Pulmonary Epithelioid Hemangioendothelioma Research Articles

Imaging features and deep learning for prediction of pulmonary epithelioid hemangioendothelioma in CT images.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumour, and its early diagnosis remains challenging. This study aims to comprehensively analyse the imaging features of PEH and develop a model for predicting PEH. Retrospective and pooled analyses of imaging findings were performed in PEH patients at our center (n=25) and in published cases (n=71), respectively. Relevant computed tomography (CT) images were extracted and used to build a deep learning model for PEH identification and differentiation from other diseases. In this study, bilateral multiple nodules/masses (n=19) appeared to be more common with most nodules less than 2 cm. In addition to the common types and features, the pattern of mixed type (n=4) and isolated nodules (n=4), punctate calcifications (5/25) and lymph node enlargement were also observed (10/25). The presence of pleural effusion is associated with a poor prognosis in PEH. The deep learning model, with an area under the receiver operating characteristic curve (AUC) of 0.71 [95% confidence interval (CI): 0.69-0.72], has a differentiation accuracy of 100% and 74% for the training and test sets respectively. This study confirmed the heterogeneity of the imaging findings in PEH and showed several previously undescribed types and features. The current deep learning model based on CT has potential for clinical application and needs to be further explored in the future.

Pulmonary Epithelioid Hemangioendothelioma: A Case Report of Sleeve Resection of this Rare Lung Tumour

Reporting a 37 year old male with pulmonary epithelioid hemangioendothelioma (PEH), whom had undergone a sleeve resection of main left bronchus en bloc with the tumour. Presented with shortness of breath & was treated as pneumonia with left lung collapse. Bronchoscopy showed lesion at left main bronchus & unable to pass the obstruction. CT scan showed left main bronchus endobronchial lesion & histopathological reported it as suspected PEH. Mass had regrown 90% stenosis within 2 weeks. PET scan showed solitary disease at left endobronchial region. Left thoracotomy with sleeve resection of left bronchus en bloc with the tumour done. Intraoperative bronchoscopy showed endobronchial tumour located at the main left bronchus 1cm from carina. Histopathology confirmed the resected lesions are PEH. Patient recovered well. PEH is a histological diagnosis, recent reviews showed that radiological CT findings can be associated. No standard guideline treatment for PEH. There are cases reported with good response to combine chemotherapy and immunotherapy. Unilateral lesions, surgery is indicated. If lesion is positive for oestrogen and progesterone receptors, studies showed that hormonal therapy may benefit. Antivascular endothelial growth factor reported as part of treatment. Prognosis is unpredictable. In view of the rarity of the disease, multi-modulated approach is essential in the management and follow-up of the patient.

A case of early recurrence of pulmonary epithelioid hemangioendothelioma

症例は60歳代男性．検診の胸部X線写真で左下肺野の結節影を指摘され，精査のCTで左肺下葉S10に2.1 cm大の不整形結節影を認めた．気管支鏡による確定診断は困難と判断し，手術による確定診断の方針とした．病変を含めた肺部分切除術を施行し，免疫組織化学染色で肺類上皮血管内皮腫（pulmonary epithelioid hemangioendothelioma：PEH）の診断を得た．単発例ながら術後早期に多発肺転移・多発骨転移をきたしたが集学的治療を行い，病勢コントロールを得られた．本症では多発症例や再発症例に対し放射線照射，化学療法，ステロイド療法などの奏効例も見られるが確立された治療方法はなく，急速な増悪を認めた場合には治療に難渋することが多いとされ，厳重な経過観察が必要である．

Compression of the Pulmonary Artery and Coronary Artery Caused by Pulmonary Epithelioid Hemangioendothelioma: A Case Report.

A 63-year-old woman with a five-month history of pulmonary epithelioid hemangioendothelioma (PEH) presented to the emergency department, due to worsening dyspnea and chest pain. The electrocardiography showed a pattern of ST-segment elevation in leads I, AVL, and poor R-wave progression consistent with anterolateral ischemia. Emergent coronary angiography revealed severe stenosis of the left main coronary artery. Then, contrast-enhanced computed tomography scan indicated the right pulmonary artery and left main coronary artery narrowing by compression of metastasized PEH. Finally, the patient died of deteriorated multi-organ failure.

Poor response to sintilimab plus chemotherapy in a pulmonary epithelioid hemangioendothelioma patient: a case report.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor with no established treatment protocol. The authors report the case of a young woman diagnosed with PEH. DNA and RNA analysis by next-generation sequencing was performed on the tumor tissue. A novel germline PALB2 mutation and classicalWWTR1-CAMTA1 fusion were identified. She experienced a poor response to sintilimab (a PD-1 inhibitor) plus platinum-based chemotherapy as the first-line treatment. PEH patients harboring a germline PALB2 mutation and WWTR1-CAMTA1 gene fusion may respond poorly to treatment with PD-1 inhibitors plus chemotherapy.

Delayed metastatic pulmonary epithelioid hemangioendothelioma of the femoral vessels: case report and literature review

Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of the blood vessels. We report a patient with vascular EHE with delayed pulmonary metastasis, of which there are no previously known case reports. A 40-year-old female presents with a painful right groin mass and swelling in the lower extremity. A 3.8 cm soft tissue mass was identified in the femoral sheath with the abutment of the femoral artery on a computerized tomography (CT) scan. Surgical resection of the femoral vein was performed, and the final pathology confirmed a diagnosis of EHE. A second en-bloc resection of the femoral artery with bypass grafting was performed with clear surgical margins. The patient did well post-operatively with annual surveillance of the right groin as well as chest X-rays. However, the patient developed a metastatic Epithelioid hemangioendothelioma (EHE) to the right lower lobe, 12 years after the initial EHE treatment. She underwent a pulmonary resection with clear margins. The patient remains disease-free after one year. EHE is a rare soft tissue sarcoma with unpredictable clinical behavior. While most commonly presenting in the lung and liver they can also originate from any vascular system. Delayed pulmonary metastasis from vascular EHE has not been reported. Our case shows that indolent metastasis can occur in EHE, despite a prolonged disease-free interval. This case highlights the need for long-term surveillance with serial imaging of not only the primary site but pulmonary imaging beyond 5 years may be beneficial.

Rapid death due to pulmonary epithelioid haemangioendothelioma in several weeks: A case report

A 49-year-old woman was admitted to our hospital because of haemoptysis for 6 days. This patient claimed no medical history except high blood sugar. Chest computed tomography (CT) showed infection and multiple nodules on both sides of the lung. Blood tests showed no obvious abnormalities. Tracheoscopy showed haemorrhagic discharge in the left upper lobe and an old thrombus obstructing the lumen in the anterior basal segment of the right lower lobe. Then, CT-guided percutaneous lung biopsy was performed. The pathological results suggested multiple nodular-like lesions in the submitted tissues, and tumour cells were round or short fusiform, forming a solid nest structure, visible mitosis, and a vascular cavity-like structure containing red blood cells. Immunohistochemistry revealed positive staining for Vimentin, Bcl-2, CD31, and CD34; negative staining for CD68, SMA, CR, and D2-40; and 40% Ki67+ positivity. Based on the earlier data, the patient was diagnosed with pulmonary epithelioid haemangioendothelioma. This patient did not receive any treatment for several reasons. Unfortunately, the patient died 8 weeks after diagnosis. In conclusion, we present a case featuring the rapid death due to PEH.

Watch the Mime Carefully! A Refractory Interstitial Lung Disease.

Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of a vascular origin which can arise in different locations such as the lungs, liver, soft tissue, and rarely, in the bones. In the lungs, pulmonary hemangioendothelioma (PEH) shows a variable clinical behavior, displaying a range from either an asymptomatic course to a highly aggressive progression with metastases. Based on radiological features, PEH differential diagnosis mainly includes primary or metastatic lymphangitic carcinomatosis, granulomatous infections, and diffuse interstitial lung diseases where ground glass pattern predominates. In this case, a transbronchial biopsy and subsequent histological and immunohistochemical analysis allowed for the attribution of the scenario to a pulmonary epithelioid hemangioendothelioma. Clinicians should always consider bronchoscopy as a useful and effective tool to better investigate indeterminate and questionable clinical pictures, sparing patients the morbidity and mortality associated with more invasive techniques such as surgical or thoracoscopic biopsy.

Clinical analysis of 16 cases of pulmonary epithelioid hemangioendothelioma

Objective: To improve the diagnosis and treatment of pulmonary epithelioid hemangioendothelioma (P-EHE). Methods: Sixteen patients diagnosed with P-EHE in Fuzhou Pulmonary Hospital of Fujian Province from January 2009 to July 2020 were collected. Their gender, age, imaging findings, pathological characteristics, treatment protocols, survival and other clinical data were summarized and analyzed. Results: The ratio of male to female among the 16 patients was 1∶1; and the average age of onset was 47.75 years. Most cases of PEHE (9/16) were found by physical examination, while some cases developed respiratory symptoms such as cough, sputum, shortness of breath, hemoptysis, chest pain, etc. (7/16). In most patients the lesions were localized to the lungs (11/16), while bone metastasis (1/16), lymph node metastasis (1/16), and pleural metastasis (4/16) also occurred. The pathological tissues were obtained mainly through surgical thoracoscopy. Chest CT images showed multiple nodules in both lungs, with most of the nodules less than 2.0 cm in diameter, and calcifications were seen, while solitary nodules and masses were rare, and pleural metastases could be manifested as pleural thickening and pleural effusion. The pathological findings were well-defined eosinophilic nodules with irregularly arranged nest-like structures. Those eosinophilic nodules had few central cells and abundant peripheral cells, which extended into the alveolar cavity like papillae. The tumor cells were epithelioid with small atypia, and vacuoles and red blood cells could be seen in the cytoplasm of individual tumor cells. Immunohistochemically, the tumor cells were positive to CD34, CD31, Factor Ⅷ andvimentin (VIM). Follow-up of 0.5 to 11 years showed that four patients died, two lost to follow-up, and the rest of the patients were in good condition, with a median overall survival (OS) of 4.58 years. Conclusions: PEHE is a rare low-grade lung tumor with no specific clinical manifestations. It can be diagnosed with chest imaging and pathological immunohistochemistry. Moreover, there is currently no standard treatment for PEHE, and most patients have a good prognosis.

PULMONARY EPITHELIOID HEMANGIOENDOTHELIOMA: A RARE ENTITY WITH INSIDIOUS ONSET

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Sarcomas are rare, malignant tumors of connective tissue origin. Epithelioid hemangioendothelioma (EHE) is an exceedingly rare vascular sarcoma of endothelial origin, with an incidence of less than one per one million. Patients are typically asymptomatic or manifest symptoms related to organ involvement. We present a case of an asymptomatic woman with an incidental discovery of pulmonary EHE. CASE PRESENTATION: A 26 year old woman with no smoking history presented with vague abdominal pain. Her exam and initial labs were unremarkable. Abdominal imaging revealed nodules at the lung bases, and subsequent thoracic imaging revealed innumerable nodules throughout the lung parenchyma without pleural involvement. CT-guided transthoracic biopsy revealed filling and distension of alveolar spaces by a neoplastic proliferation of small, bland cells with perinuclear vacuoles within a hyalinized stroma. Immunohistochemical staining showed the neoplastic cells to be positive for ERG and CD31. These imaging and pathology findings were consistent with stage IV metastatic pulmonary EHE. She was referred to a sarcoma center of excellence, and surveillance was recommended. DISCUSSION: EHE is a vascular subtype of sarcoma of low-grade malignancy most commonly involving the liver and lungs. Pulmonary EHE can be asymptomatic or present with non-specific symptoms of dyspnea, cough, hemoptysis, or weight loss. Diagnosis is made with histopathologic epithelioid morphology and immunohistochemical vascular characteristics. Our patient's biopsy tissue stained positive for markers of vascular differentiation. CD34 and Factor VIII-related antigen are additional markers that may be used to identify vascular features of EHE. Disease course is unpredictable, and factors associated with a poor prognosis include pleural effusion and hemoptysis. Disease rarity precludes established treatment guidelines. Surgical resection should be considered for solitary lesions. Past reports have shown some responsiveness to taxane, anthracycline, or IFN-2a therapy. Anti-VEGF therapies show promise for eliciting partial response or as maintenance therapy. Still, many cases are indolent and a surveillance strategy may be reasonable. In our patient, surveillance was supported by her asymptomatic disease without poor prognostic factors. The development of significant symptoms or progressive disease would prompt further consideration of treatment. CONCLUSIONS: EHE is an extremely rare sarcoma subtype most commonly affecting the liver or lungs. Diagnosis is made on immunohistopathology and treatment options include surgical resection, surveillance, or anti-angiogenic agents. It is an important, albeit uncommon diagnosis to consider for diffuse pulmonary nodules seen on imaging. REFERENCE #1: Semenisty V, Naroditsky I, Keidar Z, Bar-Sela G. Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma—a suitable treatment option: case report and review of anti-angiogenic treatment options. BMC Cancer. 2015;15(1):402. doi:10.1186/s12885-015-1395-6 REFERENCE #2: Chevreau C, Le Cesne A, Ray-Coquard I, et al. Sorafenib in patients with progressive epithelioid hemangioendothelioma: A phase 2 study by the French Sarcoma Group (GSF/GETO). Cancer. 2013;119(14):2639-2644. doi:10.1002/cncr.28109 REFERENCE #3: Agulnik M, Yarber JL, Okuno SH, et al. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Ann Oncol. 2013;24(1):257-263. doi:10.1093/annonc/mds237 DISCLOSURES: No relevant relationships by Mohammad Ahmed, source=Web Response No relevant relationships by Gustavo Cumbo-Nacheli, source=Web Response No relevant relationships by Connor Kerndt, source=Web Response No relevant relationships by Ranuka Sinniah, source=Web Response

A CASE REPORT OF HYPERTROPHIC OSTEOARTHROPATHY LEADING TO DIAGNOSIS OF PULMONARY EPITHELIOID HEMANGIOENDOTHELIOMA

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Hypertrophic Osteoarthropathy (HOA) is a condition characterized by abnormal proliferation of skin and osseous structures of extremities. Periostitis is the imaging hallmark of HOA and most commonly manifests symmetrically along the shaft of tubular bones. Incidence of HOA in patients with intrathoracic malignancy is 5-10%. CASE PRESENTATION: A 49-year-old male presented to his primary care physician for bilateral knee pain with associated swelling. After failing conservative management, bilateral knee x-rays were performed which revealed a thin periosteal reaction in both femoral shafts, prepatellar soft tissue swelling and small bilateral joint effusions.The radiologist recommended a chest x-ray for further evaluation, which showed subtle soft tissue fullness at the right lung base. Chest CT was then performed revealing a 4.2 x 2.2 x 4.7 cm spiculated soft tissue lung mass of the right lower lobe with extension into the mediastinum and right hilar, paratracheal and subcarinal lymph nodes.The patient underwent bronchoscopy with FNA of the right lower lobe mass and station 7 and 11 lymph nodes. Highly atypical cells suspicious for malignancy were noted, but analysis was limited by scant material. IR guided biopsy was subsequently performed revealing patchy proliferation of epithelioid cells with ample eosinophilic cytoplasm, occasional cytoplasmic vacuoles and necrosis. Immunohistochemically, the tumor cells stained positive for ERG, CD31, and CD24. The cytomorphologic and immunohistochemical features support the diagnosis of epithelioid hemangioendothelioma (EHE). DISCUSSION: This case illustrates EHE's heterogeneous presentations. The primary lesion of EHE has been documented in numerous locations throughout the body, but most commonly arises in the liver, lungs, and bones. HOA as the presenting sign in pulmonary EHE is not well documented in the literature and periosteal reaction is considered rare. Digital clubbing was not present in this case and the only known location of periosteal reaction was the femoral shafts. While the mechanism of EHE is not well understood, it is believed that angiogenic stimulators, such as vascular endothelial growth factor (VEGF), may act as promoters of endothelial cell proliferation.The proposed pathogenesis of HOA has been linked to hypersecretion of vasoactive agents such as VEGF, platelet derived growth factor (PDGF), and prostaglandin E2 (PGE2). The proposed pathogenesis of secondary HOA has been linked to hypersecretion of vasoactive agents such as VEGF, PDGF, and PGE2. These agents are released both systemically in response to hypoxemia and by the tumor itself. CONCLUSIONS: Pulmonary Epithelioid Hemangioendothelioma can also present with Hypertrophic Osteoarthropathy.Bone X- ray can lead to the diagnosis of Hypertrophic Osteoarthropathy if careful evaluation is done. Periostitis is frequently a sign of pulmonary and systemic pathology. REFERENCE #1: Chakraborty, Rebanta K. "Secondary Hypertrophic Osteoarthropathy." StatPearls [Internet]., U.S. National Library of Medicine, 1 Mar. 2021, www.ncbi.nlm.nih.gov/books/NBK513342/. REFERENCE #2: Sardaro, Angela, et al. "Epithelioid Hemangioendothelioma: an Overview and Update on a Rare Vascular Tumor." Oncology Reviews, PAGEPress Publications, Pavia, Italy, 13 Oct. 2014, www.ncbi.nlm.nih.gov/pmc/articles/PMC4419652/. REFERENCE #3: Silveira LH;Martínez-Lavín M;Pineda C;Fonseca MC;Navarro C;Nava A; "Vascular Endothelial Growth Factor and Hypertrophic Osteoarthropathy." Clinical and Experimental Rheumatology, U.S. National Library of Medicine, pubmed.ncbi.nlm.nih.gov/10728444/. DISCLOSURES: No relevant relationships by Julia Lawinger, source=Web Response No relevant relationships by Raul Mendoza-Ayala, source=Web Response

Pulmonary Epithelioid Hemangioendothelioma: Imaging and Clinical Features.

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. 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). 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. 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.

A Case of a Pulmonary Epithelioid Hemangioendothelioma Presenting with Multiple Nodular Shadows

A Case of a Pulmonary Epithelioid Hemangioendothelioma Presenting with Multiple Nodular Shadows

PRIMARY PULMONARY EPITHELIOID HEMANGIO-ENDOTHELIOMA WITH PLEURAL INVOLVEMENT: A CASE REPORT

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Epithelioid Hemangio-Endothelioma (EHE) is a rare low to intermediate grade malignant vascular tumor with metastatic potential [1]. Here, we present a case of primary Pulmonary EHE with pleural involvement and lymph node metastasis. CASE PRESENTATION: A 52-year old female, non-smoker presented to our ED 2 months ago with right lower rib cage pain and dyspnea. CTA chest at that time showed findings consistent with RUL consolidation and trace right sided pleural effusion [Fig.1]. She received total of 15 days of antibiotics which did not improve the symptoms and subsequently presented to ED 20 days later with worsening pain and dyspnea. Repeat CT chest showed persistent RUL consolidation with increase in effusion size. Thoracentesis was performed twice, and pleural fluid analyses revealed a exudative effusion with some atypical cells and negative flow cytometry on two occasions. Patient underwent transbronchial biopsy which was negative for malignancy. PET-CT demonstrated right perihilar upper lobe mass-like area along with right paratracheal lymph node involvement [Fig.2,3]. Due to high suspicion for malignancy, patient underwent CT guided transthoracic biopsy. Pathology showed malignancy with EHE that was confirmed with pathology lab from University of Michigan. Patient was discharged to follow up at Cleveland Clinic for further staging and management and is currently under evaluation for starting on chemotherapy. DISCUSSION: EHE has a low prevalence with only a little over 100 cases reported since it was first described in 1975 [2]. Theoretically, it can develop in any tissue but occurs primarily in the soft tissues, liver and rarely in the lung [2]. Pulmonary EHE (PEHE) presents as either a solitary nodule or more often as multiple small nodules [2,3]. Most patients require surgical biopsy for histopathological confirmation. Histopathologically, EHE is characterized by the features of epithelioid cells, but originates from vascular endothelial cells. [2] This is evidenced by the presence of some or all of the vascular endothelial markers (CD31, CD34 and factor VIII) on immunohistochemistry [4]. The prognosis is very unpredictable, with life expectancy ranging from 1-15 years [5]. However, presence of respiratory symptoms and pleural effusion at diagnosis is associated with worst prognosis, with a median survival of less than 1 year [6]. CONCLUSIONS: PEHE has a variable presentation, clinical course, and imaging appearance. Its rarity adds to the challenge of diagnosis. PEHE should be considered in the differentials when the patient presents with pulmonary manifestations and there is no apparent diagnosis found. Reference #1: Travis WD, Brambilla E, Nicholson AG, et al. The 2015 World Health Organization Classification of Lung Tumors: impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol Reference #2: Dail D, Liebow A. Intravascular bronchioloalveolar tumor. Am J Pathol 1975;78:A6–7. 2015;10:1243–60. Reference #3: Weiss SW, Enzinger F. Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma. Cancer 1982;50:970–81. DISCLOSURES: No relevant relationships by Salil Avasthi, source=Web Response No relevant relationships by DEEPTI AVASTHI, source=Web Response No relevant relationships by Upneet Chawla, source=Web Response No relevant relationships by Rathnavali Katragadda, source=Web Response No relevant relationships by Leela Krishna Vamsee Miriyala, source=Web Response

PULMONARY EPITHELIOID HEMANGIOENDOTHELIOMA: CASE REPORT

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: We present a case of a 29-year-old female with an insidious clinical presentation. At first, it was thought to be an infectious disease, but it turned out to be a very rare neoplasm. CASE PRESENTATION: 29-year-old female with healthcare-related tuberculosis exposure for working as a nurse in an emergency service and homecare. She had a 2-month history of non-productive cough and progressive dyspnea, progressing from exertion to small effort dyspnea. She visited an emergency service where a chest x-ray was obtained. It showed multiple, diffuse, bilateral, micronodular radiopacities, therefore, being referred to a Respiratory Medicine Facility for further studies. On arrival, a High-Resolution Computed Tomography (HRCT) of the lungs was performed, which showed the previously mentioned nodules as well as interlobular septal thickening. A flexible video bronchoscopy (FBO) was then performed, obtaining specimens samples of bronchial lavage for AFB, PCR for M. tuberculosis and transbronchial biopsies. The first two were reported as “negative”, and the transbronchial biopsies reported “nodular and diffuse fibrosis”, therefore proceeding to a surgical biopsy, with the following findings: “generalized micronodular pattern and multiple nodules. A surgical specimen from lingula is obtained”. Histopathology reported: “Low-grade vascular neoplasm compatible, by morphology, with Epithelioid Hemangioendothelioma. Immunohistochemistry positive to CD31 and CD 34, and negative to Actin, CK and COCKTEL.” DISCUSSION: The suspicion of an infectious disease was based on clinical findings: A young woman from a tuberculosis endemic region in México, with an important tuberculosis exposure due to her work as a healthcare professional, a clinical condition of 2-month non-productive cough, and a HRCT with micronodular hyperdensities. At first glance, Miliary Tuberculosis was suspected and accordingly approached. All tests made in fiber-optic bronchoscopy were negative for Tuberculosis and Fungi. Afterward, when a surgical biopsy was performed a neoplasm was found. Oncology assessed the case and decided expectant follow up. The patient will maintain follow up every 3 months with an HRCT. In the event of progression or worsening of the symptoms, medical therapy will be started. CONCLUSIONS: The epithelioid hemangioendothelioma is a very rare low-grade sarcoma. It appears typically in young women, with an average age of 40 years. An insidious evolution if often present, with respiratory symptoms, nodules, usually under 1cm, and interlobular septal thickening in the HRCT. The treatment consists of chemotherapy, interleukin-2 and interferon alfa-2B. The average survival of this neoplasm is 4.6 years, varying between 12 months and 26 years. Reference #1: Cronin P, Arenberg D. Pulmonary Epithelioid Hemangioendothelioma An Unusual Case and a Review of the Literature. 2nd ed. CHEST; 2004. Reference #2: Rosengarten D, Kramer M, Amir G, Fuks L, Berkman N. Hemangioendothelioma Epithelioid Pulmonary. 13th ed. IMAJ, Israel Medical Association Journal; 2011. DISCLOSURES: No relevant relationships by Paloma Barajas, source=Web Response No relevant relationships by Luis Galindo, source=Web Response No relevant relationships by Maria del Carmen Lozano Cuevas, source=Web Response No relevant relationships by Alfredo Noyola Correa, source=Web Response

Pulmonary Epithelioid Hemangioendothelioma: Two Case Reports of a Rare Neoplasm with an Unpredictable Prognosis

This is a comparative analysis of two patients diagnosed with pulmonary epithelioid hemangioendothelioma (PEH). The objective is to educate readers to PEH, a rare disease with a wide range of clinical manifestations that may be mistaken for lung adenocarcinoma or pulmonary metastases. This study is a retrospective analysis of electronic medical records and PACS diagnostic imaging files followed by literature review using PubMed. Case 1 is a male diagnosed incidentally with multiple lung nodules during a job admission exam. Case 2 is a female, symptomatic, found to have multiple lung nodules on investigation. Both patients had a CT-guided biopsy that established their diagnoses. Case 1 remains asymptomatic in his ninth year of follow-up, whereas case 2 died less than 9 months after development of symptoms despite treatment with radiotherapy, endobronchial prosthesis, and an oral multikinase inhibitor with anti-angiogenics properties. In this study, we aimed to illustrate the heterogeneity of PEH. Because of PEH’s rarity, its natural history and prognostic factors remain obscure. Medical literature is primarily composed of case studies with inconsistent methodology and diagnostic criteria.

Epithelioid hemangioendothelioma of the lung: CT findings and clinical course of 35 cases.

To evaluate computed tomography findings and assess the clinical course of patients with pulmonary epithelioid hemangioendothelioma. Patients diagnosed with pulmonary epithelioid hemangioendothelioma at our institution between 2000 and 2019 were retrospectively analyzed. Patients with pleural involvement were excluded. Computed tomography findings of the lung at diagnosis were classified into three patterns: multiple small nodules pattern (˂15mm), multiple nodules with large lesions pattern (≥15mm) and single lesion pattern. Additionally, the clinical course of patients was evaluated. Thirty-five patients (15 men and 20 women; median age, 44years) with pulmonary epithelioid hemangioendothelioma were identified. The multiple small nodules pattern, multiple nodules with large lesions pattern and single lesion pattern were observed in 25 (71.4%), 8 (22.9%) and 2 (5.7%) patients, respectively. In 22 (62.9%) patients, extra-pulmonary epithelioid hemangioendothelioma lesions were found. Most patients were followed without initial treatment, while two patients with single lesion pattern underwent surgical resection. The median follow-up period was 63months. Five-year overall survival rate of all patients was 96.3%. Latest clinical information revealed that 20 (20/25, 80%) patients with multiple small nodules pattern were alive without symptoms. In patients with multiple nodules with large lesion pattern, four (4/8, 50%) patients were alive without symptoms, three (3/8, 37.5%) patients were alive with symptoms and one (1/8, 12.5%) died. No recurrence was observed in patients with single lesion pattern. Multiple small nodules pattern was the most common findings of pulmonary epithelioid hemangioendothelioma. Patients with pulmonary epithelioid hemangioendothelioma have good prognosis.

Clinicopathological features of pulmonary epithelioid hemangioendothelioma: a study of 18 cases

Objective: To investigate the clinicopathological features of pulmonary epithelioid hemangioendothelioma (PEHE). Methods: Eighteen cases of PEHE were collected from August 2011 to December 2018 at the First Affiliated Hospital of Zhengzhou University. All cases were retrospectively studied by hematoxylin and eosin staining and immunohistochemistry (IHC). The clinicopathological features were reviewed; the status of CAMTA1 and TFE3 gene was analyzed and patients' outcome was followed up. Results: Of the 18 cases, there were 11 males and 7 females with a male to female ratio of 1.6 to 1.0. The patients' age ranged from 36 to 68 years (mean 52 years). Twelve cases (12/18) showed a single nodule and six cases (6/18) showed multiple bilateral nodules. Seven cases (7/18) involved other organs besides lung. Seventeen (17/18) patients presented with respiratory symptoms and one patient (1/18) presented with abdominal pain. Grossly, the tumors were greyish-white nodules with indistinct borders. Microscopically the tumor cells were epithelioid and arranged in strands and nests, and cytoplasmic vacuoles were commonly noted. The stroma was myxochondroid or hyaline. By IHC, the tumor cells were positive for CD31(18/18), CD34 (16/18), ERG (18/18) and Fli-1 (18/18); CKpan was focally positive in 5 cases (5/18). TFE3 was positive in 3 cases (3/18), and Ki-67 index ranged from 5% to 30%. FISH analysis showed seventeen cases (17/18) had CAMAT1 rearrangement, one case had TFE3 rearrangement displaying a split signal. Eight patients (8/18) had surgical excision, three patients (3/18) had surgery and chemotherapy, and seven patients (7/18) had chemotherapy only. Four patients (4/18) died of the disease. Conclusions: Patients with PEHE have non-specific symptoms, and correct diagnosis depends on pathologic biopsy and the exclusion of other tumors with epithelioid morphology. Some patients with PEHE have poor prognosis, particularly in those who have multiple nodules, peripheral invasion or metastasis.

Research Progress of Pulmonary Epithelioid Hemangioendothelioma

上皮样血管内皮瘤（epithelioid hemangioendothelioma, EHE）是一种罕见的血管肿瘤，起源于血管内皮细胞，可发生于身体多个部位，以软组织（四肢）、骨骼、肝脏和肺为主。相比肺部其他肿瘤而言，肺上皮样血管内皮瘤（pulmonary epithelioid hemangioendothelioma, P-EHE）比较少见。国外报道约百例，国内有数十例报道。由于P-EHE发病率低、临床症状及影像学表现缺乏特异性，因此经常被误诊或漏诊且未接受适当治疗，从而导致其预后不良。目前，组织学及免疫组织化学检查是诊断该病的重要依据。鉴于P-EHE较为少见，尚无统一的治疗方案。若病灶小且结节数量有限，手术是首选的治疗方法，同时达到诊断和治疗的目的。本文现就有关P-EHE的发病机制、临床表现、诊断、治疗方法、预后予以综述。

P1.09-38 Pulmonary Epithelioid Hemangioendothelioma (EHE) with Von Recklinghausen Disease

Malignant thoracic epithelioid tumors are an uncommon. The heterogenous group of tumors that include the low to intermediate grade epithelioid hemangioendothelioma(EHE) and epithelioid angiosarcoma under examination of the morphologic and immunohistochemical features. We here reported a rare case of multiple pulmonary epithelioid hemangioendothelioma with von Recklinghausen disease. Case A 34–year–old male was admitted to our hospital with an abnormal shadow in the bilateral lung field. He has von Recklinghausen disease, and non-smoker. The laboratory data and physical examination are normal. A chest computed tomography scan showed multiple nodal lesions of 2 mm to 12 mm in diameter in the bilateral lung fields. Thoracoscopic partial resection of the right middle lobe was performed. The tumor was well-circumscribed lesion. In the pathological findings, sections showed a proliferation of epithelioid tumor cells having oval-shaped and vesicular nuclei, prominent nucleoli and pale eosinophilic cytoplasm arranged in small nested or cord patterns. Intracytoplasmic vacuoles some of which contain erythrocytes were recognized. Mitotic figures were rarely seen. In the immunohistochemical examination, the tumor cells were positive for CD31 and CD34, but negative for D2-40, CAM5.2, Ber-EP4, alpha-SMA, desmin, S-100 protein, NSE, TTF-1, calretinin, claudin-4, CD36 and c-kit. RT-PCR analysis showed CAMTA1-WWTR1 fusions was negative. Those features were consistent with pulmonary epithelioid hemangioendothelioma. Pulmonary epithelioid hemangioendothelioma(EHE) is rare tumor and there is no report in the literature of EHE with von Recklinghausen disease. The behavior of this neoplasm is uncertain, so the methods of diagnosis and treatment will demand to do careful observation and further examination.