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Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with malignant biological behavior. It arises from endothelial cells, usually within soft tissues, and can occur in almost all locations.
We report a unique case of a 25-year-old man who presented with sudden attacks of severe back pain followed by acute non-traumatic paraplegia. Emerg...
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Context 1
... to a recurrent secondary hemorrhage, a total spinal tumor resection via transthoracic T7 vertebrectomy with VB replacement by cage interposition was performed. The extirpation of some pleural foci and pulmonary lesions in the left lower lobe was conducted during the same operation via thoracic surgical intervention ( fig. 2). After this procedure, clinical improvements were achieved, but a considerable amount of blood was lost through the continuous pleural drainage. Due to new deterioration of the clinical status, a transthoracic and dorsal reoperation was done in order to seal the pleural ...Citations
... It is commonly seen in soft tissues but can also be in other organs, such as the lung, pleura, vessels, heart, liver, and bone [5]. Primary EHE of the bones and spines is rare and accounts for only 1% of all malignant bone tumors [6,7]. According to the World Health Organization's classification of bone tumors, EHE is defined as a low-to-medium-grade malignant neoplasm that may involve the long bones of the limbs and axial skeleton, as well as the phalanges of the hands and feet [8]. ...
We report a 48-year-old male patient with spinal epithelioid hemangioendothelioma in T3 and T4 who began with symptoms of paresthesia in the lower limbs and distal weakness of the right lower limb, back pain, increased limitation in walking, urinary incontinence, and constipation. A safe maximum resection was performed, finding residual disease during the PET/CT scan, so it was decided to treat with radiotherapy, and there was a good response to this treatment. A literature review of epithelioid hemangioendothelioma of the thoracic spine was done which showed a mean age of presentation of 41 years and a male-female ratio of 1:0.53. The main symptom was pain, which was present in 100% of the patients, and wide surgery was performed in 56.8% of the patients, intralesional surgery in 31.8%, and biopsy in 11.4%. A total of 46.6% of patients received radiation therapy, and only 6.6% received chemotherapy. The patients had an average follow-up of 38 months. We recommend that extension studies such as PET/CT scans be performed after surgical resection. This can serve as a follow-up with hemangioendothelioma epithelioma patients about metastatic disease or residual disease that will guide us in giving adjuvant treatments, such as radiotherapy or chemotherapy, for better control of the disease.
... No single chemotherapy regimen has been found to be 100% successful, and the chemotherapy methods employed are based on a succession of EHEs involving many locations. In numerous documented case series, anthracyclines were the most commonly used first-line chemotherapeutic agents [24][25][26]. A series of 29 patients with multicentric or localized bone EHE was published in 1986, with a follow-up of 33 months after surgery or adjuvant radiotherapy. ...
... Primary spinal EHE is a rare type of malignancy, accounting for only 1% of all malignant bone tumors (6). EHE is characterized by polygonal or round epithelioid endothelial cells with abundant eosinophilic hyaline cytoplasm and vesicular nuclei. ...
Background
Epithelioid hemangioendothelioma (EHE) is an extremely uncommon malignant neoplasm that originates from vascular endothelial or pre-endothelial cells. In this report, we present the case of patient who was diagnosed with a primary giant EHE of the spine and underwent treatment with total en-bloc spondylectomy (TES).
Case presentation
A 43-year-old male patient with a history of he presented to our hospital with chronic and progressive back pain. Physical examination revealed weakened sensation of acupuncture and touch on the left costal arch, while relatively normal neurological functions were preserved. Radiological examinations identified a giant destructive soft tissue lesion occupying the T8 vertebral region, with moderate destruction of the pedicle and lamina, as well as the 7th left rib. A preoperative biopsy of the 8th vertebra resulted in a diagnosis of epithelioid hemangioendothelioma(EHE). Postoperative immunohistochemical and pathological reports confirmed the presence of EHE in the left ribs and T8 ribs. The patient underwent resection of the 7th left rib and posterior pedicle screw fixation with 8 pairs of screws and a titanium mesh cage. Subsequently, thoracic en bloc spondylectomy was performed on the T8 vertebra. The patient did not receive radiation or chemotherapy following surgery. Over a period of 3 years, the patient remained free of disease and relapse.
Conclusion
The use of transarterial embolization with spherical embolic agents (TES) has been demonstrated to be a safe, effective, and reliable treatment option for hepatic epithelioid hemangioendothelioma (EHE). Nevertheless, it is crucial to conduct long-term follow-up of this patient in order to assess their clinical outcome.
... [1] On radiographs and Computed Tomography (CT), EH of bone presents as a multifocal or multicentric locally aggressive expansile, lytic lesion with cortical destruction and a soft tissue component. [1,5] On magnetic resonance imaging (MRI), the signal intensity characteristics are non-specific. e lesion appears hypo-to-intermediate signal intensity on T1-weighted images and hyperintense signal intensity on T2-weighted images and shows restriction on diffusion-weighted imaging. ...
... e lesion appears hypo-to-intermediate signal intensity on T1-weighted images and hyperintense signal intensity on T2-weighted images and shows restriction on diffusion-weighted imaging. [5] To the best of our knowledge, this is a rare case of multicentric EH found in proximal humerus and scapula, having an unusual feature of matrix calcifications, which posed difficulty in arriving at a definitive diagnosis, thereby leading us to consider various other bone tumors in the differential diagnosis. As a result, the final diagnosis was confirmed only on histopathology. ...
... Matrix mineralization and periosteal reaction are usually absent. [1,5] e presence of matrix calcification is a very unusual appearance and has been described only in very few cases. [6] On MRI, it appears as a hypointense lesion on T1-weighted images and iso to hyperintense on T2-weighted images with homogenous enhancement on post-contrast images. ...
Epithelioid hemangioendothelioma (EH) is a rare malignant vascular tumor occurring mainly in the liver and lungs, with bones being a rare site and primarily seen in the adult population. We present a rare case of multicentric EH in the right humerus in an adolescent male, who presented with complaints of pain and swelling in the right shoulder. Radiographs and Computed tomography showed a large expansile lytic lesion in the proximal end of the right humerus with areas of cortical destruction and matrix calcification. On Magnetic resonance imaging, the lesion was predominantly isointense on T1-weighted image, hyperintense on T2-weighted, and Short Tau Inversion Recovery images with prominent flow voids within. On post-contrast sequences, the lesion showed intense heterogenous enhancement with a non-enhancing central necrotic area. Multiple smaller lytic lesions with similar imaging characteristics were also found in the mid and distal shaft of the right humerus and the right coracoid process. Differentials of Telangiectatic osteosarcoma, Giant cell tumor, brown tumors of hyperparathyroidism, and metastasis were considered. Core biopsy revealed that the lesion was an EH. Though the imaging features of EH are non-specific, it may be considered in the differential diagnosis of an expansile lytic bone lesion with no periosteal reaction, showing cortical break and soft tissue component, especially if it is multifocal and multicentric.
... According to previous reports, EHE exhibits an intermediate biological behavior between angiosarcoma and hemangioma. The reported recurrence rate is approximately 11% and metastasis occurs in 2.7% of patients [2][3][4] . ...
Background:
Epithelioid hemangioendothelioma (EHE) is an uncommon low-grade aggressive vascular tumor. It can occur in almost all locations, but is rarely encountered in bone.
Case summary:
We report a 23-year-old man who presented with left hip pain with no obvious cause. X-ray revealed bone destruction in the left femoral neck with sclerosis at the edges of the lesions. Magnetic resonance imaging (MRI) showed bone destruction in the medullary cavity of the left femoral head and neck. 18F-deoxyglucose-positron emission tomography/computed tomography (PET/CT) imaging showed bone destruction in the left ischium, acetabulum, and left femoral head neck, accompanied by increased radioactive uptake; the maximum standard uptake value was 4.2. Histopathologic examination revealed spindle-shaped mesenchymal tissue hyperplasia with scattered epithelioid cells. The patient underwent left femoral head replacement surgery. No signs of recurrence were observed as of the 18-mo follow-up.
Conclusion:
The definitive diagnosis of femoral EHE can be established aided by the MRI and PET/CT findings.
... The most common sites of involvement are the liver (21% of patients), bone (14%), and lung (12%) [3,6]. However, EHE has been reported to involve many other sites throughout the body [9][10][11][12][13][14]. When the tumor is seen in multiple sites simultaneously, it is difficult to Imaging Features of EHE sue sampling [2]. ...
... It is more prevalent in males and male-to-female ratio is 2:1 [6] . But Kerry et al. [5] reported that the men and women were affected in roughly equal numbers as well. According to Weissferdt et al. [7] , the overall survival for those EHE patients with unicentric tumor was 89% compared with 50% in patients with multifocal diseases. ...
... The formation of multicellular infantile angiogenic sprouts and lumens are noted in the tumor [5 ,9] . The distinct well-lined anastomosing vascular channels often seen in hemangiopericytoma are absent in this tumor [5] . Microscopically, the tumor cells are round, polygonal or fusiform with a central nucleus, and prominent intracytoplasmic vacuolation [10] . ...
... Tumor cells are arranged in cords and nests, which are embedded within a myxohyaline stroma. In immunohistochemistry, the positivity of endothelial cell markers CD34, CD31, and factor VIII-related antigen is specific for the diagnosis of EHE [5] . ...
Primary epithelioid hemangioendothelioma of the spine is the extremely rare malignant vascular neoplasm with an unpredictable outcome. A case of epithelioid hemangioendothelioma with multiple lytic lesions of thoracolumbar spine and other bones in a 29-year-old male patient is reported. A review of the published data regarding this rare neoplasm is also presented. The features of epithelioid hemangioendothelioma include the occurrence in the young male patient, multiple osteolytic lesions with thin sclerotic rim and hypermetabolic activities. However, its imaging features are not specific. Positron emission tomography/computed tomography is essential for identification of the lesions and subsequent follow-up for treatment.
... The most common sites of involvement are the liver (21% of patients), bone (14%), and lung (12%) [3,6]. However, EHE has been reported to involve many other sites throughout the body [9][10][11][12][13][14]. When the tumor is seen in multiple sites simultaneously, it is difficult to sue sampling [2]. ...
OBJECTIVE. The purpose of this article is to review the spectrum of imaging manifestations of epithelioid hemangioendothelioma across different organ systems and briefly describe its current treatment strategies. CONCLUSION. Epithelioid hemangioendothelioma is a rare, locally invasive neoplasm with metastatic potential. Although most commonly occurring in liver, lungs, and bones, it can also present at multiple other sites. Because of its nonspecific clinical and imaging manifestations, it is often misdiagnosed. The possibility of epithelioid hemangioendothelioma must be considered in the presence of a slowly growing mass that invades adjacent structures. Imaging can help plan percutaneous biopsy, detect sites of disease, and identify poor prognostic factors.
... Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with endothelial differentiation 1 and is described as of intermediate malignancy between that of hemangiomas and angiosarcomas. 2 The tumor may arise in a wide variety of locations. Pulmonary, hepatic, osseous, splenic, cardiac, intracranial and other manifestations 1,3,4 have been reported in the literature. EHE accounts for less than 1% of primary malignant bone tumors. 1 Primary vertebral manifestations are considerably less common. ...
... EHE accounts for less than 1% of primary malignant bone tumors. 1 Primary vertebral manifestations are considerably less common. Most of the vertebral manifestations involve the thoracic and lumbar regions; the cervical spine is less commonly affected. ...
... Moreover, EHE can show an osteoblastic growth pattern, which increases the range of conditions to be included in differential diagnosis. 1,3,[16][17][18] Initially, EHE is often clinically inapparent and therefore tends to be detected at an advanced stage. Back pain is the most common first symptom. ...
2019): Multifocal and hormone-dependent epithelioid hemangioendothelioma with osteolysis of the second cervical vertebral body: report of an unprecedented surgical approach by using autologous bone graft, British Journal of Neurosurgery, ABSTRACT We report the case of a 28-year-old female patient who complained of extreme neck pain when giving birth to a child. Magnetic resonance imaging (MRI) of the cervical spine demonstrated an osteolytic lesion at the second cervical vertebral body (C2). In this presentation, we highlight a transoral surgical approach in order to prevent instability of this osteolytic lesion. To the best of our knowledge, this is the first time that such a route of access has been described for this tumor entity. A histopathologic examination led to the diagnosis of epithelioid hemangioendothelioma. During a follow-up period of 33 months, the patient had no complaints. ARTICLE HISTORY
... It can occur in almost any location but is mainly reported in soft tissues [5]. The most common locations are long bones of the lower extremities [6][7][8][9]. OHE was previously known as angiolymphoid hyperplasia with eosinophilia or histiocytoid hemangioma, which is a rare vascular tumor with a biological behavior between hemangiosarcoma and hemangioma [10]. Few studies have reported on the risk factors of hemangioendotheliomas (HEs) in bones. ...
Background
Osseous malignant vascular tumors (OMVTs) are rare lesions. Moreover, the prognostic determinants of OMVTs have not been reported. This study aimed to present epidemiological data and analyze the prognostic factors of survival in OMVT patients.
Material/Methods
OMVT patients who were diagnosed between 1973 and 2015 were screened using the Surveillance, Epidemiology, and End Results (SEER) program database, with special attention paid to osseous hemangiosarcoma (OAS) and osseous hemangioendothelioma (OHE). We assessed the prognostic values of cancer-specific survival (CSS) and overall survival (OS) rates with a Cox proportional hazards regression model and univariate and multivariate analyses. OS and CSS curves were obtained using the Kaplan-Meier method.
Results
A total of 202 cases were selected from the SEER database. The specific histopathological diagnoses were osseous hemangiosarcoma (n=127) and osseous hemangioendothelioma (n=75). Among OMVT patients, histology was an important factor in determining survival. Using multivariate analysis, old age, distant tumor stage, surgery, and low tumor grade were predictors of OS for OAS patients. Old age, surgery, and low tumor grade were predictors of CSS. Using multivariate analysis, old age and surgery were predictors of OS and CSS for OHE patients.
Conclusions
This study is the largest population-based study to show the demographic characteristics and analyze the prognosis of OMVT patients. Independent predictors of OS for patients with AS included old age, distant tumor stage, low tumor grade, and surgery. Old age, surgery, and low tumor grade were also predictors of CSS for patients with OAS. Independent predictors of CSS and OS for patients with OHE included old age and surgery.
