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No abstract available. Article truncated after first page. Clinical History: A 43 -year-old woman with no past medical history presented to the Emergency Room with complaints of right chest wall pain extending into the right upper quadrant. The patient was a non-smoker, denied any allergies, and was not taking any prescription medications. Physical examination showed the patient to be afebrile with normal heart and respiratory rates and blood pressure = 110/75 mmHg. Her room air oxygen saturation was 99%. The patient’s complete blood count and serum chemistries showed normal values. Her liver function testing and renal function testing parameters were also within normal limits. Which of the following represents an appropriate next step for the patient’s management? 1. Perform abdominal ultrasound 2. Perform chest radiography 3. Perform unenhanced chest CT 4. More than one of the above 5. None of the above …

Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor and infrequently described in medical literature as case reports and case series. Diagnosis is often incidental with high index of histopathological suspicion from clinical pathologist. The pathological pattern is quite unique with distinct immunohistochemical stains. Up to this day, there is no established standard treatment owing to the scarcity of this tumor. In this case report, we describe a case of pulmonary epithelioid hemangioendothelioma unexpectedly diagnosed with transthoracic needle biopsy, along with a review of the current literature.

Background Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare disease. Thus far, consensus on a standard treatment for P-EHE has not been established given its low incidence worldwide. Apatinib combined with chemotherapy with doxorubicin/cyclophosphamide has been used as an effective combination treatment for human malignancies. However, the efficacy of this combination has not been reported in P-EHE cases. Case summary We present the case of a 64-year-old woman with chest tightness, cough, and chest pain. Computed tomography showed multiple unresectable pulmonary nodules. She had been misdiagnosed with lung carcinoma and underwent gefitinib treatment at a hospital. Subsequently, the patient underwent a cardiothoracic surgery for further disease investigation. CD31, CD34, and Vimentin expression were detected in the resected nodule specimens by immunohistochemical analyses, and pathological analyses confirmed the diagnosis of P-EHE. Following this, four cycles of apatinib combined with chemotherapy with doxorubicin/cyclophosphamide were initiated. The patient demonstrated stabilization of multiple bilateral nodules and showed a dramatic improvement in the clinical presentation after combination treatment. The patient could not tolerate the side effects of chemotherapy. Therefore, she then continued apatinib monotherapy, which is ongoing to date. The patient was stable at the last follow-up after 24 mo. Conclusion Apatinib combined with chemotherapy with doxorubicin/cyclophosphamide may be an effective therapeutic option for P-EHE treatment.

Objective The aim of this study was to analyze the computed tomography (CT), fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT), and clinical findings of pulmonary epithelioid hemangioendothelioma. Methods Eighteen patients were included. We reviewed CT and PET/CT features and clinical information. Spearman correlation analysis was used to reveal the correlation between lesion size and maximum standardized uptake values (SUVmax). Results Eleven patients complained of different symptoms. The serum levels of tumor markers were elevated in 8 patients (44.4%). Multiple lesions were shown in 14 cases (77.8%). Eleven cases (61.1%) presented as bilateral multiple perivascular lesions. The size of the largest nodule was less than 20 mm in diameter in 11 cases (61.1%). Eleven cases underwent PET/CT examination, and 8 cases (72.7%) had increased FDG uptake. There was a positive correlation (Spearman correlation coefficient, 0.56; P = 0.009) between lesion size and SUVmax. Extrapulmonary involvement was shown in 7 cases, including the liver and bone. Conclusions Pulmonary epithelioid hemangioendothelioma on CT scan usually showed multiple bilateral perivascular lung nodules, generally measuring less than 20 mm in diameter. Lesions usually showed increased FDG uptake on PET/CT scans. There was a positive correlation between lesion size and SUVmax. The liver and bone were the most frequently involved extrapulmonary organs.

Pulmoner epithelioid hemangioendothelioma is a rare and multifocal malignant tumor of vascular origin.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor of low to intermediate malignancy, which originates from vascular endothelial cells. Most patients with PEH are asymptomatic and the tumor occurs most frequently in women. Typical radiologic images of patients with PEH are multiple irregular nodules with punctate calcification and pleural indentation. Here, we describe a 54-year-old woman who presented with multiple bilateral nodules of different sizes and well-defined borders, as well as lung markings, without punctate calcification or pleural indentation. These atypical computed tomography images resulted in misdiagnosis as metastatic lung cancer. Right upper lobe wedge resection was performed; intraoperative frozen pathologic examination suggested that the tumor was benign. However, immunohistochemical analysis revealed the presence of PEH. Subsequently, the patient chose watchful waiting, rather than chemotherapy. This rare case of PEH with atypical computed tomography findings, which was misdiagnosed as metastatic lung cancer, demonstrates that intraoperative frozen analysis is unreliable; thus, histopathological analysis is necessary.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of borderline or low-grade malignancy, Vascular endothelial growth factor (VEGF) and the VEGF receptor were found on PEH tumor cells, suggesting that target therapies that block VEGFR have a logical base in this rare malignancy. We report a 25 years old woman with a pulmonary hemangioendothelioma, treated with pazopanib, the only anti angiogenic registered agent for sarcoma, with clinical improvement of symptoms and durable stabilization for more than two years of lung tumor.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare neoplasm of vascular origin. There are three different major imaging patterns identified in thoracic manifestation of epithelioid hemangioendothelioma: (1) multiple pulmonary nodules; (2) multiple pulmonary reticulonodular opacities; and (3) diffuse infiltrative pleural thickening. Radiographically, presence of bilateral multiple nodules is the most common pattern of presentation. The diagnosis is made on the basis of histopathological findings and confirmed by positive immunohistochemistry staining. Although the prognostic factors for PEH have not yet been well established, a better prognosis is usually associated with the multinodular pattern. We report two different imagological presentations of this rare disease, based on two institutional experiences, along with a review of the relevant literature. info:eu-repo/semantics/publishedVersion

Rationale: Acute myocardial infarction is usually caused by coronary atherosclerotic plaque disruption (rupture or erosion), also including other uncommon etiologies. Pulmonary epithelioid hemangioendothelioma (PEH) is a rare low to intermediate malignant vascular tumor originating from vascular endothelial cells. Here, we report a rare case of acute ST-segment elevation myocardial infarction (STEMI) due to extrinsic compression of left coronary artery from PEH. Patient concerns: A 63-year-old woman with pulmonary nodules received left pulmonary nodulectomy, and the pathological examination indicated PEH. Five months after the pulmonary nodulectomy, the patient was admitted due to progressive dyspnea. Diagnosis: Electrocardiography showed the obvious ST-segment elevation in the leads I, aVL, and V1–3, and laboratory tests revealed the elevated level of cardiac troponin T. Emergent coronary angiography and the contrast-enhanced computed tomography scan conformed STEMI due to extrinsic compression of left coronary artery from PEH. Interventions: The patient did not undergo further therapy after the pulmonary nodulectomy. During the present hospitalization, she received basic life support and nutritional support treatment. Outcomes: The patient deteriorated rapidly into multi-organ failure and eventually died. Lessons: Acute STEMI could be caused by extrinsic compression of the coronary artery from the mass effects of PEH, and active therapy and close follow-up should be considered for patients with PEH.

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm which typically originates from liver, lung, or bone. Due to the low incidence of disease, the most effective treatment is not easily studied and much of the information known about EHE has been learned through case reports and case series. In this case, we will present an uncommon form of primary soft tissue EHE with local recurrence, bone metastasis, and lymphangitic spread to the lungs leading to respiratory failure. Imaging of the chest was atypical for EHE with intraseptal thickening and hilar lymphadenopathy. Respiratory failure was progressive despite aggressive multimodal treatment. This case highlights an unusually aggressive recurrence and metastasis of primary soft tissue EHE with atypical pulmonary imaging findings.

Pulmonary epithelioid hemangioendothelioma (PEHE) is a rare vascular tumor of endothelial origin first described in 1975 as intravascular bronchioloalveolar tumor. Since then, >100 cases have been reported, and most cases require surgical lung biopsy for diagnosis. We report the case of a 46-year-old man with a diagnosis of PEHE from endobronchial biopsies of an intraluminal nodule, a rare presentation of this disease. We summarize a review of the literature and the bronchoscopic findings of PEHE.

A 69-year-old male patient presented with multiple lung nodules revealed by chest-computed tomography (CT) during a preoperative examination for an appendiceal tumor. The nodule diameters ranged from 2-10 mm without either pleural thickening or effusions. A fluorine-18-labeled fluorodeoxyglucose (18F-FDG)-positron emission tomography (PET)/CT scan showed a high FDG uptake in the appendiceal tumor, but almost normal standardized uptake values in the bilateral lung nodules. A CT-guided biopsy led to a diagnosis of pulmonary epithelioid hemangioendothelioma, a rare vascular tumor with a radiological presentation similar to that of a metastatic lung tumor. The present case is the first to describe successful treatment using a CT-guided biopsy instead of more conventional methods.

Pulmonary Epithelioid Hemangioendothelioma (PEH) is one rather rare low-grade malignant tumor. Owing to its relatively low incidence, standard treatments on PEH are relatively less. A 52 y old male PEH patient treated with endostatin had received outstanding clinical outcome, considering his brain metastases and pleural effusion were both negative effects upon prognosis. Patient’s pathological characteristics, iconographic characteristics would play an essential role on his response to clinical treatment and prognosis. So far, standard treatments upon PEH are still not available, and the inhibition of tumor growth can be achieved through inhibition of tumor angiogenesis and interrupting cellular nutritious supply.

Pulmonary epithelial hemangioendothelioma is a rare low to intermediate malignant vascular tumor originating from vascular endothelial cells. The therapy for this disease, if possible, is surgical resection. However, there is no standard treatment for patients with multiple unresectable lesions. We present the case of a 42-year-old woman treated with a natural clinical course of hemangioendothelioma for four years without therapy. The nodules have increased in number and size extremely slowly, and the patient is alive and asymptomatic four years after diagnosis.

El hemangioendotelioma epitelial es una neoplasia poco frecuente, y tiene su origen en el endotelio vascular. Puede crecer en varios órganos y tejidos. No hay ningún esquema terapéutico aprobado y el pronóstico es impredecible. Se presenta el caso de un paciente masculino de 54 años de edad, con el diagnóstico de hemangioendotelioma epitelioide pulmonar, con imagen atípica y la presentación clínica y la participación de múltiples órganos en el momento del diagnóstico. El paciente no comenzó la quimioterapia y murió dos meses después del diagnóstico histológico. Debido a la rareza de esta neoplasia y la inexistencia de régimen terapéutico aprobado, al reportar este caso, tenemos la intención de contribuir a los estudios clínicos futuros.

Rationale: Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare tumor, with no established standard treatment. Overexpression of vascular endothelial growth factor receptor 2 (VEGFR-2) has been reported in some P-EHE patients. Apatinib, a new small molecule tyrosine kinase inhibitor that specifically targets VEGFR-2, has therapeutic benefits in some advanced tumors. However, its efficacy in P-EHE cases has not been reported. Patient concerns: Herein, we presented a 44-year-old man with recurrent hemoptysis for approximately 9 years. Diagnoses: After hospitalization, relevant examinations were conducted. The disease was subsequently diagnosed as P-EHE. Interventions: The patient underwent pulmonary lobectomy, but subsequently developed multiple metastases. Within the tumor, CD31, CK, and Vimentin were found to be positive, while CD34 was negative. Apatinib was initially administered 250 mg daily doses and after 1 month was increased to 500 mg daily. Outcomes: He showed noticeable symptomatic improvements and positive imaging changes in the first month of treatment. However, the disease progressed in the following month, despite the increased apatinib dose. Lessons: Apatinib is possibly a new treatment for P-EHE. However, further clinical trials are necessary to confirm an effective dose and the efficacy and safety of apatinib in P-EHE treatment.

Epithelioid hemangioendothelioma is a very rare, vascular, low‐grade malignant tumor found in the lungs, liver, bone, and other soft tissues. Most patients with pulmonary epithelioid hemangioendothelioma (PEH) are asymptomatic but usually present with multiple bilateral nodular lesions in the lungs. Currently, surgical lung biopsy, histology, and immunohistochemical methods are essential for diagnosis. However, there is no standard therapy for the treatment for PEH. Our paper describes the clinico‐radiologic features and genomics of PEH based on next‐generation sequencing (NGS) in a 43‐year‐old male we encountered. The patient came to the hospital with right chest pain. After investigation, a lesion in the middle lobe of the right lung was found, together with smaller multiple lesions in both lungs. After resection of the lesion, histopathological analysis showed positive findings for PEH. The patient's blood and tumor tissue were sent for NGS analysis for further investigation. Results from the analysis revealed mutations of multiple genes. The information obtained from the genomic analysis of PEH using NGS may be significant for the planning and monitoring of treatment for this disease.

Pulmonary epithelioid hemangioendothelioma is a rare pulmonary neoplasm. No reliably effective systemic chemotherapy for this disease entity has been established yet. Surgery is usually applied for patients with a small number of lesions limited to one lung, while feasibility/efficacy/safety of this modality is controversial for patients with bilateral multiple lung lesions. Presence of cases showing rapid aggravation and those showing very gradual progression makes it difficult to evaluate validity of surgery as a suitable therapeutic approach. Herein, we present a summary on pulmonary epithelioid hemangioendothelioma and discuss the role of surgery in the treatment of this condition.

This study aimed to investigate the clinicopathological characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendothelioma (PEH). PEH is a rare low-grade malignant vascular tumor. The cause of PEH remains unclear. Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis. The clinical manifestations, imaging findings, histopathological characteristics and immunohistochemical phenotypes of four cases of epithelioid hemangioendothelioma occurring in the lung were retrospectively analyzed, and a review of the associated literature was conducted. The age of onset for the four PEH cases was 25-54 years, and the disease manifested as multiple nodules in the lungs or pleura. All of the patients underwent lobectomy or pulmonary wedge resection. The morphology of the tumor cells was epithelioid or spindle-shaped with abundant eosinophilic cytoplasm in which lumina or vacuoles containing erythrocytes were observed. The cells were arranged in nests and cords with degenerated interstitial mucoid. The morphology of the majority of the tumor cells was moderate, including mild atypia and little mitosis or necrosis. Immunohistochemical staining showed positive results for CD31, CD34 and F8. PEH is a rare low- to moderate-level tumor occurring in the lungs with differentiation toward vascular endothelial cells. Clinically, it is difficult to distinguish from a variety of other benign and malignant lung diseases. For diagnosis, a distinction must be made from other diseases such as chronic granulomatous disease, amyloid nodules, hamartoma, primary and metastatic lung cancers, malignant mesothelioma and vascular sarcoma. In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed. The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

No standard therapy for pulmonary epithelioid hemangioendothelioma (PEH) has yet been established due to the rarity of the disease, the lack of clear standards for treatment and the partial-to-complete spontaneous regression. This report describes three cases of PHE manifested as bilateral intrapulmonary masses with an initial diagnosis conducted by thoracoscopic lung biopsy. These patients demonstrated a partial response to combination chemotherapy with carboplatin, paclitaxel, bevacizumab or endostar, and an improvement in clinical status. Furthermore, we reviewed the literature regarding such patients who received chemotherapy and immunotherapy; this indicated that patients with PEH demonstrated a good partial response to chemotherapy with carboplatin, paclitaxel, bevacizumab, thalidomide and α-interferon. Overall, combination chemotherapy regimens may hold therapeutic potential for the treatment of this rare disease.