Primary pulmonary rhabdomyosarcoma arising within cystic adenomatoid malformation: A case report and review of the literature

Özcan, Coşkun; Çelik, Ahmet; Ural, Zeliha; Veral, Alı; Kandıloğlu, Gülşen; Balιk, Erol

doi:10.1053/jpsu.2001.24747

Cited by 122 publications

(58 citation statements)

References 25 publications

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“…Although a definitive connection between bronchial atresia with cystic changes and the complications associated with CCAMs has not been evaluated, the frequent concurrence of PBA and microcystic maldevelopment characteristic of CCAMs suggests that one consider the management of asymptomatic PBA in the context of a CCAM diagnosis. Many believe surgical resection of asymptomatic CCAMs is appropriate due to the increased risk of pneumothorax, infection and malignant degeneration [6,7,[16][17][18][19][20][21][22] . In most cases, malignancies consist mainly of pleuropulmonary blastoma in infants and young children and bronchoalveolar carcinoma in older children and adults.…”

Section: Discussionmentioning

confidence: 99%

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

et al. 2008

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Objective: Peripheral bronchial atresia (PBA), a newly identified fetal lung lesion, is often asymptomatic and managed nonoperatively. However, recent studies suggest that bronchial atresia plays a role in the etiology of microcystic maldevelopment present in congenital cystic adenomatoid malformations (CCAM) which require resection to decrease the risk of infection, pneumothorax and malignant degeneration. The purpose of this study was to evaluate the prenatal radiographic and postnatal computed tomography (CT) scan/pathology findings with attention to the pathologic diagnosis of microcystic maldevelopment in infants with the presumptive diagnosis of PBA. Methods: A retrospective review of prenatal and postnatal records of patients diagnosed with fetal lung lesions was performed. Two groups of patients were identified: (1) patients diagnosed with PBA on postnatal CT scan (n = 16), and (2) patients with the pathologically confirmed diagnosis of PBA independent of postnatal CT findings (n = 23). Results: Prenatal ultrasound diagnosis of these lesions included CCAMs, hybrid lesions, bronchopulmonary sequestrations and bronchial atresia. Eleven of the 16 patients in group 1 with the postnatal radiologic diagnosis of PBA underwent surgical resection, 6 of which were found to have microcystic changes consistent with CCAM. Evaluation of the 23 patients in group 2 with pathologically confirmed PBA identified 65% that had evidence of microcystic maldevelopment consistent with the small cyst type of CCAM. Conclusion: Radiographically diagnosed PBA as well as pathologically confirmed PBA is frequently associated with microcystic changes consistent with the small cyst type of CCAM. Thus, operative management should be considered for PBA to decrease CCAM-associated risks.

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Section: Discussionmentioning

confidence: 99%

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

et al. 2008

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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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“…9,10 Esto se da rara vez en las dos primeras décadas de la vida, pero se ha informado sobre pacientes de hasta 13 meses de edad que han desarrollado neoplasias. 11 Un metanálisis sobre el manejo posnatal de las lesiones congénitas quísti-cas pulmonares concluyó que, si bien el riesgo de presentar síntomas en los pacientes asintomáticos es bajo, la cirugía programada se asocia a mejores resultados que si se realiza en pacientes sintomá-ticos, y sugiere que la intervención se practique antes de los 10 meses, ya que esta es la edad promedio del inicio de los síntomas. 12 …”

Section: Discussionunclassified

Neumotórax hipertensivo como forma de presentación de una malformación adenomatoidea quística pulmonar

Pumara¹,

Ma²

2013

Arch Argent Pediat

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Arch Argent Pediatr 2013;111(2):e54-e57 / e54 RESUMENEl propósito de este trabajo es examinar el caso de un paciente con una forma de presentación no habitual y grave de una patología poco frecuente, como la malformación adenomatoidea quística pulmonar. Se trata de un lactante de 50 días de vida que ingresó en la unidad de cuidados intensivos pediátricos con insuficiencia respiratoria y colapso circulatorio refractario a la expansión de volumen y los inotrópicos. La radiografía de tórax mostró una imagen radiolúcida, homogénea, en la base pulmonar derecha, que parecía corresponder a una lesión bullosa en el lóbulo inferior derecho, asociada a neumotórax homolateral. Se realizó drenaje con colocación de un tubo de avenamiento pleural y se observó una mejoría clínica posterior. Se efectuó la resección del quiste mediante lobectomía inferior derecha; el diagnóstico histopatológico fue malformación adenomatoidea quística de la vía aérea de tipo 4 (clasificación de Stocker). El paciente evolucionó favorablemente. Palabras clave: malformación adenomatoidea quística congénita del pulmón, neumotórax, lactante. SUMMARYThe purpose of this paper is to examine the case of a 50-day-old patient with an unusual and severe presentation of a rare disease: congenital cystic adenomatoid malformation of the lung. The infant was admitted to the pediatric intensive care unit with respiratory failure and circulatory collapse refractory to intravenous fluids and inotropic drugs. Chest X-ray showed a radiolucent homogeneous image in the right lung base, suggesting bullous lesion of the right lower lobe associated with ipsilateral pneumothorax. Drainage was performed by placing a chest tube with subsequent clinical improvement. Later the cyst was resected by right lower lobectomy; the histopathological diagnosis was congenital cystic adenomatoid malformation type 4 (Stocker classification). The patient evolved favorably.

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Primary pulmonary rhabdomyosarcoma arising within cystic adenomatoid malformation: A case report and review of the literature

Cited by 122 publications

References 25 publications

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

Pulmonary cysts in early childhood and the risk of malignancy

Neumotórax hipertensivo como forma de presentación de una malformación adenomatoidea quística pulmonar

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