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Ann Thorac Surg 1994;57:772-776
© 1994 The Society of Thoracic Surgeons

Articles

Pulmonary capillary hemangiomatosis: A clinicopathologic review

Mahmoud A. Eltorky, MD, PhD*, A.Stacey Headley, MD, Helen Winer-Muram, MD, H.Edward Garrett, Jr, MD, John P. Griffin, MD

Departments of Pathology, Pulmonary Medicine, Diagnostic Radiology, and Thoracic Surgery, University of Tennessee and Baptist Memorial Hospital, Memphis, Tennessee USA

* Address reprint requests to Dr Eltorky, Department of Pathology, University of Tennessee, 800 Madison Ave, Memphis, TN 38163.

Pulmonary capillary hemangiomatosis is a locally aggressive benign vascular neoplasm of the lung characterized by the presence of numerous cytologjcally benign thin-walled capiltaiy-sized blood vessels proliferating diffusely through the puimonary interstitium, in and around pulmonary vessels and airways. Pulmonary capillary hemangiomatosis is a rare disease characterized by pulmonary hypertension and a slowly progressive clinical course; it is frequently misdiagnosed clinically as primary pulmonary hypertension and venoocclusive disease. The purpose of this review is to describe the clinical, radiologic, and histologic features of this rare form of pulmonary vascular neoplasm, which may present considerable diagnostic problems to both the clinician and the histopathologist. Fourteen cases of pulmonary capillary hemangiomatosis have been previously reported. In this review we describe the fourth case of pulmonary capillary hemangiomatosis in which the diagnosis was made antemortem, as well as the fourth to undergo lung transplantation.




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