ABSTRACT
Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of borderline or low-grade malignancy, and its prognosis is unpredictable. Herein, we describe the case of a 47-year-old asymptomatic female with a diagnosis of multinodular PEH. During a 7-year follow-up, the nodules with large size and high 18F-fluorodeoxyglucose uptake in the initial study showed progression with increasing sizes; however, most small nodules (size < 1 cm) demonstrated spontaneous regression with peripheral rim or nodular calcification. The patient underwent surgical resection for an enlarged nodule. Of note, it is unusual for an individual to have mixed progression and regression concomitantly, which may be helpful in predicting the prognosis.
ABSTRACT
Hepatic epithelioid hemangioendothelioma (HEHE) is a vascular tumor with a low incidence rate. We report a case of a 26-year-old man who was referred to our hospital with a misdiagnosis of liver cirrhosis. On physical examination, ascites was noted. Chest and abdominal computer tomography scans showed coalescent lesions involving the peripheral liver with heterogeneous contrast enhancement and portal vein dilation due to portal hypertension. Extrahepatic metastasis was not observed. The biopsy with immunohistochemical stains suggested HEHE (Factor VIII, CD31, and CD34). This report describes an uncommon case of HEHE with non-cirrhotic portal hypertension.
El hemangioendotelioma epitelioide hepático (HEHE) es un tumor vascular con una tasa de incidencia baja. Presentamos el caso de un varón de 26 años que fue remitido a nuestro hospital con un diagnóstico erróneo de cirrosis hepática. En el examen físico se observó ascitis. La tomografía computarizada de tórax y abdomen mostró lesiones coalescentes que afectaban al hígado periférico con realce heterogéneo de contraste y dilatación de la vena porta por hipertensión portal. No se observó metástasis extrahepática. La biopsia con tinciones inmunohistoquímicas sugirió HEHE (Factor VIII, CD31 y CD34). Este informe describe un caso poco común de HEHE con hipertensión portal no cirrótica.
ABSTRACT
Objective To study the use of abdominal enhanced CT imaging and quantitative index analysis in the differential diagnosis of hepatic epithelioid hemangioendothelioma (HEH) and hepatic metastasis.Methods A study group of 12 patients with HEH who underwent abdominal enhanced CT scanning at the First Affiliated Hospital of Zhengzhou University from February 2014 to October 2018 was retrospectively compared with a control group of 52 patients with hepatic metastases diagnosed clinically and by imaging examinations.The general information and imaging data of these patients were collected and analyzed.Results The lesions in the 2 groups mainly presented as multiple and diffuse lesions.The diffuse lesions of HEH often fused into strips.The hepatic metastasis group showed a higher CT attenuation and TNR in the portal vein phase than the HEH group (P ＜ 0.05).The area under the ROC curves of the two indexes were 0.756 and 0.841 respectively.The centers of the lesions showed almost no or slightly homogeneous enhancement in the HEH group,while the liver metastasis group showed slightly and moderately heterogeneous enhancement,with a significant difference between the two groups (P ＜ 0.05).Female,subcapsular distribution,capsular contraction,target ring sign and lollipop sign were independent risk factors for HEH (P ＜0.05),while a high CT attenuation and TNR in the portal vein phase,elevated tumor markers and lymph node metastasis were independent risk factors for liver metastasis on logistic regression analysis (P ＜ 0.05).Conclusions CT attenuation,TNR,central enhancement features in the portal vein phase,special signs and secondary changes of lesions were helpful for the differential diagnosis between HEH and liver metastasis.
ABSTRACT
Objective: To study the clinicopathological features, diagnosis, and differential diagnosis of atypical epithelioid hemangioendothelioma (EHE). Methods: Eight cases of atypical EHEs were collected from Jiangsu Province Hospital (the First Affiliated Hospital of Nanjing Medical University) between 2010 and 2018. EnVision method and fluorescence in situ hybridization (FISH) were used to detect immunophenotype, WWTR1-CAMTA1 and TFE3 gene rearrangement, respectively. Results: There were 4 males and 4 females, ranging from 42 to 59 years (median 47.5 years). The tumors located in soft tissue (3 cases), lung (3 cases), liver (1 case) and chest wall (1 case). One soft tissue EHE involved also adjacent fibula and pleural involvement was present in all three lung cases at the diagnosis. Regional lymph node metastases were present in two cases (one involving soft tissue tumor and one involving liver). Morphologically, the tumor cells were epithelioid with abundant eosinophilic cytoplasm, moderate to marked nuclear pleomorphism, irregular nuclear membrane, unevenly chromatin, and prominent nucleoli. The cells arranged in cords, small nests or solid pattern. The mitotic rate was 4.3 mitoses/2 mm(2) on average (ranging 2 to 9). Tumor necrosis was seen in every case. Among all 8 cases, blister cells were found upon careful observation. Myxohyaline stroma was present in 6 cases. Immunohistochemically, tumor cells expressed CD31 (8/8), CD34 (7/8), ERG (8/8), CKpan (2/7), and CAMTA1 (4/6). None of the tested cases stained for TFE3 (0/6). WWTR1-CAMTA1 fusion gene by FISH was found in all tested 6 cases and TFE3 gene rearrangement was not detected in any. Available clinical follow-up was obtained in 7 cases and the intervals range from 6 to 55 months (average 19.6 months). Six patients had metastasis and 3 patients died of disease. One patient was alive with no evidence of disease. Conclusions: Atypical EHE is a more aggressive tumor than classic EHE, with histological features including high nuclear grade, increased mitotic activity, the presence of solid growth pattern and tumor necrosis. The differential diagnoses include epithelioid angiosarcoma, carcinoma and epithelioid sarcoma.
Subject(s)
Hemangioendothelioma, Epithelioid , Neoplasms, Vascular Tissue , Adult , Biomarkers, Tumor , Calcium-Binding Proteins , Female , Humans , In Situ Hybridization, Fluorescence , Male , Middle Aged , Trans-ActivatorsABSTRACT
Hemangioendothelioma epithelioid is a rare tumor that originates in soft tissues. Imaging evaluation with conventional modalities (tomography and magnetic resonance) is difficult. Novel radiotracers which capably evaluate angiogenesis may have a higher impact on the therapeutic decisions. A 45-year-old man underwent workup for thrombosis and was diagnosed with hemangioendothelioma epithelioid based on the results of liver pathology and immunohistochemistry. The decision of the multidisciplinary board was to begin with thalidomide. After 4 months, progression of disease was documented and right hepatectomy was performed. A 68Ga-DOTA-E-[c(RGDfK)]2 positron emission tomography-computed tomography scan showed residual lesions. After documented angiogenesis by 68Ga-DOTA-E-[c(RGDfK)]2 positron emission tomography-computed tomography, nintedanib was administrated. And 1 year later, progression of the disease was documented by positron emission tomography-computed tomography. Ipilimumab plus nivolumab was started and partial response and excellent clinical response were documented. Molecular imaging with 68Ga-DOTA-E-[c(RGDfK)]2 positron emission tomography-computed tomography is a good biomarker of the response of hemangioendothelioma epithelioid, and ipilimumab plus nivolumab therapy demonstrated a good response.
ABSTRACT
Objective@#To study the clinicopathological features, diagnosis, and differential diagnosis of atypical epithelioid hemangioendothelioma (EHE).@*Methods@#Eight cases of atypical EHEs were collected from Jiangsu Province Hospital (the First Affiliated Hospital of Nanjing Medical University) between 2010 and 2018. EnVision method and fluorescence in situ hybridization (FISH) were used to detect immunophenotype, WWTR1-CAMTA1 and TFE3 gene rearrangement, respectively.@*Results@#There were 4 males and 4 females, ranging from 42 to 59 years (median 47.5 years). The tumors located in soft tissue (3 cases), lung (3 cases), liver (1 case) and chest wall (1 case). One soft tissue EHE involved also adjacent fibula and pleural involvement was present in all three lung cases at the diagnosis. Regional lymph node metastases were present in two cases (one involving soft tissue tumor and one involving liver). Morphologically, the tumor cells were epithelioid with abundant eosinophilic cytoplasm, moderate to marked nuclear pleomorphism, irregular nuclear membrane, unevenly chromatin, and prominent nucleoli. The cells arranged in cords, small nests or solid pattern. The mitotic rate was 4.3 mitoses/2 mm2 on average (ranging 2 to 9). Tumor necrosis was seen in every case. Among all 8 cases, blister cells were found upon careful observation. Myxohyaline stroma was present in 6 cases. Immunohistochemically, tumor cells expressed CD31 (8/8), CD34 (7/8), ERG (8/8), CKpan (2/7), and CAMTA1 (4/6). None of the tested cases stained for TFE3 (0/6). WWTR1-CAMTA1 fusion gene by FISH was found in all tested 6 cases and TFE3 gene rearrangement was not detected in any. Available clinical follow-up was obtained in 7 cases and the intervals range from 6 to 55 months (average 19.6 months). Six patients had metastasis and 3 patients died of disease. One patient was alive with no evidence of disease.@*Conclusions@#Atypical EHE is a more aggressive tumor than classic EHE, with histological features including high nuclear grade, increased mitotic activity, the presence of solid growth pattern and tumor necrosis. The differential diagnoses include epithelioid angiosarcoma, carcinoma and epithelioid sarcoma.
ABSTRACT
- Epithelioid hemangioendothelioma is a rare vascular brain tumor. It develops from endothelial cells, usually in the liver, lung, bone and soft tissue. Primary localization of this tumor in the intracranial space is very uncommon; only 47 cases have been described in the literature. This tumor was initially classified as grade I (benign) in the World Health Organization (WHO) 2007 classification. In 2016, this tumor was re-classified as grade III (malignant). Herein, the first case report of epithelioid hemangioendothelioma in the cerebellum of a male patient is presented. Complete surgical excision was done. No adjuvant therapy was administered. Magnetic resonance imaging performed 2 years after the surgery continued to show no recurrence of the tumor. To our knowledge, this is the first report of cerebellar location of this rare tumor. In addition, the authors report drastic re-classification of the epithelioid hemangioendothelioma from the benign tumor (WHO 2007) to a malignant one (2016), which significantly changes postoperative management and follow up of this brain neoplasm.
Subject(s)
Brain Neoplasms , Cerebellum , Hemangioendothelioma, Epithelioid , Neurosurgical Procedures/methods , Adult , Brain Neoplasms/pathology , Brain Neoplasms/physiopathology , Brain Neoplasms/surgery , Cerebellum/pathology , Cerebellum/surgery , Dissection/methods , Follow-Up Studies , Hemangioendothelioma, Epithelioid/pathology , Hemangioendothelioma, Epithelioid/physiopathology , Hemangioendothelioma, Epithelioid/surgery , Humans , Magnetic Resonance Imaging/methods , Male , Treatment OutcomeABSTRACT
ObjectiveTo investigate the clinicopathological features of hepatic epithelioid hemangioendothelioma (HEHE). MethodsThe clinical data, histopathological data, and immunohistochemical results of 5 patients with HEHE were observed. ResultsThere were 3 male and 2 female patients. Major clinical manifestations included persistent abdominal discomfort, abdominal pain, nausea, loss of weight, and pyrexia. One patient was found to have this disease during physical examination. Of all patients, 2 had a single nodule and 3 had multiple nodules. The tumor cells were relatively normal in morphology and were arranged in a cord-like shape or a small nested shape; the cells had a round, spindle-like, or irregular shape; the cells were rich in epithelioid eosinophilic cytoplasm, with branched cytoplasmic processes and cavity formation in cytoplasm. The tumor background was characteristic mucoid hyaline degeneration of cartilage-like matrix or glassy degeneration of fibrous matrix. Nuclear division and necrosis were observed occasionally. Vascular markers, such as CD31, CD34, Fli-1, coagulation factor VIII, and vascular endothelial growth factor, were expressed in tumor cells, and tumor cells tended to have a low proliferative activity. ConclusionHEHE has various clinical manifestations and treatment methods. Liver biopsy helps to make a confirmed diagnosis and select a proper treatment regimen before treatment.
ABSTRACT
Objective To analyze the imaging characteristics of contrast-enhanced ultrasound(CEUS) and dynamic contrast enhanced magnetic resonance imaging ( CEMRI) for diagnosis of hepatic epithelioid hemangioendothelioma ,and to increase the accuracy of diagnostic imaging . Methods The imaging characteristics of CEUS and dynamic CEMRI in 11 patients with hepatic epithelioid hemangioendothelioma confirmed by surgical pathology or fine needle aspiration biopsy were retrospectively analyzed . Results On conventional ultrasound ,14 lesions in 11 patients showed hypoechoic . The peripheral blood flow was detected in most patients by color Doppler flow imaging and the average resistant index was 0 .62 ± 0 .05 . During the arterial phase ,6 lesions showed diffuse enhancement ,6 lesions showed annular enhancement and 2 lesions showed branch enhancement .As to time of peak enhancement ,compared to the normal hepatic parenchyma ,hypoenhancement ,isoenhancement and hyperenhancement were observed in 1 lesion ,7 lesions and 6 lesions respectively . Synchronous enhancement was observed in 8 lesions on contrast-enhanced ultrasound ,rapid enhancement was observed in 5 lesions and slow enhancement was observed in 1 lesion and all lesions decreased quickly in portal phase and delayed phase . On CEMRI ,all lesions showed low signal on TIWI ,high signal on T2WI and DWI . There were 5 lesions showed obvious annular constant enhancement and 1 lesion showed obvious annular progressive enhancement . Progressive peripheral enhancement and fully filling in delayed phase were found in 4 lesions .While progressive peripheral enhancement and incompletely filling in delayed phase were found in 3 lesions . One lesion displayed constant enhancement and incompletely filling in delayed phase . Halo sign" , lollipopsign" , capsular retraction sign" and vessel sign" were appeared in 6 ,7 ,8 and 8 patients respectively . Conclusions Both contrast-enhanced ultrasound and CEMRI have characteristic manifestations for the diagnosis of hepatic epithelioid hemangioendothelioma and cominbing them would be great significance to improve the preoperative diagnostic accuracy .
ABSTRACT
Purpose To analyze and compare the imaging characteristics of contrastenhanced ultrasound (CEUS) and dynamic contrast-enhanced CT for hepatic epithelioid hemangioendothelioma (HEHE) diagnosis.Materials and Methods The imaging characteristics of CEUS and dynamic contrast-enhanced CT performed on 16 patients with pathological-confirmed HEHE were retrospectively analyzed.Dynamic contrast-enhanced CT was performed on 5 patients,real-time CEUS was performed on 8 patients,and both examinations were performed on 3 patients.Results On CEUS,14 lesions were selected from 11 HEHE cases.In the case of enhancement mode,5 lesions displayed ringenhancement,and 9 lesions displayed global enhancement;in the case of arterial phase,11 lesions were synchronously enhanced,and 3 lesions were rapidly enhanced compared with liver parenchyma;in the case of peak time,hyper-enhancement,iso-enhancement and hypo-enhancement were observed in 2 lesions,9 lesions and 3 lesions,respectively;all 14 lesions manifested hypoechoic mass in portal phase and delayed phase.On dynamic contrast-enhanced CT,29 lesions were found in 8 HEHE cases.In the case of arterial phase,9 lesions (31.0％) showed slight inhomogeneous enhancement,4 lesions (13.8％) showed no obvious enhancement and 16 (55.2％) lesions showed slight edge enhancement;all 29 lesions presented continuous hypo-enhancement in delayed phase.Conclusion Both CEUS and dynamic contrast-enhanced CT have respective characteristic manifestations,and are of high value for the diagnosis of HEHE.
ABSTRACT
Epithelioid hemangioendothelioma (EHE) is a well-differentiated and rare vascular tumor. Systemic metastases are uncommon. Herein, we present a patient with skin metastasis of pulmonary EHE (PEH) that was treated by wide excision. A 76-year-old male was evaluated due to pulmonary thromboembolism and a solitary pulmonary nodule. A biopsy was performed and pathological examination of the mass confirmed EHE. No metastasis was observed. The patient returned to care approximately two years later due to a painful nodule in the right lower leg. A skin biopsy showed metastatic EHE from the lung. We used a safety margin of 1 cm based on clinical experience, because no prior case had been reported regarding the resection margin appropriate for primary cutaneous EHE and skin metastases of PEH. At four months after surgery, the patient recovered without complications or recurrence. Skin metastasis of PEH is extremely rare, and only two cases have been reported in the literature. In this case, we report a rare case of PEH with histologically diagnosed skin metastasis that was successfully treated by curative resection. It is expected that this case report will provide a helpful contribution to the extant data regarding PEH metastases.
ABSTRACT
Objective To investigate the value of contrast enhanced ultrasonography ( CEUS) in the diagnosis of hepatic epithelioid hemangioendothelioma( HEHE) . Methods The images of CEUS had been retrospectively evaluated in 21 lesions of HEHE which were confirmed by pathology . The perfusion patterns and time of enhancement were observed ,including time to begin enhancement , time to peak enhancement ,the washout time and the appearance of internal structures . Results The mean time of begin enhancement ,time to peak ,time to isoechogenity and hypoechogenity were ( 17 .4 ± 5 .2) s ,( 22 .0 ± 7 .2) s , ( 23 .9 ± 4 .6 ) s and ( 42 .2 ± 13 .7 ) s ,respectively . During the arterial phase , 8 lesions showed diffuse enhancement ,7 lesions showed rim-like enhancement and 6 lesions showed branch enhancement . The inner margin in 6 lesions showed speculate enhancement . The central portion of the tumors was not enhanced in part lesions of 8 cases . As to time of peak enhancement ,compared to the normal hepatic parenchyma , hypoenhancement ,isoenhancement and hyperenhancement were observed in 10 lesions ,6 lesions and 5 lesions respectively . Synchronous enhancement was observed in 11 lesions on CEUS ,rapid enhancement was observed in 8 lesions and slow enhancement was observed in 2 lesions . Twenty-one lesions manifested hypoechoic mass in portal phase and delayed phase . The boundary of lesions was unclear on conventional ultrasound and became distinct after contrast injection in 21 lesions . Conclusions CEUS is useful for the diagnosis of HEHE .
ABSTRACT
Epithelioid hemangioendothelioma (EHE) is a well-differentiated and rare vascular tumor. Systemic metastases are uncommon. Herein, we present a patient with skin metastasis of pulmonary EHE (PEH) that was treated by wide excision. A 76-year-old male was evaluated due to pulmonary thromboembolism and a solitary pulmonary nodule. A biopsy was performed and pathological examination of the mass confirmed EHE. No metastasis was observed. The patient returned to care approximately two years later due to a painful nodule in the right lower leg. A skin biopsy showed metastatic EHE from the lung. We used a safety margin of 1 cm based on clinical experience, because no prior case had been reported regarding the resection margin appropriate for primary cutaneous EHE and skin metastases of PEH. At four months after surgery, the patient recovered without complications or recurrence. Skin metastasis of PEH is extremely rare, and only two cases have been reported in the literature. In this case, we report a rare case of PEH with histologically diagnosed skin metastasis that was successfully treated by curative resection. It is expected that this case report will provide a helpful contribution to the extant data regarding PEH metastases.
Subject(s)
Aged , Humans , Male , Biopsy , Dermatologic Surgical Procedures , Hemangioendothelioma, Epithelioid , Leg , Lung , Neoplasm Metastasis , Pulmonary Embolism , Recurrence , Skin , Solitary Pulmonary NoduleABSTRACT
A young female patient presenting with recurrent hemoptysis, neck swelling, and mediastinal mass mimicking lymphadenopathy was admitted to the Institute of Post Graduate Medical Education and Research and SSKM hospital, Kolkata, India. Clinical features, radiological studies, fibre optic bronchoscopy, and fine needle aspiration cytology from the neck swelling created a diagnostic dilemma until surgical resection and immunohistochemistry reports confirmed the diagnosis of multifocal epithelioid hemangioendothelioma, a rare vascular tumor with intermediate malignancy potential. Because it is a slow-progressing disease and due to the non-availability of standard chemotherapy, the patient, and her legal guardian, opted for palliative care only. She was asymptomatic for four years but again presented with hemoptysis, reappearance of the neck swelling on the same side, and a mediastinal mass compressing the superior vena cava and right pulmonary artery. This report describes the diagnostic problems and therapeutic challenges in the management of this rare tumor over a four-year follow-up period. The clinical course emphasizes the highly unpredictable nature of this tumor.
ABSTRACT
Epithelioid hemangioendothelioma (EHE) of the bone is extremely uncommon and consists of less than 1% of primary bone tumors. It is characterized by epithelioid endothelial cells and has variable biological behavior. EHE is more likely to occur between 20 and 30 years of age. Approximately half of EHE present with multifocal disease. Since the behavior of these tumors is intermediate, it is important to not misdiagnose EHE as an angiosarcoma. Here we describe the case of a 43-year-old male who presented with pain and swelling of the leg and ankle to Dr. Ram Manohar Lohia Hospital, India. Radiography and computed tomography of the ankle and leg were performed and suggested an expansile lytic lesion involving the distal tibia and talus bone. The lesion was excised and ankle arthrodesis performed. Histological evaluation of the lesion demonstrated the presence of EHE of the tibia and talus.
ABSTRACT
We report on a 41-year-old man with multiple epithelioid hemangioendothelioma of the left foot and ankle. The patient was treated with curettage and fixation with 2 separate plates, but later developed local infection. Owing to the potential for malignancy, below-knee amputation was suggested, but the patient declined and was treated symptomatically with close monitoring. The patient underwent removal of the plates, excision of the distal fourth of the fibula, intralesional curettage of all lesions, and fusion of the ankle and subtalar joints with a retrograde intramedullary nail. After 5.5 years, the patient had recovered well and had no evidence of malignancy. The tumour was considered definitively benign.
Subject(s)
Bone Neoplasms/diagnosis , Fibula , Hemangioendothelioma, Epithelioid/diagnosis , Tarsal Bones , Adult , Biopsy , Diagnosis, Differential , Humans , Magnetic Resonance Imaging , Male , Tomography, X-Ray ComputedABSTRACT
Objective To investigate the clinical and pathological features of primary hepaticepithelioid hemangio endothelioma (EHE) and try to improve the understanding and diagnosis of this disease.Methods From January 2007 to January 2013,the data of 17 patients with hepatic EHE were retrospectively analyzed,which included clinical,pathological and radiological characteristics,treatment and survival condition of these patients.Results Among 17 cases of hepatic EHE,there were 11 female and six male with the mean age of 46.2 years.Seven patients had right upper quadrant pain of dull pain in liver and the left 10 cases were without any symptoms.The liver function of most patients (15 cases) was normal.The admission diagnosis was mostly misdiagnosed as hepatocellular carcinoma (metastatic liver carcinoma six cases,primary liver carcinoma four cases).Extrahepatic metastasis was found in three patients before operation.The major lesion of the tumor was multiple nodules (10 cases) and most of which were under liver capsule.Retraction of the liver capsule was found in the imaging examination.The features of pathology were intracellular lumen and vacuoles formed in small cells,in which single or couple red blood cells could be found.Nuclear deviation was found in single tumor cell which looked like signet ring cells.The results of immunohistochemistry indicated the positive rate of factor vimentin (4/5) antigen and CD34 (15/17) was high.Surgical resection (14 cases) was the main treatment of hepatic EHE.Fourteen patients were still alive followed up for two to 72 months after operation.Conclusions The incidence of hepatic EHE is low,and it was susceptible to middle aged women and without obvious symptom.No obvious abnormality is found in laboratory examination.Surgical resection is the main treatment which enabled a good prognosis.
Subject(s)
Hemangioendothelioma, Epithelioid/pathology , Liver Neoplasms/pathology , Adult , Antigens, CD34/metabolism , Female , Hemangioendothelioma, Epithelioid/diagnostic imaging , Hemangioendothelioma, Epithelioid/metabolism , Humans , Immunohistochemistry , Liver Neoplasms/diagnostic imaging , Liver Neoplasms/metabolism , Magnetic Resonance Imaging , Platelet Endothelial Cell Adhesion Molecule-1/metabolism , Positron-Emission Tomography , Tomography, X-Ray Computed , von Willebrand Factor/metabolismABSTRACT
No abstract available.
