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The International Pleuropulmonary Blastoma Registry
The International Pleuropulmonary Blastoma Registry
The Genetics of PPB

Critical Issues


Constitutional & Familial Issues: Genetics of PPB:


A Very Rare Disease: Enrolling Patients in the Registry


Cerebral Metastases in PPB


Genetic Study of PPB


PPB Associated with Cystic Nephroma and Other Renal Tumors


Type I PPB: Treatment Issues


Type II and III THERAPY RECOMMENDATION CHANGE

Join Our PPB Study

Whats New

04/09/2010
2010 PPB FAMILY WEEKEND IN WASHINGTON DC

02/15/2007
THERAPY CHANGE: PPB Types II and III

11/15/2006
PPB, Cystic Nephroma & Small Bowel Polyps

10/01/2005
Genetic Study in PPB - PPB Tissue Bank

Pathology
Pathology of Metastases

Metastatic disease demonstrates solid tumor patterns similar to that observed at the primary site. The histologic hallmark of Type I disease, cystic spaces with ciliated columnar respiratory epithelium with foci of underlying condensations of malignant cells, is not found in metastatic lesions. In only one of many examined cases of brain metastasis have there been microscopic remnants of cyst and septal elements. There is a tendency toward simplification of the histologic appearance, compared to the primary disease, with an abundance of either the rhabdomyoblastic or spindle cell elements. In a given patient, the microscopic appearances of different metastatic lesions are identical to each other.

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Data and recommendations on this site are conscientiously presented but some are unpublished observations and have not undergone peer review. Consultation with the Registry is encouraged to clarify any topics. The International PPB Registry advises caution in the citation of website information.
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