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A4010 - Hemoptysis as a Presentation of Composite Hemangioendothelioma
Author Block: J. S. Harrington1, S. R. Midoneck2, A. C. Borczuk3, E. Shostak4, N. K. Altorki4, A. Sanders1; 1Department of Pulmonary and Critical Care Medicine, New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, United States, 2Department of Medicine, New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, United States, 3Department of Pathology and Laboratory Medicine, New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, United States, 4Department of Cardiothoracic Surgery, New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, United States.
INTRODUCTION: Composite hemangioendothelioma is an exceedingly rare vascular neoplasm of intermediate malignancy displaying an amalgam of histologically distinct components that primarily involves the skin and superficial soft tissues. Herein, we provide clinical, radiographic, and pathologic evidence of a primary composite hemangioendothelioma of the lung.
CASE REPORT: A 71-year old man presents for evaluation of hemoptysis. His past medical history was significant for hypertension, hyperlipidemia, type II diabetes mellitus, tonsillectomy, resection of a papillary transitional cell carcinoma of the bladder, distal pancreatectomy with splenectomy for suspected pancreatic adenocarcinoma that was found to be sclerosing pancreatitis with an eosinophilic infiltrate, and former tobacco abuse. CT Chest was notable for a right middle lobe peribronchial consolidation with ground glass opacities in the right middle lobe and right lower lobe as well as a 5.8cm subcarinal mass. These findings were interpreted as pneumonia with pulmonary hemorrhage and a possible foregut duplication cyst prompting treatment with antibiotics. Follow-up CT Chest revealed increasing nodular consolidation in the right middle lobe with airway involvement and progressive lymphadenopathy; these abnormalities displayed mild FGD-avidity on PET/CT. A bronchoscopy with bronchoalveolar lavage, bronchial brushings, and endobronchial ultrasound guided biopsy of the right middle lobe consolidation, hilar lymphadenopathy, and subcarinal mass was performed. Cultures grew mycobacterium avium complex. Cytology was negative for malignant cells and biopsy revealed reactive lymphoid tissue. The patient was subsequently referred to cardiothoracic surgery for video-assisted thoracoscopic right middle lobectomy and drainage of the subcarinal cyst. Surgical pathology found a CD31 positive vasoformative tumor with vascular filling that formed anastomosing channels between large vessels and the bronchial wall with evidence of ulceration of the bronchial mucosa. Additionally, the tumor formed large blood filled lakes, accounting for the subcarinal mass. AFB stains were negative. The Ki-67 score was low. These findings were most consistent with a composite hemangioendothelioma. Recently, he was found to have new bilateral renal masses for which he is undergoing evaluation.
DISCUSSION: To date, there are less than 40 reported cases of composite hemangioendothelioma in the medical literature with this report representing one of the first cases involving the lung. It commonly presents as a slow-growing, locally invasive vascular tumor of the skin. While diagnosis is complicated by an admixture of histological components the prognosis is generally felt to be favorable with a high risk of local recurrence, but low risk of metastasis. The role of chemotherapy in surgically unresectable or metastatic disease is unknown.