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R. Quercia
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P2.04 - Poster Session with Presenters Present (ID 466)
- Event: WCLC 2016
- Type: Poster Presenters Present
- Track: Mesothelioma/Thymic Malignancies/Esophageal Cancer/Other Thoracic Malignancies
- Presentations: 2
- Moderators:
- Coordinates: 12/06/2016, 14:30 - 15:45, Hall B (Poster Area)
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P2.04-046 - A Rare Case of Pleuro-Pulmonary Epitheliod Hemangioendothelioma (ID 6024)
14:30 - 14:30 | Author(s): R. Quercia
- Abstract
Background:
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 , cought, 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 a extensive pleuro-pulmonary involvement.
Methods:
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.
Results:
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.
Conclusion:
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 involment and a very aggressive clinical course.
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P2.04-047 - A Rare Case of Extramedullary Plasmacytoma Occurring in the Posterior Mediastinum (ID 6040)
14:30 - 14:30 | Author(s): R. Quercia
- Abstract
Background:
Extramedullary plasmacytoma (EMP) is a rare neoplasm that is derived from a monoclonal proliferation of plasma cells in the soft tissues or organs outside the bone marrow and is present in about 3% of all plasma cell neoplasms. The average age of patients is about 60 years. The most frequent site is the upper respiratory tract (approximately 80%). The endotoracic forms usually manifest as nodules or pulmonary masses. Rarely may it present with mediastinal mass as a primitive solitary lesion. We present a case of extramedullary plasmacytoma of the posterior mediastinum.
Methods:
A 82 years old female presented to us with a history of chest pain, persistent cough, dysphagia, asthenia and dyspnea for few weeks. She denied smoking and had ischemic heart disease and atrial fibrillation as comorbidities. Her serum protein electrophoresis and hemocythometric parameters were normal. Chest X-ray showed a posterior voluminous endothoracic opacity and chest Computed Tomography showed an expansive hypodense lesion of the posterior mediastinum of 15.6 x 9.1 cm, which surround and displaced the thoracic aorta causing compression of the esophagus and central airways without pathological lesions of the lung parenchyma and the presence of a modest bilateral pleural effusions. Transbronchial needle aspiration under eco-endoscopic guide (EBUS-TBNA) has revealed the presence of isolated elements plasma cell-like (CD138+, lambda chains+). For a precise (correct) histological definition we performed a surgical biopsy of the mediastinal mass through right uniportal video-assisted thoracoscopic surgery (VATS).
Results:
Pathological examination revealed solid tissue with massive infiltration of elements plasma cell-like. Immunohistochemical analysis showed positive staining for CD138 (plasma cell marker), CD56 (pathological plasma cells marker), monoclonal light chains lambda and negative for CD3 (marker of T line lymphoproliferative diseases). Therefore, a final diagnosis of extramedullary plasmacytoma was made.
Conclusion:
Our case of EMP without systemic signs of multiple myeloma is extremely rare, especially like a mediastinal mass as a primitive solitary lesion. In terms of posterior mediastinal manifestations of EMP, it should be differentiated from neurogenic tumor, lymphoma, and lymphangioma. 1 EMP could be concurrent with multiple myeloma or the sequences of proceedings for multiple myeloma, the prognosis is very poor and worse than primitive forms. Therefore, precice and timely framing (classification) of the disease is essential for diagnostic and therapeutic purposes.
