Cystic Adenomatoid Malformation Of Lung Research Articles

Clinicopathological and genetic characteristics of congenital cystic adenomatoid malformation of lung and its associated lung cancer in adults

Objective: To investigate the clinicopathological features and genetic characteristics of congenital cystic adenomatoid malformation (CCAM) of lung and CCAM associated lung cancer in adults. Methods: A total of 13 cases of CCAM of lung in adults, diagnosed from June 2015 to May 2023, were collected from the Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, China. Their histopathological features were correlated with probable development into lung cancer. Next-generation sequencing was performed on the benign and malignant areas of all cases. Results: The pathological classification of all cases were of CCAM of lung type 1. There were 4 male and 9 female cases, age ranged from 18 to 65 years, with a mean age of 41 years. Six cases were accompanied by lung cancer, all of them were mucinous adenocarcinoma. Next-generation sequencing showed no gene mutation in 2 of the 13 cases; KRAS mutations in exon 2 were detected in 7 cases, in which there were 6 cases complicated with lung mucinous adenocarcinoma and no matter in the malignant or benign regions, the same case exhibited the same mutation sites in KRAS gene. Conclusions: CCAM of the lung is a congenital disease, and in adults, type 1 is most commonly found in the pathological classification, and it is often accompanied by cancer. Gene mutations are frequently detected in CCAM of the lung, KRAS being the most recurrent mutation which may play an important role in the carcinogenesis.

Application and effects of an early childhood education machine on analgesia and sedation in children after cardiothoracic surgery

ObjectiveTo study the effect of an early childhood education machine on sedation and analgesia in children after cardiothoracic surgery.MethodsA prospective randomized controlled study was conducted in a provincial hospital in China. Fifty-two patients (aged from 1 to 5 years) underwent cardiothoracic surgery (including: ventricular septal defect, patent ductus arteriosus, atrial septal defect, pulmonary stenosis, pulmonary sequestration and congenital cystic adenomatoid lung malformation) were divided into the study group (n = 26) and the control group (n = 26). The patients in the study group underwent intervention with an early childhood education machine (uniform type) in addition to routine standard treatment and nursing, while the patients in the control group only received routine standard treatment and nursing. Richmond agitation sedation score (RASS) and face, legs, activity, cry, consolability (FLACC) score of all of the patients were evaluated, and the negative emotions (self-rating anxiety scale (SAS) score and self-rating depression scale (SDS) score) of the parents of the two groups were compared.ResultsThere was no significant difference in the general clinical data between the two groups. The RASS and FLACC scores in the study group were significantly lower than those in the control group, and the SAS and SDS scores of the parents in the study group were significantly lower than those in the control group.ConclusionThe application of an early childhood education machine for children after cardiothoracic surgery can effectively reduce postoperative agitation, improve sedation and analgesia of the patients, and ease the pessimistic mood of the patients’ parents.

Congenital Cystic Adenomatoid Malformation of Lung in an Infant

Congenital Cystic Adenomatoid Malformation (CCAM) is considered a hamartomatous lesion of lung with excessive proliferation of bronchial structure without alveoli. We have treated a child of 12 months who presented with difficulty in breathing after birth and chest x-ray PA view shows increase lucency in the right upper zone suggestive of CCAM. Patient underwent right upper lobectomy under general anaesthesia and now she is fine.
 JAFMC Bangladesh. Vol 15, No 2 (December) 2019: 235-236

The First of Congenital Cystic Adenomatoid Lung Malformation in Iraq: A Radiologic and Pathologic Study

Background: Congenital cystic adenomatoid lung malformation is a very rare condition characterized by replacement of normal pulmonary tissue with cysts of variable size and distribution. The condition is usually unilateral. Very few cases of bilateral and congenital cystic adenomatoid malformation with good outcome after resections of the lesions have been reported, and most cases were stillborn or died early during life. Patients and methods: A forty-day male infant presented with progressive respiratory distress since the first week of life observed at the Children Teaching Hospital of Baghdad Medical City was studied. The literature was reviewed with aim of describing the early documentation of the condition in the literature. Results: Clinically, the infant had significant respiratory distress interfering with feeding and evidence of shift of the mediastinum to the right. Chest X ray showed increased translucency of left lung field and mediastinal shit to the right. CT-scan showed large air-filled cyst in the left lung and small air-filled cyst in the right lung. The child was treated successfully by left upper lobecomy. The gross examination of two gray pieces of lung tissue showed on cut section microcysts that were apparent grossly. Microscopical examination showed intercommunicating cysts lined by cuboidal epithelium with gland like appearance confirming the diagnosis of congenital cystic adenomatoid malformation of the lung. Conclusion: A very rare case of bilateral congenital cystic adenomatoid malformation of the lung is reported and the early documentation of the condition in the literature is described.

Congenital cystic adenomatoid malformation of lung mimicking lung cancer: a case report

Abstract: We report a case of a 66-year-old woman with congenital cystic adenomatoid malformation (CCAM) that presented as a thin walled cyst on radiological imaging and mimicked lung cancer. The right pulmonary wedge resection was performed via thoracoscopic surgery. Pathologic results proved CCAM which though, uncommon may be misdiagnosed in adults.

A case of congenital cystic adenomatoid malformation of lung with poor outcome

This article describes a case of ultrasound diagnosis of congenital cystic adenomatoid lung malformation (CCALM) of the right lung type I at 31+2 weeks of gestation associated with mediastinum displacement, nonimmune hydrops and massive polyhydramnios. The volume of amniotic liquid was approximately 4000 ml. The newborn’s weight was 2800 g, heart failure and lung disorders were revealed. The diagnosis of CCALM was confirmed by pathologoanatomic examination.

CONGENITAL ADENOMATOID LUNG TYPE 1 MALFORMATION IN A NEWBORN

Представлен случай кистозно-аденоматоидной мальформации легкого у новорожденного. Описаны методы лучевой диагностики и оперативного лечения этой врожденной аномалии развития легких: ультразвуковое исследование плода в пренатальном периоде, рентгенография и компьютерная томография – в антенатальном.

Congenital cystic adenomatoid malformation of lung: Report of three cases and review of this rare entity

Congenital cystic adenomatoid malformations (CCAMs) are rare congenital, developmental, nonhereditary, hamartomatous pulmonary airway malformations. Etiology is unknown. It is a rare disease with an incidence of 1 in 11,000 to 1 in 35,000. It is a disease of infancy with most of the cases diagnosed within the first 2 years of life. We report cases of three infants with CCAM (Stocker Type II and I) with brief review of literature.

Late presentation of congenital cystic adenomatoid malformation of lung

Late presentation of congenital cystic adenomatoid malformation of lung

Congenital cystic adenomatoid malformation of lung in fetus: Report of two cases with brief review of literature

Congenital cystic adenomatoid malformations (CCAMs), also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology. It is a rare disease with an incidence of 1 in 25,000 to 1 in 35,000. It is a disease of infancy with most of the cases diagnosed within first 2 years of life. We report autopsy findings of two fetuses with CCAM (Stocker Type I and IV) with brief review of literature.

Congenital cystic adenomatoid malformation of lung in adult complicated by aspergilloma presenting as recurrent hemoptysis- 3 rd case report in world literature

CCAM is a congenital anamoly of lung, usually uncommon in adults. Presentation in adults is mostly as acute fever and lung abscesses, secondary to bacterial infections. We present a case of 28 year old man having CCAM of left lung ,complicated by aspergilloma ,presenting with recurrent hemoptysis. Patient underwent curative resection and is symptom free in follow up.

Congenital cystic adenomatoid malformation of lung: Report of two cases with review of literature

Congenital cystic adenomatoid malformations (CCAM) also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology. It is a rare disease with an incidence of 1 in 25,000 to 1 in 35,000. It is a disease of infancy with most of the cases diagnosed within first 2 years of life. We report autopsy findings of two fetuses with congenital cystic adenomatoid malformation (Stocker Type II and I) with brief review of literature.

Occlusive vascular Ehlers–Danlos syndrome accompanying a congenital cystic adenomatoid malformation of the lung: report of a case

An 8-year-old male presented with a cystic lung lesion in the left lower lobe, which was initially detected during surgery for a spontaneous rupture of the sigmoid colon at the age of 6 years. Tissue fragility and a tendency to bleed easily were noted during the surgery, which strongly suggested vascular Ehlers-Danlos syndrome. Although there was no abnormality in the hemostasis screening test, or any suspicious hereditary problem in his pedigree, genetic gene testing for vascular Ehlers-Danlos syndrome was recommended, and showed a de novo mutation in the COL3A1 gene. This report presents the case of patient with occlusive vascular Ehlers-Danlos syndrome accompanying a congenital cystic adenomatoid malformation of lung, in addition to a duplicated infrarenal vena cava.

Congenital cystic adenomatoid malformation of lung in adults: 2 rare cases report and review of the literature

Congenital cystic adenomatoid malformation (CCAM), also named congenital pulmonary airway malformation (CPAM), is a congenital abnormality of lung which is uncommon in adults. Here we present 2 adult cases of CCAM with unusual clinical and pathologic findings. One case was complicated with aspergillosis which was seldom reported. The other case was suffered bilateral lesions and the patient's mother had been previously radiographically discovered bilateral cystic lesions that CCAM could not be ruled out. A review of currently published related literatures has also been provided.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/6406766736634578.

Antenatal and postnatal management of congenital cystic adenomatoid lung malformation diagnosed by ultrasound and Magnetic Resonance Imaging (MRI)

Purpose: Antenatal diagnosis of congenital cystic adenomatoid lung malformation (CCAM) is vital for disease surveillance and postnatal care. Ultrasonography (US) has been the imaging gold standard for antenatal CCAM assessment. However, one of the limitations of US is the “vanishing phenomenon” caused by isoechogenicity of CCAM tissue and adjacent normal lung parenchyma. Methods: Antenatal serial US were concurrently used with magnetic resonance imaging (MRI) to monitor macro- and microcystic lesions. Results: In both pregnant women, antenatal US and MRI confirmed the presence, in the fetus, of cystic lesions and predicted disease regression/progression as well as the need for postnatal surgical intervention. Several advantages were detected by using both—serial US and MRI (over serial US alone)—including improved signal intensity, exact volume size measurements, precise CCAM location in particular for patients with adverse ultrasound conditions. Both neonates underwent surgical resection and had an uneventful post-operative course. Conclusions: Antenatal use of MRI as well as serial US improved information regarding tissue resolution and delineation of CCAM. The information from two imaging modalities was complementary. Our literature review confirmed the emerging role of prenatal MRI for postnatal monitoring and management of CCAM.

Congenital Cystic Adenomatoid Malformation: Is There a Need for Pregnancy Termination?

Aim. Congenital cystic adenomatoid lung malformation is a rare unilateral dysplasia of the lung. Three pathologic types are described in the literature: type I with cysts >2 cm, type II with cysts <1 cm, and type III with microcysts. The aim of this paper is to present a case of a fetus with congenital cystic adenomatoid lung malformation and discuss the necessity for pregnancy termination according to its prognosis and future mortality. Case. A 36-year-old pregnant woman (para: 1, gravida: 1) presented in our department for anatomy ultrasound screening at 20 + 1 weeks of gestation. The ultrasound detected a cystic adenomatoid right lung malformation measuring 1.45 × 1.67 cm which caused mediastinal shift of the heart and the lung to the left side. Other findings were cysts of the choroid plexus and echogenic intracardiac foci. The parents after genetic counseling decided pregnancy termination. The pregnant received cabergoline for ablactation. Conclusion. Congenital cystic adenomatoid lung malformation has different prognosis according to the type (69% in type I, 0% in types II and III). Fetal hydrops, cardiac and skeletal anomalies, Potter's syndrome, and gastrointestinal atresia are common cofindings. Genetic counseling is necessary, and pregnancy termination is proposed to the cases with poor prognosis.

Malignancy of Congenital Cystic Adenomatoid Malformation of Lung in Aged

Congenital cystic adenomatoid malformation is rare in adults, and often asymptomatic. We describe a case of malignant degeneration of a congenital cystic adenomatoid malformation in a 77-year-old man. Congenital cystic adenomatoid malformation must be considered a premalignant lesion, even in older patients. Rigorous preoperative staging must be undertaken. Anatomic resection with extensive lymphadenectomy remains the treatment of choice.

Adult congenital cystic adenomatoid malformation of lung co-existing with carcinoid: report of a case

患者女,66岁.因反复咯血、咳嗽40余年,再发10 d于2005年11月7日入院.胸部MRI示左肺下叶大部分肺段见大小不一囊状影,囊壁厚,囊内未见明显液平面(图1);右肺下叶、中叶部分肺段见多个囊状影,壁薄,未见液平.考虑原发性囊状支气管扩张,不除外肺囊肿.术中见左肺下叶稍粘连,肺裂发育不好,泛红,含气不全。

Congenital Cystic Adenomatoid Malformation of Lung in Adults: Clinical, Pathologic and Radiologic Evaluation of Six Patients

Background: Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare congenital developmental anomaly of the lower respiratory tract. Most cases are diagnosed within the first 2 years of life, so adult presentation of CCAM is rare. We describe here six adult cases of CCAM and the patients underwent surgical resection, and all these patients were seen during a five and a half year peri od. The purpose of this study was to analyze the clinical, radiological and histological characteristics of adult patients with CCAM. Methods: Through medical records analysis, we retrospectively reviewed the clinical characteristics, the chest pictures (X-ray and CT) and the histological characteristics. Results: Four patients were women and the mean age at diagnosis was 23.5 years (range: 18∼39 years). The major clinical presentations were lower respiratory tract infection, hemoptysis and pneumothorax. According to the chest CT scan, 5 patients had multiseptated cystic lesions with air fluid levels and one patient had multiple cavitary lesions with air fluid levels, and these lesions were surrounded by poorly defined opacities at the right upper lobe. All the patients were treated with surgical resection. 5 patients underwent open lobectomy and one patient underwent VATS lobectomy. On the pathological examination, 3 were found to be CCAM type I and 3 patients were CCAM type II, according to Stocker 's classification. There was no associated malignancy on the histological studies of the surgical specimens. Conclusion: As CCAM can cause various respiratory complications and malignant changes, and the risks associated with surgery are extremely low, those patients who are suspected of having or who are diagnosed with CCAM should go through surgical treatment for making the correct diagnosis and administering appropriate treatment. (Tuberc Respir Dis 2008;65:110-115)

Antenatal Diagnosis and Outcome of 12 Congenital Cystic Adenomatoid Malformation of Lung

Objective To investigate the ultrasonic detection probability, type, prenatal diagnosis, and outcome of congenital cystic adenomatoid malformation of lung (CCAM). Methods In this retrospective study, all 12 cases which were diagnosed with suspected prenatal CCAM over the period in the hospital were analyzed. Information on diagnosis time, types and progression of the lesions during pregnancy, the additional abnormalities, and the outcome of pregnancies were recorded. Results The positive rate of ultrasonic detection of CCAM was about 1.01‰ (1/11 124) before 28 gestation weeks. There were 1 case of type I (8%), 2 cases of type II (17%) and 9 cases of type III (75%). Nine pregnancies were terminated and 2 cases were confirmed by pathology. Three neonates were alive and without any symptom now. Conclusions Type III is the major type. Ultrasound examination during 20–28 weeks and following up, prenatal consultation, chromosome examination of fetus, delivery with a pediatrician standing by, and all suspected neonates being investigated are recommended.