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Difference between revisions of "Pancreatoblastoma"
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'''Pancreatoblastoma''' is a rare malignant tumour of the [[pancreas]] that is seen in children. | |||
==General== | |||
*Very rare. | |||
*Tumour of childhood - age of diagnosis ~5 years old.<ref name=pmid22525406>{{Cite journal | last1 = Glick | first1 = RD. | last2 = Pashankar | first2 = FD. | last3 = Pappo | first3 = A. | last4 = Laquaglia | first4 = MP. | title = Management of pancreatoblastoma in children and young adults. | journal = J Pediatr Hematol Oncol | volume = 34 Suppl 2 | issue = | pages = S47-50 | month = May | year = 2012 | doi = 10.1097/MPH.0b013e31824e3839 | PMID = 22525406 }}</ref> | |||
*Prognosis ~80% year survival in children<ref name=pmid21696948>{{Cite journal | last1 = Bien | first1 = E. | last2 = Godzinski | first2 = J. | last3 = Dall'igna | first3 = P. | last4 = Defachelles | first4 = AS. | last5 = Stachowicz-Stencel | first5 = T. | last6 = Orbach | first6 = D. | last7 = Bisogno | first7 = G. | last8 = Cecchetto | first8 = G. | last9 = Warmann | first9 = S. | title = Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). | journal = Eur J Cancer | volume = 47 | issue = 15 | pages = 2347-52 | month = Oct | year = 2011 | doi = 10.1016/j.ejca.2011.05.022 | PMID = 21696948 }}</ref> more aggressive in adults. | |||
*May be seen in adults.<ref name=pmid22572137>{{Cite journal | last1 = Balasundaram | first1 = C. | last2 = Luthra | first2 = M. | last3 = Chavalidthamrong | first3 = D. | last4 = Chow | first4 = J. | last5 = Khan | first5 = H. | last6 = Endres | first6 = PJ. | title = Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology. | journal = JOP | volume = 13 | issue = 3 | pages = 301-3 | month = May | year = 2012 | doi = | PMID = 22572137 }}</ref> | |||
Associations:<ref name=pmid17228135/> | |||
*[[Beckwith-Wiedemann syndrome]]. | |||
*[[Familial adenomatous polyposis]]. | |||
==Microscopic== | |||
Features:<ref name=pmid17228135>{{Cite journal | last1 = Saif | first1 = MW. | title = Pancreatoblastoma. | journal = JOP | volume = 8 | issue = 1 | pages = 55-63 | month = | year = 2007 | doi = | PMID = 17228135 }}</ref><ref name=pmid15943785/> | |||
*Acinar-like structures. | |||
*Squamoid corpuscles. | |||
*Undifferentiated component. | |||
Image: | |||
*[http://www.nature.com/modpathol/journal/v20/n1s/fig_tab/3800686f16.html#figure-title Pancreatoblastoma (nature.com)].<ref name=pmid17486055>{{Cite journal | last1 = Klimstra | first1 = DS. | title = Nonductal neoplasms of the pancreas. | journal = Mod Pathol | volume = 20 Suppl 1 | issue = | pages = S94-112 | month = Feb | year = 2007 | doi = 10.1038/modpathol.3800686 | PMID = 17486055 }}</ref> | |||
==IHC== | |||
Features:<ref name=pmid15943785>{{Cite journal | last1 = Nishimata | first1 = S. | last2 = Kato | first2 = K. | last3 = Tanaka | first3 = M. | last4 = Ijiri | first4 = R. | last5 = Toyoda | first5 = Y. | last6 = Kigasawa | first6 = H. | last7 = Ohama | first7 = Y. | last8 = Nakatani | first8 = Y. | last9 = Notohara | first9 = K. | title = Expression pattern of keratin subclasses in pancreatoblastoma with special emphasis on squamoid corpuscles. | journal = Pathol Int | volume = 55 | issue = 6 | pages = 297-302 | month = Jun | year = 2005 | doi = 10.1111/j.1440-1827.2005.01829.x | PMID = 15943785 }}</ref> | |||
*CK7 +ve -- acinar, undifferentiated component. | |||
*CK8 +ve -- squamous component. | |||
*CK18 +ve -- squamous component. | |||
*CK19 +ve -- squamous component. | |||
==See also== | |||
*[[Small round cell tumours]]. | |||
*[[Pancreas]]. | |||
==References== | |||
{{Reflist|1}} | |||
[[Category:Gastrointestinal pathology]] | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||