Intravascular Bronchioloalveolar Tumor Research Articles

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

P2.04-046 A Rare Case of Pleuro-Pulmonary Epitheliod Hemangioendothelioma: Topic: Esophageal Cancer and Other Malignancies

Epithelioid hemangioendothelioma (EHE) of lung and pleura are rare vascular neoplasm with an epithelioid and histiocytoid appearance that originated from vascular endothelial cells. Pulmonary EHE was first described as “intravascular bronchioloalveolar tumor” (IVBAT) by Dail et al. in 1975, since it was believed to be an aggressive form of bronchoalveolar cell carcinoma with propensity to invade adjacent blood vessels. It is described by the World Health Organization 2002 classification as lesion locally aggressive with metastatic potential, a neoplasm of low to intermediate-grade malignancy. It occurs with a predilection for younger (mean age 39) female (60% of cases). Most patient are asymptomatic (50%) although dyspnea, cough, chest pain, hemoptysis can occur. By immunohistochemistry, EHE shows the typical markers of vascular differentiation. Primary epithelioid hemangioendotheliomas of pleura are extremely rare, usually affecting males, and associated with a variety of clinical manifestations and poor prognosis. We present a rare case of EHE with an extensive pleuro-pulmonary involvement. A 50-year old non-smoking woman presented to our institution complaining of persistent cough and progressive dyspnea first treated with antibiotics without improvement of the symptoms. Chest computed tomography (CT) showed multiple disseminated nodules of both lungs mostly involving the upper lobes with associated right-sided pleural effusion and thickening. There is a elevated serum level of Cancer Antigen (CA) 125. In a right triportal video-assisted thoracoscopy surgery (VATS) we performed wedge resections of the right upper, middle and lower lobes with diffuse nodular process and multiple biopsies of thickened pleura. Pathological examination of the pleuro-pulmonary samples showed multiple, diffuse nodular infiltrates of epithelioid cells with frequent cytoplasmic vacuoles, rare mitoses and foci of tumor necrosis. Immunohistochemistry was positive for Vimentin, CD31, CD34, ETS-related gene (ERG). Therefore, a final diagnosis of Epithelioid hemangioendothelioma was made on the basis of the radiographic, cytomorphologic, and immunohistochemical findings. Pulmonary Epithelioid hemangioendothelioma is a vascular tumor with low to intermediate-grade malignancy. Pleural epithelioid hemangioendothelioma is less common and the clinical behavior is more aggressive and has a poorer prognosis. Our case is an epithelioid hemangioendothelioma with a clinical and pathologic pleuro-pulmonary involvement and a very aggressive clinical course.

Surgical resection of a primary pulmonary epithelioid hemangioendothelioma in bilateral lungs

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare pulmonary neoplasm that was initially described in 1975 as an intravascular bronchioloalveolar tumor. This report presents the case of a patient with multifocal primary pulmonary PEH (11 tumors) in the bilateral lungs. All of the tumors detected in the preoperative computed tomography scan were surgically resected. The patient has been doing well for 9 years after surgery. No tumor recurred for 8 years after surgery until a single recurrent nodule appeared and was thoracoscopically resected.

Unusual Endobronchial Pulmonary Epithelioid Hemangioendothelioma

A case of an unusual pulmonary epithelioid hemangioendothelioma is described in which two distinctive types of histologic lesion were combined. The patient was a 54-year-old man with persistent hemoptysis. The main lesion was an endobronchial tumor that had classic features of epithelioid hemangioendothelioma of soft tissue. Multiple peripheral parenchymal nodules resembled features of intravascular bronchioloalveolar tumor of the lung. The endothelial origin of the tumor cells was evident on both ultrastructural and immunohistochemical findings, including the presence of Weibel-Palade bodies and positive immunostaining to Ulex lectin and factor VIII-related antigen. This case seems to be an example with mixture of endobronchial epitheloid hemangioendothelioma of soft tissue type and peripheral intravascular bronchioloalveolar tumor histology, an association that has not been reported as yet. Int J Surg Pathol 3(1):59-64, 1995

Epithelioid hemangioendothelioma of the lung: Pleural effusion cytology, ultrastructure, and brief literature review

Epithelioid hemangioendothelioma (EHE) of the lung is a low-grade malignant tumor of vascular origin initially described under the name intravascular bronchioloalveolar tumor (IVBAT). We present a case of a 44-yr-old Caucasian female with severe radiating back pain, shortness of breath, recurrent malignant pleural effusions, and a negative malignancy workup. Cytopathologic examination of the four pleural fluid specimens revealed large undifferentiated plasmacytoid malignant cells with abundant pink and finely granular cytoplasm, round nuclei, and prominent nucleoli. The differential diagnosis based on the cytologic findings included hepatocellular, adrenal, and renal carcinomas, melanoma, mesothelioma, and neuroendocrine tumors. Electron microscopy performed on a pleural fluid specimen and subsequent histologic examination of pleural and lung biopsies established the diagnosis of EHE.

Intravascular Bronchioloalveolar Tumor With Skin Metastases

Intravascular bronchioloalveolar tumor (IVBAT) is a rare pulmonary tumor that occurs in the younger age groups. In the present article, we describe a patient who manifested systemic growth after the resection of the primary tumor. Skin metastasis, which has never been reported, is histologically examined.

Sclerosing epitheloid angiosarcoma of bone and lung — Intravascular sclerosing bronchioloalveolar tumor

A 39-year-old woman was admitted to hospital with severe pain in the left shoulder due to an endothelial bone tumor. The tumor showed the same histologic signs as an intravascular sclerosing bronchioloalveolar tumor of the lung (IVBAT) which had been enucleated 2 years previously. This combination is uncommon. It is discussed whether the IVBAT is a malignant tumor with metastases or a benign tumor with coincidental tumors in other organs of the same endothelial vascular origin. We consider this to be an epitheloid angiosarcoma of the lung with, in our case, bone metastases; this is in agreement with some most recent reports on the disease.

Intravascular bronchioloalveolar tumour

A case of intravascular bronchioloalveolar tumour (IVBAT) presenting in a 49 yr old asymptomatic man is reported. IVBAT was diagnosed by open lung biopsy. The disease had a presumable duration of ten years and showed a low tendency to progress. The endothelial origin of the tumour was immunohistochemically proven.

Advanced intravascular bronchioloalveolar tumour and review of reports in Japan

Intravascular bronchioloalveolar tumour (IVBAT) is a rare pulmonary neoplasm. We report the clinical and pathologic findings of two patients who demonstrated unusual clinical features. Both of them were young males who showed a large mass or consolidated shadow and small nodules with pleurisy on chest X-rays due to the invasive growth of tumours. In the first case the presumptive diagnosis of lung carcinoma was made from the discovery of atypical cells in pleural fluid. In the second case pleural mesothelioma was suspected. However, the histological diagnoses of these cases, including the immuno-histological and ultrastructural data, were reported and clinical features of IVBAT described previously in Japan were summarized.

Intravascular Bronchioloalveolar Tumor: A Distinctive Surgical and Pathological Entity

A patient with intravascular bronchioloalveolar tumor is described, and the clinical and pathological characteristics of the tumor are discussed. So-called intravascular bronchioloalveolar tumor (IVBAT) is a relatively new, but well-recognized lung neoplasm. This tumor is a lung neoplasm of distinctive histogenesis newly incorporated into the World Health Organization histologic typing of lung tumors under the category “miscellaneous tumors, malignant.” The importance of the neoplasm is that it is rarely recognized clinically and frequently misdiagnosed. Awareness of this lesion can be enhanced by recognizing its particular clinical presentation, surgical appearance, and unique histopathologic factors.

Epithelioid Hemangioendothelioma of Bone

In a review of 29 cases of solitary and multicentric hemangioendothelial sarcomas of bone, 14 were found to have histologic features of epithelioid hemangioendotheliomas. These were characterized by the presence of epithelioid or "histiocytoid" endothelial cells that were either round or spindle-shaped. Intracytoplasmic vacuolization was noted, and some showed nesting of cells, mimicking metastatic adenocarcinoma. The presence of a myxoid matrix suggested the diagnosis of chondrosarcoma in some cases. Some of the tumors strongly resembled the lesions formerly designated as intravascular bronchioloalveolar tumor, angioglomoid tumor of bone, or malignant myxoid angioblastoma of bone. Factor VIII-related antigen was detected in five of the six cases examined, and ultrastructural study in five cases confirmed the endothelial nature of the tumor cells; Weibel-Palade bodies were present in three cases. There were 11 men and three women, and the tumors were seen predominantly in patients who were under 30 years of age (10 cases). Four cases involved solitary tumors, and nine were multicentric in bone. The multicentric tumors had a predilection for the bones of one lower extremity (five cases). The total series had a protracted clinical course, and the multicentric tumors appeared to follow a less aggressive course.

The intravascular bronchioloalveolar tumor and the sclerosing hemangioma of the lung: misnomers of pulmonary neoplasia.

Two very different uncommon pulmonary tumors continue to bear the misleading names originally given them. The intravascular bronchioloalveolar tumor (IVBAT) is not an epithelial tumor, but is an incurable multifocal primary pulmonary angiosarcoma with a variable and often indolent clinical course. Conversely the sclerosing hemangioma of the lung is not a vascular tumor, but is a solitary benign epithelial tumor of uncertain histogenesis with a complex architecture.

Intravascular bronchioloalveolar tumour: A 20‐year survival

An unusual case of intravascular bronchioloalveolar tumour in a 40-year-old Chinese woman is reported. The woman has survived 20 years after radiological abnormalities were first noted in her lungs. This slowly progressing endothelial lung tumour has only recently been recognized as a distinct pathological entity.

Intravascular bronchiolo-alveolar tumor (IVBAT): A low-grade sclerosing epithelioid angiosarcoma of lung.

Intravascular bronchiolo-alveolar tumor (IVBAT) is a rare and highly distinctive pulmonary tumor of disputed cellular nature. Both epithelial and endothelial differentiation of this neoplasm have been suggested. We have studied multiple nodules of IVBATs from three patients by light and electron microscopy and by immunohistochemical methods for Factor VIII-related antigen (FVIII RAG). Our light and ultrastructural studies are in essential agreement with the previous suggestion of the endothelial nature of the neoplasm and our demonstration of the presence of FVIII RAG in many of the tumor cells offers new evidence strongly supportive of their endothelial differentiation. We believe that IVBAT and a group of extrapulmonary tumors described as epithelioid hemangioendothelioma and endovascular papillary angioendothelioma are similar biologically indolent neoplasms of epithelioid and dendritic endothelial cells characterized by stromal sclerosis, intravascular spread, a low incidence of metastases and slow clinical evolution. Thus, we regard IVBAT as a low-grade sclerosing angiosarcoma of the lung.

A case of spindle cell hemangioendothelioma involving the lung, mediastinum and brain.

Intravascular bronchioloalveolar tumor is now recognized as a pulmonary form of hemangioendothelioma(HE). HE is an unusual tumor of adult life which is characterized by proliferation of an epithelioid or endothelial cell. In the lung it usually presents as multiple bilateral slowly growing nodules less than 2 cm in diameter. The aetiology and pathogenesis of this disease are unknown. Spindle cell HE occurs at any age, but approximately one half of patient are 25 years of age or younger and males are affected twice more frequently than females. On light microscopic examination, the tumor show mild cellular atypia, nearly absent mitoses and electron-microscopic studies reveal evidence of endothelial cell differentiation. Intracytoplasmic localization of Factor VIII-related antigen is demonstrated on immunohistochemical study, which confirmed the endothelial origin of the tumor. No effective therapy is yet known for HE, but survival of this tumor can be quite long. However, one half of the patient have died, usually of progressive pulmonary insufficiency. This 19-yr-old male complained of Rt. chest pain and intermittent hemoptysis. Simple chest film and chest CT scan showed the Rt. pleural effusion, variable sized bilateral pulmonary nodules, irregular large heterogenous tumor with well enhancement and extensive necrosis in the anterior mediastinum. The mediastinal mass was biopsied and diagnosed as spindle cell HE by light microscopic finding and immunohistochemical studies.