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.

Congenital Cystic Adenomatoid Malformation, Type II: A Rare Cause of Haemoptysis

Congenital cystic adenomatoid malformation (CCAM) occurs secondary to the cystic adenomatous over-growth of terminal bronchioles, which results in the secondary inhibition of alveolar growth. In most of the cases, respiratory distress is the presenting feature during the neonatal period. In about 90% of patients, recurrent respiratory infections necessitating chest imaging reveal CCAM before the age of two years. We describe here the occurrence of congenital cystic adenomatoid malformation of right lung in a 12-year-old girl presenting with haemoptysis and hypovolaemic shock. She underwent right middle lobectomy; and histopathological examination confirmed the diagnosis. She has been doing well on follow-up.

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.

DIAGNOSIS AND TREATMENT POLICY FOR SEVERE CONGENITAL CYSTIC ADENOMATOID MALFORMATION OF THE LUNG IN NEWBORN INFANTS

Objective. To present the diagnosis of and treatment policy for severe cystic adenomatoid malformation (CAM) of the lung in newborn infants, allowing one to rule out a fatal outcome and to ensure good quality of life in the future. Material and methods. The authors describe their own 12 clinical cases in newborn infants with the histologically verified diagnosis of type 1 CAM with a pronounced mass effect, as well as pregnancy management tactics, the time and algorithm of postnatal radiation examination, a treatment method and give long-term results. Results. The traced long-term results are predominantly good (77.8%); there are no poor outcomes or mortality in this patient group. Conclusion. The prognosis for life in severe forms of CAM with a high risk of antepartum fetal demise can be favorable if there is a correct pregnancy management tactics, early examination, and surgical treatment. Computed tomography and computed tomographic angiography are main postnatal diagnostic techniques that determine the further tactics of a neonatal surgeon. Emergency surgery is indicated after the diagnosis of type 1 CAM with a pronounced mass effect is confirmed.

Cystic adenomatoid malformation of the lung associated with trysomy 18

Cystic adenomatoid malformation of the lung (CAML) is a rare lesion with a common incidence of 1/25000 livebirths to 1/33000 livebirths. We present a case of a 22 weeks pregnancy with CAML type III, a hybrid lesion and no other abnormality associated with trisomy 18. In CAML hybrid lesion the feeding blood supply was derived from the systemic circulation mainly aorta, in contrast with the clasical CAML lesion in which the blood supply is derived from pulmonary vessel. Also, CAML lesion do not have a known genetic defect responsible for its appearence, but an early developmental deficiency of an unknown cause. The association between CAML lesion and chromosomal abnormality is very rare and in reports were it was detected a slightly increase minor risc, there were present also some extrapulmonary abnormalities.

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.

嚢胞羊水腔シャントチューブ留置術を行い生児を得た胎児水腫合併macrocystic congenital cystic adenomatoid malformationの1例

胎児水腫を合併した先天性肺嚢胞性腺腫様形成異常（Congenital cystic adenomatoid malformation: CCAM）を待機的に管理した場合の予後は不良である．また，CCAM volume ratio（CVR）が1.6を超えるものが胎児水腫発症のリスクであることも知られている．今回我々は，妊娠20週で胎児水腫を呈したものの，妊娠22週で胎児嚢胞羊水腔シャントチューブ留置術を施行し嚢胞の縮小と胎児水腫の改善に成功，生児を得たmacrocystic CCAMの症例を経験した．本症例では初回治療時のCVRが2.0と高値であり，胎児胸腔内をほぼ病変が占拠する状態で，胎児水腫も来していた．嚢胞のドレナージが有効であり，心臓の圧排が解消されるとともに胎児水腫が消失し，生児を得ることができた．ただ，macrocystic CCAMでありながら嚢胞の数が多く，シャント術を行って2つの嚢胞を消失させることに成功したものの，ドレナージされていない複数の嚢胞がトータルとしてCVR換算で0.6-0.8程度で残存した．生後は正常肺が十分に膨らむに至らず，肺高血圧治療に難渋することとなった．胎児水腫を合併したmacrocystic CCAMに対するシャントチューブ留置術の循環動態改善効果は示されたが，多房性のmacrocystic CCAMにおいては，肺低形成の予防の観点から胎児治療の適応と方法をさらに検討する必要があると考えた．

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 with Associated Mucinous Bronchioloalveolar Carcinoma in a Neonate

Congenital cystic adenomatoid malformation (CCAM) of lung is a rare hamartomatous disorder characterized by abnormal branching morphogenesis of the lung. We report an unusual case of a 2-day-old male newborn with a pulmonary cystic lesion and lobectomy revealed a CCAM of the lung that has overlapping features of type 1 and type 2, complicating with multifocal mucinous bronchioloalveolar carcinoma (BAC). The case indicates that malignant transformation can occur in very early stage of the infancy in the patients with CCAM of lung.

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.

Unusual Presentation of Congenital Cystic Adenomatoid Malformation of the Lung

Unusual Presentation of Congenital Cystic Adenomatoid Malformation of the Lung

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.