ABSTRACT
The term cystic lung disease encompasses a heterogeneous group of entities characterised by round lung lesions that correspond to cysts with fine walls, which usually contain air. The differential diagnosis of these lesions can be challenging, requiring both clinical and radiological perspectives. Entities such as pulmonary emphysema and cystic bronchiectasis can simulate cystic disease. High-resolution computed tomography (HRCT) is the imaging technique of choice for the evaluation and diagnosis of cystic lung disease, because it confirms the presence of lung disease and establishes the correct diagnosis of the associated complications. In many cases, the diagnosis can be established based on the HRCT findings, thus making histologic confirmation unnecessary. For these reasons, radiologists need to be familiar with the different presentations of these entities. A wide variety of diseases are characterised by the presence of diffuse pulmonary cysts. Among these, the most common are lymphangioleiomyomatosis, which may or may not be associated with tuberous sclerosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. Other, less common entities include Birt-Hogg-Dubé syndrome, amyloidosis, and light-chain deposit disease. This article describes the characteristics and presentations of some of these entities, emphasizing the details that can help differentiate among them.
Subject(s)
Cysts , Histiocytosis, Langerhans-Cell , Lung Diseases, Interstitial , Lymphangioleiomyomatosis , Humans , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/pathology , Lung/pathology , Lymphangioleiomyomatosis/diagnostic imaging , Lymphangioleiomyomatosis/pathology , Histiocytosis, Langerhans-Cell/diagnostic imaging , Histiocytosis, Langerhans-Cell/pathology , Cysts/diagnostic imagingABSTRACT
The pleuropulmonary blastoma is an aggressive primary lung tumor. Is most frequent in paediatric population, and there are a few cases reported worldwide. It consists of embrionary primitive mesenquimal tissue, and is different of the adult Pulmonary Blastoma. The clinical presentation can be missed by other prevalent diseases or may be an accidental diagnosis. The outcome following diagnosis is poor, overall for types ii and iii, with bad response to surgery and quimiotherapy, high rates or recurrence to more aggressive forms (eg. BPP type i to type ii o iii). This report describes the clinical picture of a two years old preschool child with aggressive BPP. We reviewed the actual literature about this topic.
El blastoma pleuropulmonar (BPP), es un tumor agresivo primario de pulmón. Afecta sobre todo en la edad pediátrica, habiendo sido reportado pocos casos a nivel mundial. El BPP consiste de tejido mesenquimal primitivo embrionario, de características diferentes al blastoma pulmonar del adulto. La presentación clínica suele confundirse con otras patologías frecuentes o puede ser incidental. La sobrevida luego del diagnóstico es pobre, sobre todo para los tipos ii y iii, con poca respuesta a la quimioterapia- cirugía, y alta frecuencia de recaídas a formas más agresivas. Se describe el caso de una pre-escolar de 2 años, con diagnóstico de BPP, que presentó una evolución clínica agresiva, se realizó la revisión de la literatura sobre los principales tópicos concernientes a esta patología.
Subject(s)
Humans , Female , Child, Preschool , Pulmonary Blastoma/diagnosis , Pulmonary Blastoma/therapy , Lung Neoplasms/diagnosis , Lung Neoplasms/therapy , Cysts , Diagnosis, Differential , Fatal Outcome , Pleural Neoplasms/diagnosis , Pleural Neoplasms/therapy , PrognosisABSTRACT
Congenital pulmonary airway malformation or CPAM (congenital cystic adenomatoid malformation) that involve the lung is an embryonic anomaly. CPAM is a rare condition in chilhood, and even more so in adulthood. In this article we report the case of a young adult man with CPAM and present a brief review about this condition. Case study: 23 years old man with several episodes of pneumonia and obstructive bronchial syndrome during his childhood. The patient complained of pleuritic chest pain, malaise and fever. On physical examination lung sound was diminished in the lower right zone of the chest, abundant coarse crackles were detected and there was dullness on percussion. Chest roentgenogram and computed tomography (CT) revealed a complex image of multiple cysts with air and fluid in the right lung. Because of the lack of response to medical treatment a right lower lobectomy was performed. Histopathology revealed a type 1 CPAM. After being discharged from the hospital the patient developed a broncho-pleural fistula, that was succesfully treated by a second surgical intervention. Three years after surgery the patient is free of symptoms. Discussion: CPAM is a rare congenital hamartomatous condition. This malformation in adults it is exceptional. Chest CT is the choice diagnostic technique. Considering the high neoplastic potential of this malformation, its treatment should be the surgical resection, even in asymptomatic patients.
La malformación congénita de la vía aérea pulmonar (MCVAP), llamada también malformación congénita adenomatoidea quística que compromete al pulmón es una anomalía embrionaria. La MCVAP es de rara presentación en la infancia y es excepcional en los adultos. En este artículo comunicamos el caso de un hombre adulto con MCVAP y presentamos una breve revisión sobre esta condición. Caso clínico: Hombre de 23 años de edad con varios episodios de neumonía y de síndrome bronquial obstructivo en su niñez. El paciente se quejaba de dolor torácico pleurítico, malestar general y fiebre. En el examen físico se encontró disminución del murmullo pulmonar en la parte inferior del hemitórax derecho, abundantes crepitaciones y matidez. La radiografía y la tomografia computada de tórax (TC) revelaron múltiples imágenes hidro-aéreas complejas en el pulmón derecho. Debido a la falta de respuesta al tratamiento médico, se practicó una lobectomía inferior derecha. La histopatología demostró una MCVAP tipo 1. Después de ser dado de alta el paciente presentó una fístula broncopleural, la cual fue tratada exitosamente con una segunda intervención quirúrgica. Tres años después de la cirugía el paciente está asintomático. Discusión: La MCVAP es una malformación congénita hamartomatosa muy poco frecuente. En adultos es excepcional. La técnica diagnóstica de elección es la TC de tórax. Considerando el alto potencial neoplásico de esta malformación, su tratamiento debe ser la resección quirúrgica, incluso si el paciente está asintomático.
Subject(s)
Humans , Male , Adult , Cystic Adenomatoid Malformation of Lung, Congenital/surgery , Cystic Adenomatoid Malformation of Lung, Congenital/diagnosis , Cystic Adenomatoid Malformation of Lung, Congenital/classificationABSTRACT
Los quistes congénitos del pulmón son un grupo diverso de anomalías que pueden ser únicos o múltiples y variar grandemente en su volumen. Por lo regular están confinados a un segmento o lóbulo y son asiento frecuente de infección. Se presenta el caso de un quiste broncogénico intrapulmonar gigante acompañado de hipoplasia de la arteria pulmonar izquierda y del lóbulo pulmonar superior ipsilateral. Se hacen consideraciones relacionadas con el origen del quiste, su relación con la hipoplasia arterial y pulmonar y se discuten las posibles causas de la atelectasia y edema posoperatorios, que llevaron a la necesidad de completar la neumonectomía ante un cuadro séptico severo(AU)
The congenital lung cysts are a group of diverse abnormalities, unique or multiple, and of different volumes. Generally, they are confined to a segment or lobe and they are a frequent site of infection. The case of a bronchogenic intrapulmonary giant cyst accompanied with hypoplasia of the left pulmonary artery and of the upper ipsilateral pulmonary lobe, is reported. Considerations are made on the origin of the cyst, and on its connection with arterial and pulmonary hypoplasia. The possible causes of postoperative atelactasis and edema, leading to the need of completing pneumonectomy in the face of a severe septic picture, are discussed(AU)
