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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.

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.

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.

Pulmonary epithelioid hemangioendothelioma is an uncommon lung malignancy of endothelial origin. Besides demonstrating unpredictable presentation features and prognosis, the paucity of established treatment guidelines remains a challenge in managing these patients. We present two patients. The first patient presented with chronic productive cough over 1-year duration. He was initially diagnosed and showed partial response to treatment for cardiac failure. A persistent right upper zone consolidation on chest radiograph prompted further investigations which revealed the diagnosis of pulmonary epithelioid hemangioendothelioma. The second patient presented with right-sided hemiparesis for 1-month duration. Initial computer tomography scan of the brain showed findings of distant metastatic foci. Subsequent investigations revealed pulmonary epithelioid hemangioendothelioma as the primary lesion. Both patients succumbed without any treatment due to rapid progression of the disease. We believe that pulmonary epithelioid hemangioendothelioma is undoubtedly rarely reported in south-east Asia region. In these two case reports, the patients were diagnosed in west and east Malaysia, respectively, in the same year (2015). Both cases highlight the increasing prevalence of pulmonary epithelioid hemangioendothelioma. We postulate that this could possibly be secondary to the advancement in diagnostic capabilities and improved healthcare facilities available in this region. Late presentation of pulmonary epithelioid hemangioendothelioma generally results in grave prognosis. Further investigations are required to elucidate the nature of progression and therapeutic options for patients with pulmonary epithelioid hemangioendothelioma.

Pulmonary Epithelioid Hemangioendothelioma is a rare and low grade tumor of endothelial origin found in the lungs. At onset patients are usually asymptomatic or present with non-specific symptoms. Chest imaging shows the presence of multiple, bilateral small nodules and diagnosis usually requires a lung biopsy. At this time there is no standardized treatment regimen and the prognosis is variable.

肺類上皮血管内皮腫（PEH）は稀な肺腫瘍の1つであり，かつて血管内細気管支肺胞上皮腫瘍と呼称されていた疾患である．PEHの多くは，画像上境界明瞭な多発小結節陰影を示すとされているが，今回我々は原発性肺癌と類似する画像所見を呈したPEH症例を経験したので，文献的考察を加えて報告する．症例は78歳，女性．咳嗽を主訴に近医を受診した．胸部CTで左S1+2に石灰化を伴う境界明瞭な15mm大の腫瘤影，右S9末梢に10mm大の境界不鮮明で胸膜陥入を伴う結節影を指摘された．FDG-PET検査で右下葉の結節にのみ強い集積を認め，原発性肺癌の疑いで胸腔鏡下肺部分切除を行った．術中迅速病理検査でPEHと診断され，手術を終了した．肺類上皮血管内皮腫は様々な陰影をとり得る可能性があり，両肺に多発結節陰影を認めた際は鑑別診断に上げる必要があると思われた．

肺類上皮性血管内皮腫は本邦での症例報告数が50例程度の稀な腫瘍である．われわれは肝転移を認めた肺類上皮性血管内皮腫の1切除例を経験したので報告する．症例は67歳，女性で他疾患経過観察中に胸部異常影を指摘され当院を受診した．胸部X線および胸部CTで右肺野に比較的境界明瞭な結節影を認めた．確定診断に至らず，胸腔鏡下肺部分切除術を施行．病理組織検査では，腫瘍細胞は肺胞内に充満するように存在し，空胞を有する異型細胞の増殖を認めた．免疫組織染色では血管内皮細胞マーカーであるCD31，CD34で腫瘍細胞が陽性となり肺類上皮性血管内皮腫の診断となった．その後早期に肝転移を生じ，肝部分切除術を施行した．外科的切除以外に有効な治療法が確立されておらず，胸腹部CT，脳MRIなどの画像検査を含め注意深い経過観察が必要である．

We report here a case of primary pulmonary epithelioid hemangioendothelioma diagnosed in a 67-year-old Caucasian man, presenting with exertion dyspnoea, dry cough, and multiple bilateral pulmonary nodules revealed by computed tomography. At the 18F-fluorodeoxyglucose positron emission tomography, these nodules were negative. The histopathological diagnosis was made on a pulmonary wedge resection (performed during video-thoracoscopic surgery).

In this report we describe a case of pulmonary epithelioid hemangioendothelioma (PEH) in a young woman. The neoplasm manifested with dry cough, chest pain, finger clubbing, and multiple bilateral pulmonary nodules on chest x-ray and computed tomographic (CT) scan. She underwent thoracoscopy, and the histological features of the lung biopsies were initially interpreted as consistent with a not-well-defined interstitial lung disease. Our patient was clinically and radiologically stable over a period of four years, after which the disease progressed to involve not only the lung but also mediastinal lymph nodes, liver and bone. Fiberoptic bronchoscopy showed subtotal occlusion of the right middle and lower lobe bronchi. The histologic examination of bronchial biopsies revealed a poorly differentiated neoplasm immunohistochemically positive for vimentin and vascular markers CD31, CD34 and Factor VIII. A diagnosis of malignant hemangioendothelioma was made. Positron emission tomography (PET) is more sensitive than CT scan and bone scintigraphy in detecting PEH metastases. Furthermore, 18-fluorodeoxyglucose (FDG) uptake seems to be related to the grade of malignancy of PEH lesions. Therefore, we suggest that FDG-PET should be included in the staging system and follow-up of PEH.