Mesenchymal neoplasia and congenital pulmonary cysts

Weinberg, Arthur G.; Currarino, Guido; Moore, G. C.; Votteler, Theodore P.

doi:10.1007/bf01464317

Cited by 49 publications

(13 citation statements)

References 9 publications

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“…chondrosarcoma and fibrosarcoma which developed in a unilocular cyst, described as ªmalignant mesenchymomaº (15). In 1982, Weinblatt first noted the relationship between these kinds of tumors of mesenchymal origin and pulmonary cystic diseases (16).…”

Section: Discussionmentioning

confidence: 99%

Pleuropulmonary Blastoma in Congenital Cystic Adenomatoid Malformation: Report of a Case

Federici¹,

Domenichelli²,

Tani³

et al. 2001

Eur J Pediatr Surg

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Pulmonary blastoma is a rare malignant tumor seen in both adults and children. Approximately only 25% of cases occur in pediatric patients, many of whom affected by a congenital pulmonary cystic lesion. The clinical features, radiological findings and management of a 3-year-old boy affected by a pulmonary blastoma which arose in a congenital cystic adenomatoid malformation are reported, and an extensive review of the literature is also made. Because of the well-known tendency of cystic pulmonary diseases to develop malignancies, authors recommend the surgical excision of these kind of lesion or at least their close radiological follow-up.

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Section: Discussionmentioning

confidence: 99%

Pleuropulmonary Blastoma in Congenital Cystic Adenomatoid Malformation: Report of a Case

Federici¹,

Domenichelli²,

Tani³

et al. 2001

Eur J Pediatr Surg

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“…8). 54,60,61 It is very likely that cysts which preceded the diagnosis of advanced PPB in these children were unrecognized Type I PPB.…”

Section: Progression Of Type I Cystic Ppbmentioning

confidence: 98%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

266

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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“…Before 1988, the terminology used for these neoplasms included the following: pulmonary blastoma (a true congenital neoplasm), pulmonary blastoma associated with cystic lesions, childhood pulmonary blastoma, pulmonary sarcoma arising in mesenchymal cystic hamartoma, mesenchymal cystic hamartoma, embryonal sarcoma, rhabdomyosarcoma arising in congenital cystic adenomatoid malformation, and sarcoma arising in bronchogenic cyst. [42][43][44][45][46][47] Manivel and coworkers 48 coined the term pleuropul monary blastoma in their report of 11 pediatric tumors that involved not only the lung but also the pleura and mediastinum. …”

Section: Historical Aspectsmentioning

confidence: 99%

“…Patients may present with clinical signs and symptoms of cough, fever, chest pain, lethargy, respiratory distress, anorexia, and weight loss. 46 Radiologically, partial or total opacification of the lung field and mediastinal shifting, pleural effusion, and hydropneumothorax have been observed. 48 Although the tumor usually manifests as a unilateral pulmonary mass, one case of bilateral cystic pleuropulmonary blastoma has been reported.…”

Section: Clinical Featuresmentioning

confidence: 99%

See 1 more Smart Citation

Biphasic Tumors of the Lung

2010

Tumors and Tumor-Like Conditions of the Lung and Pleura

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Mesenchymal neoplasia and congenital pulmonary cysts

Cited by 49 publications

References 9 publications

Pleuropulmonary Blastoma in Congenital Cystic Adenomatoid Malformation: Report of a Case

Pleuropulmonary Blastoma in Congenital Cystic Adenomatoid Malformation: Report of a Case

Pulmonary cysts in early childhood and the risk of malignancy

Biphasic Tumors of the Lung

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