Difference between revisions of "Pancreatoblastoma"

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#redirect [[Pancreas#Pancreatoblastoma]]
'''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]]

Revision as of 04:21, 21 May 2016

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.[1]
  • Prognosis ~80% year survival in children[2] more aggressive in adults.
  • May be seen in adults.[3]

Associations:[4]

Microscopic

Features:[4][5]

  • Acinar-like structures.
  • Squamoid corpuscles.
  • Undifferentiated component.

Image:

IHC

Features:[5]

  • CK7 +ve -- acinar, undifferentiated component.
  • CK8 +ve -- squamous component.
  • CK18 +ve -- squamous component.
  • CK19 +ve -- squamous component.

See also

References

  1. ↑ Glick, RD.; Pashankar, FD.; Pappo, A.; Laquaglia, MP. (May 2012). "Management of pancreatoblastoma in children and young adults.". J Pediatr Hematol Oncol 34 Suppl 2: S47-50. doi:10.1097/MPH.0b013e31824e3839. PMID 22525406.
  2. ↑ Bien, E.; Godzinski, J.; Dall'igna, P.; Defachelles, AS.; Stachowicz-Stencel, T.; Orbach, D.; Bisogno, G.; Cecchetto, G. et al. (Oct 2011). "Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT).". Eur J Cancer 47 (15): 2347-52. doi:10.1016/j.ejca.2011.05.022. PMID 21696948.
  3. ↑ Balasundaram, C.; Luthra, M.; Chavalidthamrong, D.; Chow, J.; Khan, H.; Endres, PJ. (May 2012). "Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology.". JOP 13 (3): 301-3. PMID 22572137.
  4. ↑ 4.0 4.1 Saif, MW. (2007). "Pancreatoblastoma.". JOP 8 (1): 55-63. PMID 17228135.
  5. ↑ 5.0 5.1 Nishimata, S.; Kato, K.; Tanaka, M.; Ijiri, R.; Toyoda, Y.; Kigasawa, H.; Ohama, Y.; Nakatani, Y. et al. (Jun 2005). "Expression pattern of keratin subclasses in pancreatoblastoma with special emphasis on squamoid corpuscles.". Pathol Int 55 (6): 297-302. doi:10.1111/j.1440-1827.2005.01829.x. PMID 15943785.
  6. ↑ Klimstra, DS. (Feb 2007). "Nonductal neoplasms of the pancreas.". Mod Pathol 20 Suppl 1: S94-112. doi:10.1038/modpathol.3800686. PMID 17486055.
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