1/4. An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension.We report a case of pulmonary capillary hemangiomatosis (PCH) affecting a 37-year-old woman. PCH is a rare disease accompanying intrapulmonary capillary growth and pulmonary hypertension. In the present case, capillaries infiltrated the alveolar and bronchial walls but not the vascular walls. No signs or symptoms of pulmonary hypertension or right heart hypertrophy/dilatation were present. These findings suggest that vascular involvement in PCH is responsible for the induction of pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. Congenital pulmonary capillary hemangiomatosis: Report of two cases and review of the literature.Pulmonary capillary hemangiomatosis (PCH) is a rare disease characterized by pulmonary hypertension and excessive neovascularization within the pulmonary interstitium, vasculature, and airways. We describe two unusual cases of congenital PCH. Both cases had concurrent anomalies, including renal and urinary bladder agenesis and hypertropic cardiomyopathy. In one case, capillary proliferation caused significant impingement of the proximal bronchial airways. A review of the current literature is described.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. Senile angioma-like eruption: a skin manifestation of intravascular large B cell lymphoma.Intravascular large B cell lymphoma (IVL) is a rare disease characterized by intravascular proliferation of neoplastic mononuclear cells within the vascular lumina. The diagnosis requires skin, liver, renal, meningeal, or brain biopsy, but is rarely made antemortem. We report a 67-year-old man in whom the histological examination of senile angioma-like eruption enabled us to establish the diagnosis of IVL. In a suspected case of IVL, a skin biopsy of the senile angioma-like eruption may be beneficial for an early diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. Pulmonary capillary hemangiomatosis: a clinicopathologic review.Pulmonary capillary hemangiomatosis is a locally aggressive benign vascular neoplasm of the lung characterized by the presence of numerous cytologically benign thin-walled capillary-sized blood vessels proliferating diffusely through the pulmonary 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 veno-occlusive 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |