Correction: Pleuropulmonary blastoma, not rhabdomyosarcoma in a congenital lung cyst
| Autor: | John R. Priest, Shun Chen Huang, D. Ashley Hill, Wan-Ting Huang, Chih Cheng Hsaio, Hock Liew Eng, Shin Yi Lee, Louis P. Dehner, Shyun Pai |
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| Rok vydání: | 2006 |
| Předmět: |
Male
Pathology medicine.medical_specialty Lung Neoplasms Pleuropulmonary blastoma Metastasis Cystic Adenomatoid Malformation of Lung Congenital medicine Humans Cyst Rhabdomyosarcoma Embryonal Diagnostic Errors Rhabdomyosarcoma Lung business.industry Hematology medicine.disease Prognosis respiratory tract diseases Pulmonary Blastoma medicine.anatomical_structure Oncology Dysplasia Child Preschool Pediatrics Perinatology and Child Health Blastoma Neoplasm Recurrence Local business |
| Zdroj: | Pediatric bloodcancer. 48(3) |
| ISSN: | 1545-5009 |
| Popis: | TotheEditor:In2005,we(SP,S-CH)reportedachildwitha resected pulmonary congenital cystic adenomatoid mal-formation (CCAM) who later developed a botryoid embry-onal rhabdomyosarcoma (RMS) at the site of the lung cyst[1]. We have since shared our case with the InternationalPleuropulmonary Blastoma Registry. Together with thoseconsultants,wewanttocorrecttheoriginalreport.Thecaseisan example of pleuropulmonary blastoma (PPB) rather thanRMS at the site of a lung cyst.PPB Registry pathologists (DAH, LPD) reviewed theclinical case and histologic sectionsfrom all three surgeries.Clinical findings and pathology from the initial surgerysupport the diagnosis of PPB in its cystic early form, Type IPPB (Fig. 1, original report). Beneath the benign lining ofthe multilocular cystic lesion is a condensed layer of smallproliferatingcellswitharhabdomyosarcomatousphenotype.Ayearlater,apredominantlysolidneoplasmemerged,whichhad a blastemal appearance in areas and spindle elementsintermixed with the rhabdomyosarcomatous component(Fig. 2, original report). The post-chemotherapy specimenrevealschemotherapyeffectandresidualrhabdomyoblasts,anodule of primitive cartilage and areas with a chondroidappearance.TheblastemalandchondroidareasdifferentiatePPB from typical embryonal RMS. Including the clinicalprogressionfromacysticTypeIlesiontotheemergenceofacomplex solid Type II/III neoplasm, the overall findings aretypical of PPB.TypeIPPBisasingleormoreoftenamultilocularcystinperipherallungparenchymafeaturingaprimitivemesenchy-mal-cellproliferation(cambiumlayer)inthestromabeneatha benign epithelial cyst lining [2] (Fig. 1, original report).The proliferation may be subtle in the absence of overtrhabdomyoblastic differentiation. It is often mistaken bothclinically and radiographically for a benign congenital cyst,inparticularCCAMtype4[3],asdiscussedelsewhere[4–6].Upon reevaluation, many such cases in the literature arediagnosed PPB (data available from the PPB Registry).Recurrences of Type I PPB are usually Type II and IIIdisease. The solid components of Types II and III PPB area collage of fibrosarcoma-like, blastemal, cartilaginous,rhabdomyosarcomatous,and anaplasticfoci (Fig.2,originalreport) [2].WhyisitimportanttoidentifylesionsspecificallyasPPB?First, cystic Type I PPB is not a benign cystic malformation.It is the initial morphologic manifestation of a uniqueprogression sequence into Type II and/or Type III PPB.Second,unlikeRMS,therearestronggeneticimplicationstothe PPB diagnosis; in approximately 25% of cases there isadditional neoplasia and dysplasia in PPB patients or theiryoung relatives [7]. Third, in contrast to other childhoodsarcomas including RMS, PPB has a striking propensity tocentral nervous system metastasis [8,9].Weappreciatetheopportunitytocorrecttheinterpretationof this case.Shyun Pai |
| Databáze: | OpenAIRE |
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