Articles

Epithelioid hemangioendothelioma: Real-world data about a rare entity in a Portuguese tertiary cancer center.

e23525 Background: Epithelioid hemangioendothelioma (EHE) is a rare entity of vascular origin, whose malignant potential is uncertain. It can develop in different locations, most commonly bone, liver and lungs. Since there are no specific associated symptoms, the diagnosis is often incidental. Due to its rarity there is no standard treatment. Our aim was to evaluate the demographic characteristics, course of disease and treatment effectivity in EHE patients in our institution in the last 25 years. Methods: We performed a retrospective study of all patients with EHE treated at our institution between January/1997 and September/2023. Statistical analysis was performed using SPSS. Normal distribution was checked using Shapiro-Wilk test. Results: From the 23 patients diagnosed with hemangioendothelioma, 20 were epithelioid (EHE) and 3 were other type. Of note, all the biopsies were centrally reviewed and since 2022 the diagnosis were confirmed with WWTR1 rearrange (3 out of 5 patients were positive). From the total number of EHE, the majority were women (n = 13, 65%). The mean age at diagnosis was 46 ± 22 years-old. Seven (35%) were asymptomatic and 13 (65%) had metastatic disease at diagnosis, all of them with lung metastasis, 2 (10%) with hepatic metastasis and 2 (10%) with bone metastasis. The most frequent primary locations were lungs (n = 5, 25%); soft tissue (n = 5, 25%), and liver (n = 4, 20%), followed by bone (n = 2,10%), stomach (n = 1, 5%), parotid gland (n = 1, 5%), superior vena cava (n = 1, 5%) and one case of unknown primary EHE. The majority of tumors were multifocal (12, 60%). Eight (40%) patients were under active surveillance, 4 (20%) underwent surgery with or without adjuvant radiotherapy, 2 (10%) underwent systemic treatment with tyrosine kinase inhibitor (pazopanib) and 2 (10%) chemotherapy (paclitaxel, doxorubicin). Two (10%) patients received a liver transplant and 2 (10%) patients underwent multiple treatment modalities. With a median follow-up of 30.89 months (3-253), 6 (30%) patients were dead at the time of analysis and mean overall survival was 142.3 months. Patients with an angiosarcoma differentiation had significantly worse mean survival outcomes (p < 0.001), 7.9 ± 4.5 months vs. 158.9 ± 37.2 months. Conclusions: The biological behavior of these tumors remains uncertain. In our cohort, the majority of patients presented with distant metastasis, but the course of the disease was very heterogeneous, regardless of the primary or metastatic site or the treatment modality. Our data emphasizes that this disease should be managed on a case-by-case basis, with a multidisciplinary approach and tailored treatment and surveillance.

Surgical treatment of intracranial epithelioid hemangioendothelioma: a case report

Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular neoplasm with an intermediate pattern of malignancy between benign neoplasms and angiosarcomas. Only 46 cases of intracranial localization of primary EHE in adults have been published.Case report: A 47-year-old male patient developed pronounced speech disorder of sensory and amnestic aphasia type, right-sided pyramidal hemiparesis within 3–4 days. Magnetic resonance imaging revealed a delimited left sided brain islet neoplasm with signs of hemorrhage and small perifocal edema. Intraoperatively, the neoplasm was represented by a conglomerate of pathologic vessels, with involvement of the terminal branches of the M2 segment of the middle cerebral artery, which prevented radical resection of the neoplasm. Based on histopathological and immunohistochemistry analysis of the neoplasm (positive expression of CD31, CD34, proliferative activity index Ki-67 10%) the morphologic diagnosis of EHE was established. 3 months after surgery, continued growth of the residual part of the neoplasm was noted. Temozolomide chemotherapy was clinically ineffective. The patient died due to the development of dislocation syndrome after 9 months.Discussion. The malignant characteristics of EHE include invasive growth, recurrence, and metastasis, which is more common in intracranial localization of the neoplasm. If EHE is suspected, radical surgical removal of the neoplasm should be sought.

Primary epithelioid hemangioendothelioma of the eyelid: A case report

Eyelid epithelioid hemangioendothelioma (EHE) is uncommon. The present study reports the clinical, histopathological and immunohistochemical characteristics of a 10-year-old girl with EHE and reviews the related literature. The patient was admitted with a painless red mass on the left eyelid, which had been present for 6 months. Under the microscope, tumor cells from the surgical specimen were composed of epithelioid cells (70%) and spindle cells (30%). The cytoplasm was eosinophilic, and some areas of the cytoplasm showed signet ring-like or vacuolization changes. The presence of red blood cells could be seen in the luminal part of the primary blood vessel. Immunohistochemical results showed that the tumor cells were positive for CD34 and CD31, and the Ki-67 proliferation index was 3%. As the first resection margin was positive, recurrence occurred 3 months after surgery, Therefore, patients with such tumors should undergo full resections to ensure a negative margin to prevent recurrence.

Subcutaneous axillary primary epithelioid hemangioendothelioma: report of a rare case

BackgroundEpithelioid hemangioendothelioma (EHE) is a rare and slow-growing malignant vascular neoplasm composed of epithelioid endothelial cells within a distinctive myxohyaline stroma. It most commonly involves somatic soft tissue, lungs, liver and bone. Herein, we describe a case of EHE arising in the axillary region.Case presentationA 61-year-old man was under observation for multiple hepatic hemangiomas. Fluorodeoxyglucose–positron emission tomography/computed tomography showed specific uptake in a right axillary tumor. The patient was referred to our department for further investigation of the axillary tumor. An elastic-soft and poorly mobile tumor was palpable in the right axilla. Contrast-enhanced computed tomography showed a right axillary tumor and enlarged hepatic hemangiomas. In addition, multiple nodules in both lungs, a left renal angiomyolipoma, and left adrenal adenoma were revealed. Ultrasonography showed masses in both lobes of the thyroid gland, and a 30-mm lobulated hypoechoic mass in the axilla with well-defined and rough borders, showing internal heterogeneity. Fine-needle aspiration cytology was performed on the thyroid and axillary tumors: the thyroid tumor was class V, raising suspicion of papillary thyroid cancer (PTC); the left superior internal jugular node was class V, raising suspicion of metastasis of PTC; and the axillary tumor was class III, raising suspicion of a mesenchymal tumor with few epithelioid cells. The multiple lung nodules were diagnosed as metastatic tumors derived from thyroid cancer. We diagnosed these diseases as PTC of T1b(m)N1bM1(lung) Stage IVB and a right axillary tumor of unclear origin. However, it was assumed to be a primary mesenchymal tumor or a lymph node metastasis from lung cancer or occult breast cancer. We performed total thyroidectomy, left cervical lymph node dissection, and right axillary tumor excision. Histopathologic examination revealed the thyroid tumor as a PTC and the axillary tumor as an EHE. The EHE showed nuclear atypia, necrosis and high mitotic figures. Hence, it was considered to be a high-risk EHE.ConclusionsWe experienced a rare primary subcutaneous axillary EHE with metastatic thyroid cancer in the lung. Since our case was classified as a high-risk EHE, a close follow-up would be appropriate.

Imaging Findings and Clinical Features of Primary Spinal Epithelioid Hemangioendothelioma.

Rationale and ObjectivesPrimary spinal epithelioid hemangioendothelioma (SEHE) is an extremely rare angiocentric vascular neoplasm with inherent metastatic potential, which pursues a clinical course intermediate between hemangioma and angiosarcoma. The present study sought to present computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical features of primary SEHE and review the literature.Materials and MethodsClinical data of four patients with primary SEHE confirmed by pathology from January 2011 to May 2020 were analyzed retrospectively. Two cases underwent CT scan, while 3 cases underwent MRI scan. Image observation included the tumor location, bone changes, morphology, density/signal characteristics, and enhanced features.ResultsThe four patients were all women who ranged in age from 25 to 74 years. Four cases of the lesions were in the vertebral body; among them, two cases involved the accessory of the vertebra. Soap bubble appearance was detected in 2 cases, honeycomb osteolytic appearance in 2 cases, a sclerotic margin in 3 cases, and accompanying vertebral compression fracture in 1 case. CT showed a slightly inhomogeneous low density with punctate high-density foci. MRI showed an inhomogeneous low signal on T1-weighted image (T1WI) and a high signal on T2WI. Contrast-enhanced CT/MRI demonstrated moderate to intensive inhomogeneous enhancement of the lesions. The discs were normal. In one case, lesions presented a dural tail sign.ConclusionsPrimary SEHE is so rare in the clinic as an aggressive vascular tumor. The relatively typical clinical features and radiographic findings can help in preoperative diagnosis.

Magnetic Resonance Imaging of Uncommon Hepatic Mesenchymal Tumours: Haemangioendothelioma and Angiosarcoma.

Primary Hepatic Epithelioid Haemangioendothelioma (HEHE) and Primary Hepatic Angiosarcoma (PHA) are rare mesenchymal tumours with different malignant potential. Whereas HEHE demonstrates low to intermediate malignant potential, PHA is an aggressive malignancy with poor prognosis. The knowledge of typical imaging features of these lesions may facilitate correct diagnosis; however, the ultimate diagnosis of HEHE and PHA is based on histopathologic examination. The most typical findings helpful in diagnosing HEHE are: Presence of multiple, confluent nodules located at the liver periphery (in young to middle-aged woman), retraction of the liver capsule, marked hyperintensity on T2-weighted images, "target-sign" appearance, progressive centripetal contrast enhancement, and relatively high Apparent Diffusion Coefficient (ADC) values. More than ≥50% of nodules are hyper- or isointense on Hepatobiliary Phase (HBP) images. The imaging features suggestive of PHA are: Occurrence of metastases (lungs, spleen) at the time of diagnosis, presence of a large dominant mass with smaller satellites, heterogeneity and areas of haemorrhage in a dominant mass, progressive contrast enhancement, slightly elevated ADC values as compared to other malignant liver tumours.

A CASE OF RADIOLOGICAL DIAGNOSIS OF EPITHELIOID HEMANGIOENDOTHELIOMA OF THE RIGHT ATRIUM

We present a case of a very rare heart tumor, a primary epithelioid hemangioendothelioma with secondary pulmonary metastases. Despite of the expanded diagnostic possibilities of current radiological diagnosis procedures, the final diagnosis in such patients is made only after histological examination and immunohistochemistry.

Primary pleural haemangioendothelioma in an Italian female patient: a case report and review of the literature

Primary epithelioid haemangioendothelioma (EHE) of the pleura is a rare vascular tumour that occurs mainly in men. Pleural effusion and thickening are the most common clinical presentations. A 58 year old female, nonsmoking patient presented to us with dry cough, dyspnoea and left chest pain for several weeks (no asbestos exposure). Standard chest X-ray and contrast enhanced multislice computed tomography revealed a large-size lobulated mass originating from the pleura which was diagnosed as primary pleural haemangioendothelioma (PHE) by histology and immunohistochemistry (reactivity for vimentin, CD31, CD34, Factor VIII and ulex europeaus). No metastases were detected. The patient refused treatment and died three months later due to the onset of acute and progressive respiratory failure. Despite the lack of high-grade malignancy, primary PHE displays a poor prognosis while curative therapies are actually not available. To our knowledge, this is the first case of primary PHE in a female patient occurring in Italy and the third one to have been reported in English literature. Difficulties in diagnosis and treatment management are discussed below.

肝切除を2回施行した肺転移を伴った肝類上皮血管内皮腫の1例

類上皮血管内皮腫（EHE）は血管内皮由来の比較的稀な低悪性度の非上皮性腫瘍である．今回，主病変および再発病変を手術にて切除しえた，肺転移を伴った肝原発EHEの1例を経験したので報告する．症例は61歳，男性．腹痛を主訴に受診し，多発肝腫瘍を指摘され，肝腫瘍生検にてEHEと診断された．両肺に多発肺転移を認めたが，増大傾向を認めなかったため，肝原発EHE（10個）に対して肝部分切除術（6カ所）を施行した．術後，単発の肝転移を認めた．多発肺転移は依然増大傾向を認めない状態であったため，肝転移に対して残肝右葉切除術を施行した．病理組織検査では，いずれの切除標本においても豊富な線維性間質を伴う異型を有する上皮様細胞の増殖を認め，免疫組織化学染色ではCD31，CD34陽性であり，EHEとして合致する所見であった．再肝切除から6カ月経過した現在，新たな再発の兆候を認めず，外来通院中である．

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.

Primary Epithelioid Hemangioendothelioma of the Kidney: The First Case Report in a Child and Literature Review

Epithelioid hemangioendothelioma is a distinct vascular tumor with low malignant biologic behavior that is very rare in the kidney. Only 3 adult patients have been reported to date in English-language reports. We report the case of a 9-year-old boy who presented with painless gross hematuria. Magnetic resonance imaging and positron emission tomography-computed tomography demonstrated a primary mass in the left kidney. The pathologic diagnosis of the tumor was epithelioid hemangioendothelioma after radical nephrectomy. To the best of our knowledge, this is the first reported case of primary renal epithelioid hemangioendothelioma in the pediatric age group.

Abstract No. 342 - Y-90 radioembolization of primary and metastatic soft tissue sarcomas of the liver

Purpose Soft tissue sarcomas (STS) are a group of uncommon neoplasms that are difficult to treat when unresectable. We present our initial data on Y-90 radioembolization (RE) of STS, both primary and metastatic to the liver. Materials and Methods Between November 2006 and June 2012, 11 patients with primary and metastatic STS involving the liver underwent 12 Y-90 RE treatments. Nine of 11 (81.8%) patients had prior unsuccessful treatments to control tumor progression in the liver including systemic chemotherapy, surgery, and locoregional therapies such as bland embolization or RFA. Eleven treatments were with resin and 1 treatment was with glass microspheres. Eight were whole liver treatments, and 4 were single lobar treatments. Sarcomas treated were 4 metastatic leiomyosarcomas (abdominal, retroperitoneal, uterine, and lower extremity in origin), 3 metastatic hemangiopericytomas, 1 metastatic prostatic rhabdomyosarcoma, 1 metastatic pleomorphic spindle cell sarcoma, 1 primary hepatic sarcoma, and 1 primary epithelioid hemangioendothelioma. Clinical and biochemical toxicities were recorded at baseline, 2 weeks, 4 weeks, and every 4 weeks thereafter. Radiographic response was recorded every 3 months. Results The median dose delivered was 1.68 GBq. The most common toxicities encountered were self-limited grade 1 fatigue in 6/11 (54.5%) and grade 1 abdominal pain in 4/11 (36.4%) patients. Follow up imaging was available in 11 cases. Partial (PR) or complete response (CR) was seen in 8/11 (72.7%) and 9/11 (81.8%) patients at 3 and 6 month imaging, respectively, according to mRECIST criteria. CRs were seen in 2 hemangiopericytomas, 1 uterine leiomyosarcoma, and 1 primary hepatic sarcoma. Median overall survival was 261 days with 5 (45.5%) patients surviving 491 days or longer. Two patients died within 50 days of treatment, though hepatic disease was not the cause of death in either patient. Conclusion Y-90 RE of advanced primary or metastatic STS of the liver appears to be a promising treatment option for patients who are poor surgical candidates or refractory to other salvage therapies.

A Primary Epithelioid Hemangioendothelioma of the Right Atrium: Report of a Case and Literature Review

Primary cardiac epithelioid hemangioendothelioma (EHE) is extremely uncommon. To our knowledge, only fourteen cases have been reported. We report a case of a primary EHE of the right atrium which incidentally detected in 77-year-old woman. Echocardiograhpy revealed a 26 × 16 mm right atrial mass attached to the base of the interatrial septum and moderate regurgitation of the tricuspid valve. The tumor and stalk were completely resected from the interatrial septum with a partial resection of the septal leaflet of the tricuspid valve. The patient had an uneventful postoperative course and no signs of recurrence nor metastasis of EHE 4 years after operation. Because of malignant potential of EHE, a longer period of close follow up is necessary.

Primary Renal Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma is a rare vascular tumor with intermediate biologic behavior and metastatic potential. Primary renal epithelioid hemangioendothelioma is extremely rare and we present the second report of this rare tumor in an interesting clinical scenario. A 59-year-old male with established history of widely metastatic high grade esophageal adenocarcinoma was found to have an isolated renal nodule on a followup computed tomography (CT) scan. Surgical excision, with the suspicion of metastatic carcinoma, and subsequent pathologic examination revealed an epithelioid hemangioendothelioma. The various differential diagnoses and use of morphological clues and immunohistochemistry are discussed.

Primary epithelioid hemangioendothelioma arising at vulva: A case report

Vulvar epithelioid hemangioendothelioma as primary site is extremely rare tumor that may be confused with other mesenchymal tumors due to their rarity and its histologic features. We report a case of 46-year-old woman who presented to hospital with left vulvar mass. The mass was located in subcutaneous area of vulva, and well marginated contrast enhanced on radiologic examination. Local excision was performed. After surgery, she has remained free of disease without recurrence for 6 months. Here we report a case of primary vulvar epithelioid hemangioendothelioma as very rare site and this is the first case in Korea.

Primary epithelioid hemangioendothelioma in the clival region: A case report and literature review

Epithelioid hemangioendothelioma (EHE) is a rare and low-grade vascular tumor, which usually occurs in the soft tissue, liver, breast, lung and skeleton. Here we submit a case with EHE of the clival region. A 58-year-old woman was admitted with a medical history of 3 months headache and 1 month visual deterioration. MRI revealed a well-circumscribed mass of 4.0 cm × 3.0 cm with bony invasion. The tumor was subtotally removed in a piecemeal fashion. Histologically, the tumor was composed of epithelioid cells with eosinophilic cytoplasm and intracytoplasmic vacuoles. Immunohistochemically, the tumor cells were positive for the markers CD31, CD34, factor VIII and vimentin. The pathological result was interpretated as EHE of the clival region. EHE is an uncommon vascular tumor, which is rarely seen in the clival region. Definitive diagnosis depends on histopathologic and immunohistochemical features.

Surgical resection of a primary pulmonary epithelioid hemangioendothelioma in bilateral lungs

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare pulmonary neoplasm that was initially described in 1975 as an intravascular bronchioloalveolar tumor. This report presents the case of a patient with multifocal primary pulmonary PEH (11 tumors) in the bilateral lungs. All of the tumors detected in the preoperative computed tomography scan were surgically resected. The patient has been doing well for 9 years after surgery. No tumor recurred for 8 years after surgery until a single recurrent nodule appeared and was thoracoscopically resected.

A rare case of primary cutaneous epithelioid haemangioendothelioma and review

A 73-year-old lady presented with a 6-month history of a violet dome-shaped rubbery, papular tender lesion on the calcaneum (Fig. 1). MRI showed no deep extension, and the lesion was excised and reconstructed with a fullthickness skin graft. Fibrous histiocytoma was diagnosed, and 4 months later was observed to have recurred. This was re-excised and defect was with a further full-thickness skin graft, and a new diagnosis of epithelial haemangioendothelioma (EHE) was made after specialist histological review. The surgical wound sloughed and broke down 2 months later, and a second recurrence of the tumour was detected. A bone scan and bone and blood profile, and CT chest, abdomen, and pelvis excluded underlying bone metastasis or systemic malignancy. The patient therefore proceeded to excision with a 1-cm margin, and reconstruction was performed using a Superficial Inferior Epigastric Artery (SIEA) free flap anastomosed to the posterior tibial vessels.

Epithelioid Hemangioendothelioma of the Lung Presenting With Pneumonia and Heart Rhythm Disturbances in a Teenage Girl

The epithelioid hemangioendothelioma (EHE) is a rare low-grade tumor of vascular origin that may arise at any site. However, lung and liver represent the 2 main locations. Symptoms of the pulmonary EHE are usually nonspecific and mild. Distant metastases of PEHE are frequent. However, heart metastases have only been reported in connection with primary EHE of the liver. We describe the case of a 15-year-old girl presenting with an abscess forming pneumonia and severe rhythm disturbances associated with an EHE of the lung. The untypical fulminant clinical course, the surgical interventions, and the involvement of the heart as a life threatening complication, eventually on the basis of cardiac metastases of PEHE, are emphasized.

Hemangioendothelioma of Urinary Bladder—Report of Rare Case

Although cavernous hemangiomas and a few cases of angiosarcoma in the urinary bladder have been described, only a single case of epithelioid hemangioendothelioma of the urinary bladder has been documented in English publications, to date. Therefore, the biologic properties of epithelioid hemangioendothelioma in this location are uncertain. We report the clinical and pathologic findings of a case of primary epithelioid hemangioendothelioma of the urinary bladder in a 17-years-old boy. The tumor was treated by transurethral resection.