Pulmonary capillary hemangiomatosis: a lesson learned

Pulmonary capillary hemangiomatosis (PCH) is a rare and controversial entity that is known to be a cause of pulmonary hypertension and is microscopically characterized by proliferation of dilated capillary-sized channels along and in the alveolar walls. Clinically, it is mostly seen in adults. Clinical features are characterized by nonspecific findings such as shortness of breath, cough, chest pain, and fatigue. It can be clinically indistinguishable from pre-capillary pulmonary arterial hypertension disorders such as primary pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension. However, the diagnostic distinction, which usually requires a multidisciplinary approach, is crucial in order to avoid inappropriate treatment with vasodilator medications usually used for PAH treatment. Prognosis of PCH remains poor with lung transplant being the only definitive treatment. We report an autopsy case of pulmonary capillary hemangiomatosis unmasked at autopsy that was treated with a prostacyclin analog, usually contraindicated in such patients. We emphasize that this entity should always be on the differential diagnosis in a patient with pulmonary hypertension and requires great vigilance on the part of the clinician, radiologist and pathologist to make the diagnosis and guide appropriate management.

Hemangioma capilar como causa de colestasis extrahepática: reporte de caso / Capillary hemangioma as cause of extrahepatic cholestasis: case report

El hemangioma capilar es una patología epitelial benigna que se presenta usualmente en la piel, sin embargo también puede presentarse en mucosas, hasta el momento en la literatura revisada no hay reportes de hemangiomas capilares como causa de colestasis extrahepáticas. Se reporta el caso de un paciente femenino de 35 años con clínica de colestasis extrahepática sin evidencias de lesiones ocupantes de la luz de las vías biliares, se le realiza duodenoscopia donde se evidencia lesión de aspecto adenomatoso a nivel de la papila de Vater, la cual es resecada. El reporte anatomopatológico concluye la lesión como un hemangioma capilar. En vista de que en la literatura no se encuentran reportes de hemangiomas capilares como etiologías de colestasis se decide reportar dicho hallazgo

The capillary hemangioma is a benign epithelial disease usually occurs in the skin, but can also occur in mucous membranes, so far in the literature there are no reports of capillary hemangiomas as a cause of extrahepatic cholestasis. We report the case of a female patient 35 years old, with clinical signs of extrahepatic cholestasis and no evidence of occupying lesions of the bile ducts, duodenoscopy was performed which revealed a lesion of adenomatous aspect of the papilla of Vater, which is resected and sent to pathology. The report from pathology was of a capillary hemangioma. Since in the literature are no reports of capillary hemangiomas as etiologies of cholestasis is decided to report this finding

Recurrent Pulmonary Capillary Hemangioma: Dynamic Contrast-Enhanced CT and Histopathologic Findings

We report the dynamic contrast-enhanced CT and histopathologic findings of a rare case of recurrent pulmonary capillary hemangiomas. The findings consisted of peripheral nodular enhancement at the early arterial phase and a subsequent "central filling-in" enhancement pattern on the delayed scans, which was identical to the well-known enhancement pattern of hemangiomas of the liver. Although there was no evidence of histological malignancy, pulmonary capillary hemangiomas manifested as multiple nodular lesions and showed postoperative recurrence.

Importância do exame oftalmológico na doença de von Hippel-Lindau / The importance of the ophthalmological examination in von Hippel-Lindau disease

Von Hippel-Lindau (VHL) é uma síndrome tumoral autossômica dominante. Esses tumores incluem hemangioblastoma da retina e sistema nervoso central (CSN), carcinoma de células renais, feocromocitoma, tumores de pâncreas, cistoadenoma de rins, pâncreas e epidídimo. Os sintomas mais comuns são perda da visão, aumento da pressão intracraniana, déficits neurológicos, aumento da pressão arterial sistêmica paradoxal e dor local. Relatamos o caso de um paciente com perda de visão e história de hemangiomas cerebelares cujo diagnóstico de VHL foi feito após exame oftalmológico.

Von Hippel-Lindau (VHL) disease is an autossomical, dominant inherited tumour syndrom. These tumours may include haemangioblastoma in the retina and central nervous system (CNS), renal cell carcinoma, phaeochromocytoma, islet cell tumours of the pancreas, cystadenoma in the kidney, pancreas, and epididymis. The most common symptoms include: loss of vision, raised intracranial pressure, neurological deficits, paroxysmal raised blood pressure and local pain. We report herein a 29-year-old man with visual loss and cerebellar haemangioblastoma that despite neurological manifestations the diagnosis of VHL was established after the ophthalmological examanination.

Large capillary haemangioma arising from the nasal columella--a case report.

A 39-year-old male presented with a mass arising from the nasal columella for last 8 months. The mass was lobulated, dark red in colour, firm in consistency and approximately 3 cm in diameter. It was attached to the columella of the nose by a narrow stalk. The mass was excised completely under general anaesthesia and histopathological examination suggested it to be a case of capillary haemangioma.