ABSTRACT
Pulmonary capillary haemangiomatosis (PCH) is a rare disorder of unknown aetiology, characterised by proliferating capillaries that invade the pulmonary interstitium, alveolar septae and the pulmonary vasculature. It is often mis-diagnosed as primary pulmonary hypertension and pulmonary veno-occlusive disease. Pulmonary capillary haemangiomatosis is a locally aggressive benign vascular neoplasm of the lung. We report the case of a 19-year-old female who was referred to us in the early post-partum period with severe pulmonary artery hypertension, which was diagnosed as PCH by open lung biopsy.
Subject(s)
Biopsy , Diagnosis, Differential , Female , Hemangioma, Capillary/diagnosis , Hemangioma, Capillary/physiopathology , Hemangioma, Capillary/therapy , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/therapy , Lung/pathology , Lung/diagnostic imaging , Lung Neoplasms/diagnosis , Lung Neoplasms/physiopathology , Lung Neoplasms/therapy , Oxygen Inhalation Therapy/methods , Thoracic Surgery, Video-Assisted/methods , Young AdultABSTRACT
Los hemangiomas se clasifican según su variedad histológica en capilares, cavernosos, esclerosantes, venosos (racemosos) para las variedades benignas y dentro de éstas existen otras subvariedades que dependen de la respuesta de los tejidos vecinos, como el angioqueratoma, etc. La forma maligna es el angiosarcoma. El hemangioendotelioma y hemangiopericitoma son entidades específicas que constituyen cuadros bien definidos con patrón histológico diferente y comportamiento biológico específico. Cada una de esta entidades tiene un tratamiento específico y el diagnóstico de certeza es histopatológico.