Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma

Priest, John R.; Magnuson, Jeffrey E.; Williams, Gretchen M.; Abromowitch, Minnie; Byrd, Rebecca L.; Sprinz, Philippa G.; Finkelstein, Marsha; Moertel, Christopher L.; Hill, D. Ashley

doi:10.1002/pbc.20937

Cited by 75 publications

(76 citation statements)

References 42 publications

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“…49,62 Finally, as PPB develops its more overtly ''malignant'' pathologic features, it acquires the capacity for metastatic spread, especially to the central nervous system. 63 Type I PPB has not been observed to metastasize; Types II and III PPB metastasize in approximately 10% and 55% of cases, respectively. 63 Understanding this evolutionary sequence of PPB underscores the importance of recognizing PPB in its earliest cystic stage.…”

Section: Progression Of Type I Cystic Ppbmentioning

confidence: 90%

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Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

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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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Section: Progression Of Type I Cystic Ppbmentioning

confidence: 90%

“…63 Type I PPB has not been observed to metastasize; Types II and III PPB metastasize in approximately 10% and 55% of cases, respectively. 63 Understanding this evolutionary sequence of PPB underscores the importance of recognizing PPB in its earliest cystic stage.…”

Section: Progression Of Type I Cystic Ppbmentioning

confidence: 90%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

Self Cite

271

215

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“…Recently, a similar correction was reported in Pediatric Blood and Cancer and the importance of identifying PPB was stressed [4]. The treatment approaches for PPB and RMS are similar, but unlike RMS, PPB has a propensity for brain metastasis [2]. In addition, PPB is a strikingly familial cancer with genetic implications for others in the immediate and extended families [5].…”

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confidence: 80%

“…Although these patients received initial complete surgery, postoperative chemotherapy and local radiotherapy, the clinical evolution of the tumor was fatal in both cases. One of these children died from brain metastases, a unique and common complication of PPB [2]. The other child died from respiratory distress after relapse.…”

mentioning

confidence: 99%

Primary pulmonary rhabdomyosarcoma in childhood: Clinico‐biologic features in two cases with review of the literature—erratum

Schiavetti

Indolfi²,

Hill

et al. 2008

Pediatric Blood & Cancer

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, we reported on two cases of rhabdomyosarcoma (RMS) of the lung arising from normal lung tissue without previous cystic malformations [1]. The histology of these two cases was recently reviewed by the consultants of the International Pleuropulmonary Blastoma Registry. Together with those consultants, we want to amend the original report. After pathologic review, these cases are now classified as Type III pleuropulmonary blastoma (PPB) rather than primary pulmonary RMS. Although these patients received initial complete surgery, postoperative chemotherapy and local radiotherapy, the clinical evolution of the tumor was fatal in both cases. One of these children died from brain metastases, a unique and common complication of PPB [2]. The other child died from respiratory distress after relapse. Microscopically, both tumors contained a rhabdomyosarcomatous element, but both also had blastematous and spindle cell sarcomatous areas. The rhabdomyosarcomatous areas in PPB are identical to embryonal RMS. The presence of high grade blastemal elements, cartilage, spindle cell sarcoma and/or diffuse, marked anaplasia should suggest a diagnosis of PPB for lung masses in children 6 years of age or under [3]. Recently, a similar correction was reported in Pediatric Blood and Cancer and the importance of identifying PPB was stressed [4]. The treatment approaches for PPB and RMS are similar, but unlike RMS, PPB has a propensity for brain metastasis [2]. In addition, PPB is a strikingly familial cancer with genetic implications for others in the immediate and extended families [5]. We appreciate the opportunity to correct the histopathology of these two cases as the importance of the distinction between PPB and RMS is now more evident 12 years after the original report.

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“…The most common location for distal metastasis is the brain, followed by bone. Cerebral metastases are much more frequent in PPB than in other childhood sarcomas [ 56 ]. Metastases to the liver, adrenal glands, and ovary have also been reported [ 47 , 49 ].…”

Section: Imaging Featuresmentioning

confidence: 98%