Multiple bilateral pulmonary nodules revealing a congenital cystic adenomatoid malformation

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. It is very rare that presentation is delayed until adulthood. We report a case of 63-year-old woman without notable pathological antecedents in whom a systematic chest X-ray revealed multiple bilateral pulmonary nodules. The patient was asymptomatic and her physical examination was normal. CT scan showed bilateral liquid rounded thin-walled densities of various size, with a homogeneous non calcified content. The diagnosis was based on radiological findings and surgery. The result of histopathological examination obtained by thoracic surgery confirmed CCAM without malignancy. The postoperative follow up showed an excellent recovery.     

Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: A case report and review on malignancies originating in congenital cystic adenomatoid malformation

A type I congenital cystic adenomatoid malformation (CCAM) in the left lower lobe was removed from a 11-year-old boy with a 3-month history of recurrent pneumonia. As incidental finding, a bronchioloalveolar carcinoma (BAC) was found in the lung parenchyma adjacent to the cyst. A left lower lobectomy was performed. At 18 months after surgery the patient is well and free of neoplastic disease. To the best of our knowledge, this association has not been reported previously in a pediatric patient. Malignancies complicating CCAM are rarely seen, but have been reported in adults. Including our case, eight cases of BAC and five cases of rhabdomyosarcoma (RMS) in association with CCAM have been reported so far. As CCAM can host metaplastic mucous cells, primitive mesenchymal cells and differentiated but poorly organized striated muscle fibers, it has been proposed that CCAM may act as a predisposing condition for oncogenesis. Our experience adds further support that CCAM can act as a premalignant lesion. Previous reports of both BAC and RMS in asymptomatic CCAM suggest prompt resection shortly after diagnosis. Pediatr. Pulmonol. 1998; 25:62–66. © 1998 Wiley-Liss, Inc.     

Haemopneumothorax from congenital cystic adenomatoid malformation in a cryptorchidism patient.

Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon congenital anomaly, especially in young adults. This study reports an 18-yr-old male with CCAM involving the right upper lobe, who presented with a moderate spontaneous haemopneumothorax initially. The patient also had bilateral abdominal cryptorchidism which required surgical treatment earlier in childhood. The chest radiographs and contrast-enhanced computed tomographic scan of the chest showed a multicystic lesion with air-fluid levels in the right upper lung. The right upper lobe was resected through a posterolateral thoracotomy. Histological examination confirmed the diagnosis of CCAM. To the authors' knowledge, congenital cystic adenomatoid malformation presenting with spontaneous haemopneumothorax and haemoptysis has never been described in the literature.     

Congenital cystic adenomatoid malformation of the lung. Prenatal diagnosis in a case

We report a case of congenital cystic adenomatoid lung malformation, type I in the Stocker classification, diagnosed at ultrasonography at 27 weeks gestation. The lesion stabilized as was the hydramnios. Vaginal delivery was uneventful. Early surgery with lobectomy led to a favorable outcome at 8 months follow-up. Congenital adenomatoid lung malformation is a rare finding. Prenatal ultrasound diagnosis has enabled early treatment and improved prognosis.     

The glandular component in congenital cystic adenomatoid malformation of the lung

Although severe congenital cystic changes (CCC) of the lung may be fatal, less severe forms may regress or vanish spontaneously. With recent advances in sonography, asymptomatic CCC are increasingly found. Whether all CCC should be promptly excised, or not, is uncertain. Congenital cystic changes conceptually are bronchopulmonary foregut malformations (BPFM) with a predilection for malignant degeneration. Among all BPFM, congenital cystic adenomatoid malformation (CCAM) is most common. We therefore searched for evidence of early malignant transformation in five surgically excised and three autopsy lungs with CCAM. By light microscopy, CCAM resembled poorly formed and dilated bronchi, bronchioles and respiratory air spaces. Four lungs had multiple nodular aggregates of mucus producing cells; the glandular component (GC). By scanning electron microscopy, GC appeared as multiple micropolyps, resembling neuroepithelial bodies. By transmission electron microscopy, GC had a surface proliferation of cells with granules of the mucous type and a basal increase in cells with owl-eyed neuroendocrine granules. The glandular component in CCAM appeared similar to the mucous cells in hyperplastic polyps of the colon and a type of mucus producing bronchioloalveolar carcinoma. Our findings support the hypothesis that CCAM is caused by dysregulated paracrine growth of mature cells and extracellular matrices and that GC could have the potential for malignant transformation. Further clinical and laboratory studies of BPFM are needed for the appropriate management of congenital cystic changes.     

Video-assisted thoracoscopic surgery in 3 cases of adult cystic adenomatoid malformation

Congenital cystic adenomatoid malformation involving the lung is a rare hamartomatous condition that is usually diagnosed in the neonatal period. The presentation of this malformation in older patients is exceptional and usually manifests in a series of recurrent lung infections affecting a single lobe or segment. The treatment of choice is complete surgical exeresis. This report of 3 cases of late presentation focuses on the surgical approach used and the unusual manifestation of recurrent spontaneous pneumothoraces in 1 patient. The patients were females aged 15, 16, and 25 years with histories of various respiratory diseases (extrinsic asthma, recurrent pneumonias, and pneumothoraces). The patients were referred to us for surgery with suspected diagnoses that were different from the final diagnoses in all cases. All underwent diagnostic video-assisted thoracoscopy to explore the affected hemothorax, and definitive treatment was possible during the procedure for 2 patients (a lobectomy and an atypical segmentectomy) by video-assisted surgery. The third patient underwent lobectomy by lateral thoracotomy after exploratory video-assisted thoracoscopy. Short- and long-term outcomes were excellent for all 3 patients.     

Late presentation of congenital cystic adenomatoid malformation of lung in ten-year-old girl

Congenital cystic adenomatoid malformation of the lung (CCAML) is uncommon, and usually presents in children with respiratory distress. We describe a girl presenting with pneumonia at age 10 years. She had been previously healthy and active, and with no previous hospitalizations or X-rays. A chest CT scan showed right lower lobe cystic lesions suggestive of CCAML.     

Congenital cystic adenomatoid malformation of the lung resembling bronchiectasis in an adult

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare anomaly which is characterized by a proliferation of dilated bronchiolar-like air spaces. It is generally seen in newborns and infants. When seen in adults, which is more uncommon, it presents itself mostly with recurrent pulmonary infections. In this article, 31-year-old man with cough and purulent expectoration and a history of recurrent pulmonary infections who had cystic changes resembling bronchiectasis at the left lower lobe on the computed tomography of the chest and diagnosed CCAM-type 2 after the histopathological examination of the left lower lobectomy specimen is presented.     

Congenital cystic adenomatoid malformation complicated by esophageal duplication cyst in a 6-month-old girl

We report a case of congenital cystic adenomatoid malformation (CCAM) complicated by an esophageal duplication cyst in a 6-month-old girl. The patient presented with recurrent pneumonia. Magnetic resonance imaging revealed two cystic lesions in the upper and lower lobes of the right lung. After cystectomy, histopathological investigation revealed that the lower cyst was a CCAM Type I, and the upper cyst was an esophageal duplication cyst. The coexistence of these complex anomalies supports the concept that the esophageal duplication cyst is one entity of a broad spectrum of developmental abnormalities caused by abnormal budding of the primitive foregut.     

In-flight spontaneous pneumothorax: congenital cystic adenomatoid malformation of the lung

Congenital cystic adenomatoid malformation (CCAM) is a rare congenital abnormality. Symptomatic presentation in adult life is extremely uncommon. The usual radiological appearance of CCAM is a cystic space-occupying lesion. Patients with underlying cystic lung disease can develop in-flight complications because of pressure-volume changes during ascent. We report the first ever case in which spontaneous pneumothorax during flight was the presenting manifestation of CCAM of the lung in a previously healthy and asymptomatic young adult. We also discuss the physiological changes during air travel which contribute to the pathogenesis of respiratory complications during air travel.     

Spontaneous pneumothorax in cystic adenomatoid malformation. Unusual clinical and histologic features

Pneumothorax is a rare presentation of congenital cystic adenomatoid malformation (CCAM) in the newborn period and is presumed to be due to resuscitative measures. A previously well three-week-old baby presented with spontaneous tension pneumothorax due to CCAM. In the lung resection specimen, a malformation was seen, which in addition to the histologic changes of CCAM, showed diffuse vascular proliferation in the interstitium and lining of air space by type 2 pneumocytes. We propose that this is a new variant of CCAM rather than one of the classic three types. The unusual clinical manifestation may be related to the unusual histologic features.     

Pulmonary cystic adenomatoid malformation in an adult patient: an underdiagnosed disease

Currently, most congenital lower respiratory tract malformations are detected during pregnancy or at birth, thanks to antenatal imaging. However, a pulmonary congenital cystic adenomatoid disease may be found in adulthood. The diagnosis is difficult, due to its rarity. We present the case of a patient whose diagnosis of pulmonary cystic adenomatoid malformation was confirmed when she had tuberculosis. A lobectomy was performed, which enabled identification of tuberculosis and also multiple cysts of adenomatoid malformation. The risk posed by this malformation, i.e. the risk of developing bronchioloalveolar carcinoma and of infection or pneumothorax, is the incentive for proposing formal surgical removal.     

Application of ultrasound in a congenital cystic adenomatoid malformation in an adult: A case report

Introduction:Congenital cystic adenomatoid malformation (CCAM) is a rare developmental lung abnormality, that typically manifests in neonates and infants but rarely in adults. Ultrasound is an important method of diagnosing CCAM in neonates and infants; however, few articles have reported the value of transthoracic lung ultrasound in the diagnosis of CCAM in adults.Patient concerns:We present a case of a 34-year-old woman with a cavitary lesion in her left lower lobe, that suggested chronic inflammation.Diagnosis:The patient underwent ultrasound examination and contrast-enhanced ultrasound-guided transthoracic core biopsy; histology suggested the diagnosis of lung hamartoma. Surgical resection of the lesion followed by histopathological analysis confirmed the diagnosis of CCAM.Interventions:The patient underwent transthoracic core biopsy under contrast-enhanced ultrasound guidance. A left lower lobectomy was then performed subsequently.Outcomes:The patient had a smooth recovery and remained asymptomatic during the 12-months of postoperative follow-up.Conclusion:We report a rare case of CCAM to suggest that transthoracic ultrasound combined with contrast-enhanced ultrasound is a safe and effective method of diagnosing the subpleural lung malformations in adults, thereby avoiding multiple radiation exposures and associated complications.     

Congenital cystic adenomatoid malformation of the lung: Hazards of delayed diagnosis

Congenital cystic adenomatoid malformation is a rare pulmonary developmental anomaly, which typically manifests in neonates and infants. Presentation in adulthood is uncommon, with <60 cases reported in the literature. The majority of cases involve one lobe only. We report a case of type 1 congenital cystic adenomatoid malformation in an adult presenting with a respiratory tract infection and haemoptysis. At thoracotomy, complex cystic masses were noted in the right upper and lower lobes. Lung-sparing surgery, in the form of two segmentectomies and a non-anatomical resection, was performed in order to avoid pneumonectomy. Such presentations may be problematic as potentially incomplete resections may increase the risk of complications and malignant transformation. This suggests the importance of appropriate clinical and radiological follow up.     

Congenital cystic adenomatoid malformation of the lung. Report of 3 cases with late presentation

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. In many cases, respiratory distress occurs during the neonatal period, and in about 80- 85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. It is very rare that presentation is delayed until adulthood. We report three cases of CCAM presenting in adults. The diagnosis was based on clinical and radiological findings in one case and two patients were not diagnosed until surgery. The lesion was present in the right lung in two and in left lung in one patient. All patients underwent surgical resection. The result of histopathological examination confirmed CCAM Stocker type 1, without malignancy. The post operative follow up showed an excellent recovery. Clinicians and pathologists need to be aware of the fact that CCAM can be present for the first time in adolescents or in adults. The clinical diagnosis is suggested by radiographic findings and is confirmed at pathology as surgery is generally indicated.     

Congenital cystic adenomatoid malformation in an adolescent: an unusual presentation with pleural effusion and pneumatocele

Congenital cystic adenomatoid malformation (CCAM) encompasses a continuum of hamartomatous cystic lung lesions characterised by the presence of abnormal bronchiolar structures of varying sizes or distribution. The CCAM is a disorder of infancy with majority of the cases being diagnosed within the first two years of life. We describe CCAM in a 13-year-old girl complaining of recurrent lower respiratory tract infections since infancy who presented with post-infectious pneumatocele with loculated pleural effusion, and suspected abscess formation and had undergone resection.     

Congenital cystic adenomatoid malformation of the lung in adults: clinical and CT evaluation of seven patients

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality that usually presents in childhood. Some associated malignancies have been reported. This study aimed to describe the clinical and multidetector CT (MDCT) image characteristics of CCAM of the lung in adults. Adult patients with congenital cystic lung diseases in association with surgery for CCAM were evaluated over a five and a half year period. Seven (four women, age range 17-64 years) of 109 congenital cystic lung disease patients were histologically confirmed as having CCAM. The most frequent symptom was productive cough (n = 5) and one patient was admitted with haemoptysis. The diagnosis was based on clinical and radiological findings and one patient was not diagnosed until surgery. MDCT images consisted of having a multiple loculated unilobar cystic mass in six patients and a cavitary mass in one and/or normal systemic arteries. The lesion was present in the right lung in four and in the left lung in three patients. The involved lobe was the upper in three, lower in three and middle lobe in one. Six patients underwent lobectomy and there was no associated malignancy or mortality. The mean length of hospital stay was 17.5 +/- 7.3 days. In adult patients who suffer from a recurrent productive cough and who have a multiloculated cystic mass in one lobe and normal vascular images in MDCT, CCAM, although rare, should be considered.