Multiple sclerosing pneumocytomas: a review.

Sclerosing pneumocytoma (SP) is a rare benign low-grade tumour of the lung, and typically presents as single discrete coin lesions on imaging. Multiple SP is an exceedingly rare entity and thus reported sparingly. We review the literature on multiple SP, their clinical presentations, histopathology, relevant differential diagnoses and molecular histogenesis of this entity. SP has a predilection for East Asian origin females who have never smoked. Patients are either asymptomatic or have symptoms such as cough, haemoptysis that may be persistent, chest pain if involving the pleura and presents as discrete coin lesion on chest X-ray. Histologically, they are papillary, solid, angiomatoid or sclerotic, or combinations of these four basic patterns. Multiple lesions have the same or slightly different histological patterns. They can be distributed in either lung, in any lobe and can even be bilateral. AKT-1 molecular pathways are pivotal in their molecular pathogenesis. In this review, we further propose a classification based on five types of distribution of multiple SP.

Enfermedad venooclusiva pulmonar y hemangiomatosis capilar pulmonar / Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis

La enfermedad venooclusiva pulmonar es una causa rara de hipertensión pulmonar que forma junto a la hemangiomatosis capilar pulmonar una designación especial (subgrupo 1′) dentro del grupo 1 de hipertensión pulmonar en la última clasificación del Simposio Mundial sobre Hipertensión Pulmonar. El reciente descubrimiento de que las mutaciones del gen eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) son responsables de las formas hereditarias de la enfermedad venooclusiva pulmonar, ha provocado que el test genético adquiera un papel determinante en el diagnóstico de estos pacientes. A pesar de los avances en el conocimiento de las bases genéticas, celulares y moleculares de la enfermedad venooclusiva pulmonar en la última década, sigue siendo clásicamente una enfermedad pulmonar rara sin ningún tratamiento farmacológico eficaz aprobado y con un pronóstico muy pobre. El presente documento pretende revisar los avances que se han producido en el conocimiento de esta enfermedad en los últimos años (AU)

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension which is part, together with pulmonary capillary hemangiomatosis, of the special designation (subgroup 1’) within pulmonary hypertension group 1 in the latest classification of the pulmonary hypertension World Symposium. Recent discovery that gene mutations in eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) are responsible for inherited forms of pulmonary veno-occlusive disease has changed the role of genetic testing, acquiring relevant importance in the diagnosis of these patients. Despite the advances in genetic, cellular and molecular basis knowledge in the last decade, pulmonary veno-occlusive disease remains as a rare aetiology of pulmonary hypertension without any effective medical treatment approved and poor outcomes. This document aims to review the advances occurred in the understanding of pulmonary veno-occlusive disease in the last years (AU)

Pulmonary sclerosing pneumocytoma (sclerosing haemangioma): Radical radiation therapy.

We present a case of pulmonary sclerosing pneumocytoma (PSP) - (Formerly known as pulmonary sclerosing haemangioma) which was successfully treated with definitive radical external beam radiation therapy (EBRT). To our knowledge, such a treatment response has not been described in the literature.

Synchronous occurrence of breast cancer and pulmonary sclerosing hemangioma: management and review of the literature.

Pulmonary sclerosing hemangioma (PSH) is a rare tumor accounting for 0.2-1% of all primary lung tumors. Simultaneous occurrence of PSH with breast cancer has very rarely been reported in the literature. We describe here a case of simultaneous occurrence of PSH with breast cancer. A pathological diagnosis of PSH was confirmed by computed tomography (CT)-guided biopsy. Due to the patient's poor performance status and the benign nature of PSH, surgical excision was not considered and the patient was managed conservatively with regular follow-up. Although surgical excision is the preferred treatment for PSH, conservative management may be a reasonable option in carefully selected patients.