Rhabdomyosarcoma arising within congenital pulmonary cysts: Report of three cases

Murphy, James J.; Blair, Geoffrey K.; Fraser, Graham C.; Ashmore, P. G.; LeBlanc, Jacques G.; Sett, Suvro; Rogers, Paul C.; Magee, J.Fergall; Taylor, Glenn; Dimmick, James E.

doi:10.1016/0022-3468(92)90299-m

Cited by 147 publications

(56 citation statements)

References 19 publications

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“…Foram sugeridas duas principais hipóteses em relação à origem do rabdomiossarcoma em associação à MACC. É possível que ocorra a partir de fibras musculares esqueléticas bem diferenciadas, mas com organização deficiente, presentes em algum subtipo de MACC ou por diferenciação rabdomioblástica da camada do câmbio 23,24 .…”

Section: Discussionunclassified

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

Giubergia¹,

Barrenechea²,

Siminovich³

et al. 2012

J Pediatr (Rio J)

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Objective: Congenital cystic adenomatoid malformation (CCAM) is the most common surgically resected pulmonary malformation in children. This retrospective study was undertaken to present the experience of 172 CCAM cases in a pediatric hospital. Methods:Published series with a small number of patients reports details of lesions, progress and management. As this study addresses clinical characteristics, progress and surgical procedures in 172 children with CCAM diagnosis, the population includes cases treated and followed up in a pediatric hospital throughout 25 years .Results: Mean age at diagnosis was 48 months (r = 0.03-213), 52% (n = 90) were male. The most common presenting symptoms were respiratory distress in children under 6 months of age (40%) and recurrent pneumonia in older ones (75%; p = 0.001). Lobectomy was the procedure of choice in the majority. All histological types were found: 1 (70%), 2 (24%), 4 (4%), and 0 and 3 (n = 1). A mixed pattern was observed in nine patients. Associated anomalies were found in 47% of children. The most frequent was sequestration (71%), mostly present in CCAM type 2 (p = 0.001). Severe anomalies were mostly related to type 2 (p = 0.008). A pleuropulmonary blastoma and a bronchioloalveolar carcinoma were also observed. Mortality was 5% (n = 9). Risk factors for mortality were respiratory failure ; p = 0.03), sepsis (OR = 9.9 [95%CI 8.2-12]; p = 0.002), respiratory assistance requirements (OR = 9.5 [95%CI 2.3-37]; p = 0.04), and severe associated comorbidities (OR = 3.3 [95%CI 1.2-22]; p = 0.008). Conclusions:Related anomalies were observed in almost half of the population. Due to the possibility of recurrent infection or development of malignancies, surgical resection should be considered when CCAM is diagnosed. Surgical outcome is favorable with manageable complications. J Pediatr (Rio J). 2012;88(2):143-8:Congenital cystic airway malformation, associated malformations, surgical procedures, children. ResumoObjetivo: A malformação adenomatoide cística congênita (MACC) é a malformação pulmonar ressecada cirurgicamente mais comum em crianças. Este estudo retrospectivo foi realizado para apresentar a experiência de 172 casos de MACC em um hospital pediátrico.Métodos: Séries publicadas com um pequeno número de pacientes relatam detalhes de lesões, evolução e tratamento. Como este estudo lida com características clínicas, evolução e procedimentos cirúrgicos em 172 crianças com diagnóstico de MACC, a população inclui casos tratados e acompanhados em um hospital pediátrico ao longo de 25 anos .Resultados: A idade média ao diagnóstico foi de 48 meses (r = 0,03-213), 52% (n = 90) eram do sexo masculino. Os sintomas de apresentação mais comuns foram dificuldade respiratória em crianças com menos de 6 meses de idade (40%) e pneumonia recorrente nas que tinham mais idade (75%; p = 0.001). Lobectomia foi o procedimento de escolha na maioria dos casos. Todos os tipos histológicos foram encontrados: 1 (70%), 2 (24%), 4 (4%) e 0 e 3 (n = 1). Foi observado um padrão misto em nove...

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Section: Discussionunclassified

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

Giubergia¹,

Barrenechea²,

Siminovich³

et al. 2012

J Pediatr (Rio J)

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“…rhabdomyosarcoma in children [27][28][29][30] and mucinous BAC (mostly in adults) [31][32][33][34][35][36][37][38][39][40]. These associations have been significantly underappreciated and it is unlikely that they represent random associations or publication bias.…”

Section: From Cpam To Bac and Acmentioning

confidence: 99%

From cystic pulmonary airway malformation, to bronchioloalveolar carcinoma and adenocarcinoma of the lung

Ioachimescu

Mehta²

2005

European Respiratory Journal

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Bronchioloalveolar carcinoma (BAC) of the lungs is a known morphological subtype of nonsmall cell cancer. The current study presents several carcinogenetic theories of BAC and the possible relationship with atypical adenomatous hyperplasia and congenital pulmonary airway malformation (CPAM).The authors present an unusual case of BAC developed in an area of CPAM, with subsequent progression to metastatic adenocarcinoma (AC). The case is unique due to the combination of: early age of presentation; neoplastic transformation of a CPAM; unaltered course over 15 yrs; and its particular pattern of slow morphogenesis and degeneration into an invasive AC of the lung.The case also presents the unique features of a long-standing, unaltered natural course of paediatric BAC towards invasive and metastatic AC, illustrating that lack of growth over many years cannot be entirely trusted as a criterion of benignity.In conclusion, clinicians and pathologists need to be aware of the fact that congenital pulmonary airway malformation so far represents the only known pre-invasive lesion for mucinous bronchioloalveolar carcinoma.KEYWORDS: Adenocarcinoma, atypical adenomatous hyperplasia, bronchioloalveolar carcinoma, carcinogenesis, congenital pulmonary airway malformation, cystic adenomatoid malformation CASE HISTORY A 6-yr-old male presented in 1988 with recurrent episodes of fever, left-sided pleuritic chest pain, cough with significant bronchorrhea, intermittent haemoptysis, fatigue, anorexia and weight loss. He was diagnosed with left lower lobe pneumonia and treated with antibiotics three times over a period of 7 months. During the last episode, the chest radiograph showed an infiltrate and airfluid level in the left lower lobe. He was hospitalised and started on antibiotic therapy. He had a negative tuberculin test, fungal serologies and bronchoalveolar lavage cultures for bacteria, acid-fast bacilli and fungi. A thoracotomy with left-lower lobe lobectomy was performed; macroscopically, the lesion resembled a pneumonic process or a congenital malformation (intra-lobar sequestration with a large secondary abscess versus a congenital cystic malformation). Microscopic examination of the resected lung revealed multicentric mucinous-type bronchioloalveolar carcinoma (BAC) developed in an area of congenital adenomatoid malformation (CAM), or congenital pulmonary airway malformation (CPAM) type I, without pleural involvement. No abscesses were found. Synchronous nodules in the right upper, middle, lower and left-lower lobes were present and surgically explored. A right-middle lobe wedge resection biopsy revealed similar lesions of BAC. Chemotherapy was offered, but in light of poor predicted prognosis, the patient's family refused any therapeutic modality.During the follow-up, the patient had intermittent chest pains, but no other symptoms. He finished school with good grades, smoked cigarettes infrequently and continued to live an active life for the following 15 yrs. Follow-up chest radiographs and computed tomography (CT...

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“…19 The risk of malignancy in early childhood lung cysts has not been thoroughly addressed in the pediatric pulmonology literature. Until the recognition of the purely cystic PPB, the association of malignancy with alleged ''congenital lung cysts'' was confined to case reports of rhabdomyosarcomas [20][21][22][23] or carcinomas associated with cysts. 24,25 Early and even recent reports 26,27 of ''rhabdomyosarcoma in lung cysts'' almost certainly are cases of cystic PPB progressing to advanced PPB.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

264

215

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Summary. Surgery for congenital and early childhood lung cysts is often dictated by symptoms such as respiratory distress, infection or pneumothorax. Asymptomatic cysts present a therapeutic dilemma: surgical intervention and ''conservative'' observation have advocates. The risk of malignancy in such cysts is considered by some an indication for surgical intervention and is reviewed in this paper. Pleuropulmonary blastoma (PPB) is the most frequent malignancy associated with childhood lung cysts. Although rare, PPB occurs predictably in certain clinical and familial situations. This unique biology of PPB can inform the cyst management decision. The earliest manifestation of PPB is a malignant lung cyst in young children, clinically and radiographically indistinguishable from benign congenital lung cysts. Histopathologic examination differentiates cystic PPB from the benign cystic variants. Surgical excision of cystic PPB (with or without chemotherapy) cures approximately 85-90% of children. If not excised, cystic PPB evolves to cystic/solid or solid high-grade sarcoma (cure rate 45-60%) by age 2-6 years. Numerous reports of ''malignancy in a congenital lung cyst'' are now understood as the characteristic progression of cystic PPB. PPB is genetically determined in many cases. Detailed family history may reveal the hallmarks of PPB in the patient or young relatives: a unique constellation of diseases including lung cysts, cystic nephroma, childhood cancers, stromal sex-chord ovarian tumors, seminomas or dysgerminomas, intestinal polyps, thyroid hyperplasias, and hamartomas. Pneumothorax and multifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst is more likely PPB than a benign congenital cyst. Patients fitting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

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Rhabdomyosarcoma arising within congenital pulmonary cysts: Report of three cases

Cited by 147 publications

References 19 publications

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

From cystic pulmonary airway malformation, to bronchioloalveolar carcinoma and adenocarcinoma of the lung

Pulmonary cysts in early childhood and the risk of malignancy

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