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Pulmonary congenital cystic disease in adults. Spiral computed tomography findings with pathologic correlation and management

Malattia polmonare cistica congenita nell’adulto. Aspetti semiologici in tomografia computerizzata spirale, correlazioni istopatologiche e trattamento

  • Chest Radiology/Radiologia Toracica
  • Published:
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Abstract

Purpose

The aim of this study was to assess the computed tomography (CT) features of intrapulmonary congenital cystic diseases in adults and to correlate the imaging features with the pathological findings, with emphasis on the oncogenic potential of the lesions.

Materials and methods

We retrospectively reviewed the CT scans in three institutions from August 1996 to December 2008, of nine patients (six men, three women; mean age 48.6 years; range 26–75 years) who had histological diagnosis of pulmonary cystic disease after surgery. Six patients had a diagnosis of intrapulmonary bronchogenic cyst (IBC), and three had a type-I cystic adenomatoid malformation (CAM). In one case, intralobar sequestration (ILS) was associated with type-I CAM.

Results

Three patients were symptomatic and six were asymptomatic. On CT scans, IBCs showed homogeneous fluid attenuation (n=2), air-fluid level (n=2), air attenuation (n=1) or soft-tissue attenuation (n=1). The surrounding lung tissue showed areas of band-like linear attenuation in three IBCs, atelectasia in two and mucocele-like areas in one. On CT, type-I CAM appeared as a unilocular cystic lesion with air-fluid level (n=1) or air content (n=1). Both cases had thin walls surrounded by normal lung parenchyma. ILS appeared as a fluid-filled cyst with afferent and efferent vessels. Of the six IBCs, one occurred in the upper right lobe, two in the middle lobe and three in the lower right lobe. Of the three type-I CAMs, one was in the upper left lobe and one in the middle lobe. The type-I CAM associated with ILS was located in the left lower lobe.

Conclusions

The similar CT patterns preclude differentiation between IBC and type-I CAM. Surgical resection of all intrapulmonary cystic lesions detected in adults is mandatory because type-I CAM is a precursor of mucinous bronchioloalveolar carcinoma.

Riassunto

Obiettivo

Scopo di questo lavoro è stato valutare con tomografia computerizzata (TC) gli aspetti semeiologici delle malattie cistiche congenite intrapolmonari nell’adulto e correlarle con i reperti patologici, in riferimento al potenziale oncogenetico di tali lesioni.

Materiali e metodi

Abbiamo analizzato retrospettivamente gli esami TC di tre centri, da agosto 1996 a dicembre 2008, relativi a nove pazienti (sei maschi e tre femmine, età media 48,6 anni, range compreso tra 26-75 anni) trattati chirurgicamente per patologia cistica polmonare, in cui l’esame istologico del pezzo operatorio ha consentito di ottenere la diagnosi definitiva: in sei pazienti cisti broncogena intrapolmonare (CBI) e in tre malformazione adenomatoide cistica (MAC) di tipo I, associata in un caso a sequestro polmonare intralobare (SPI).

Risultati

Tre pazienti erano sintomatici e sei asintomatici. All’esame TC le CBI si presentavano: in due casi come lesioni completamente liquide, in due casi con livello idroaereo contestuale, in un caso come una formazione completamente ripiena di aria e in un caso come una formazione omogenea rispetto alla densità dei tessuti molli. Nel parenchima contiguo alle CBI erano presenti bande lineari di fibrosi in tre casi, atelettasia in due e lesione simile a mucocele in un caso. Le MAC di tipo I si presentavano una come lesione cistica uniloculata con livello fluido, e l’altra a contenuto aereo; entrambe erano delimitate da una parete sottile con parenchima adiacente normale. La MAC associata a SPI appariva come cisti completamente ripiena di liquido con un arteria bronchiale ectasica afferente ed una vena efferente. Delle sei CBI, una era localizzata nel lobo superiore di destra, due nel lobo medio, e tre in quello inferiore di destra. Le tre MAC di tipo I erano localizzate nel lobo superiore di sinistra, nel lobo medio e quella associata a SPI, nel lobo inferiore sinistro.

Conclusioni

L’aspetto TC analogo tra CBI e MAC di tipo I non consente una diagnosi differenziale tra queste due lesioni. Nell’adulto è sempre raccomandabile la resezione chirurgica di tutte le lesioni intrapolmonari cistiche poiché la MAC di tipo I è il precursore del carcinoma mucinoso bronchiolo-alveolare.

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Authors and Affiliations

  1. Department of Surgical, Radiologic and Odontostomatologic Sciences, Section of Diagnostic and Interventional Radiology, University of Perugia, S. Maria della Misericordia Hospital, S. Andrea delle Fratte, 06156, Perugia, Italy

    M. Scialpi & L. Lupattelli

  2. Department of Radiology, Radiotherapy and Nuclear Medicine, Magrassi Lanzara II Naples University, School of Medicine, 80100, Naples, Italy

    S. Cappabianca & A. Rotondo

  3. Department of Radiology and Laboratory, S. Maria della Misericordia Hospital, S. Andrea delle Fratte, 06156, Perugia, Italy

    G.B. Scalera

  4. Department of Surgical, Radiologic and Odontostomatologic Sciences, Section of Oncologic Surgery, University of Perugia, S. Maria della Misericordia Hospital, S. Andrea delle Fratte, 06156, Perugia, Italy

    F. Barberini

  5. Section of Thoratic Surgery, University of Perugia, S. Maria della Misericordia Hospital, S. Andrea delle Fratte, 06156, Perugia, Italy

    L. Cagini

  6. Department of Radiology, Bentivoglio Hospital, Via Marconi 35, ASL Bologna, 40010, Bentivoglio, Bologna, Italy

    S. Donato

  7. Department of Radiology, University of Molise, C. da Tappino, 86100, Campobasso, Italy

    L. Brunese

  8. Department of Radiology, Budrio Hospital, Via Benni 44, ASL Bologna, 40054, Budrio, Italy

    I. Piscioli

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  1. M. Scialpi
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Scialpi, M., Cappabianca, S., Rotondo, A. et al. Pulmonary congenital cystic disease in adults. Spiral computed tomography findings with pathologic correlation and management. Radiol med 115, 539–550 (2010). https://doi.org/10.1007/s11547-010-0467-6

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  • DOI: https://doi.org/10.1007/s11547-010-0467-6

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