SESSION TITLE: Procedures Student/Resident Case Report Posters
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that behaves clinically between a benign hemangioma and malignant angiosarcoma. Most cases involved the liver and lung parenchyma. Less than fifty cases of pulmonary EHE have been reported. We describe the first case of EHE limited to the trachea and bronchus in a 61 year-old female who was successfully treated with endobronchial electrocautery.
CASE PRESENTATION: A 61-year old female was admitted due three months cough and hemoptysis. She had no history of smoking and had no family history of malignancy. Physical examination was unremarkable. Chest radiograph and sputum AFB smears were negative. CT scan of the chest revealed a polypoid lesion at the distal trachea. No enlarged lymph nodes were seen and the lung parenchyma was unremarkable. Bronchoscopy confirmed a 0.5 cm x 0.5 cm vascular polyp in the distal trachea (Figure 1). A second smaller polyp was noted near the right lower lobe bronchus. Biopsy of both lesions done was consistent with EHE (Figure 2). Positivity for CD34 and Factor VII antigen confirmed the diagnosis. Repeat bronchscopy was done and electrocautery of both lesions was performed with curative intent. No recurrence of symptoms was noted after 1 year follow-up. Follow-up bronchoscopy showed no reappearance of the lesions.
DISCUSSION: The EHE is an intermediate grade malignancy with an estimated prevalence of less than one in 1 million. It commonly affects the liver, lungs and bone and less frequently for other organs. The etiology is unknown but a chromosomal translocation t(1;3)(p36.3;q25) in which exon 4 of WWTR1 is fused with either exons 8 or 9 of CAMTA1 has been implicated.(1) Biopsy establishes the diagnosis of EHE. Immunohistochemical staining with endothelial cell markers, such as CD-31, factor VIII related antigen, and CD 34 confirms the diagnosis. Surgical resection, chemotherapy, immunotherapy and radiotherapy have been used to treat EHE with variable success. However, no standard treatment has been established. One treatment option for localized airway lesions is bronchoscopic electrocautery and is considered to be safe and effective. Electrocautery was done to the patient on repeat bronchoscopy and the lesions were successfully resected. Follow-up bronchoscopy after 1 year showed no tumor recurrence. Hence, endobronchial electrocautery for localized airway EHE may be an acceptable treatment.
CONCLUSIONS: We described a rare case of pulmonary EHE limited to the airway in a 61 year-old female. The diagnosis was made by histopathological examination and was confirmed by immunohistochemical staining for CD 34 and Factor VIII. Bronchoscopic electrocautery may be used as a treatment option for localized lesions and is generally safe and effective.
Reference #1: Errani C, Zhang L, Shao SY, Hajdu M et al. A Novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic site. Genes Chromosomes Cancer. 2011;50(8):644-653.
DISCLOSURE: The following authors have nothing to disclose: Jose Paolo Panuda, Yoshiko Ikeda-Maquiling, Albert Rafanan
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