Sacrococcygeal teratoma pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
Sacrococcygeal teratoma originates from the pluripotent cells in primitive knot or Hensen's node, which is the primary organizer of embryonic development, located on the anterior surface of the sacrum or coccyx by 2rd or 3rd gestational week.<ref>http://www.hindawi.com/journals/criog/2012/131369/</ref> Development of | Sacrococcygeal teratoma originates from the pluripotent cells in primitive knot or [[Hensen's node[[, which is the primary organizer of embryonic development, located on the anterior surface of the sacrum or coccyx by 2rd or 3rd gestational week.<ref>http://www.hindawi.com/journals/criog/2012/131369/</ref> Development of sacrococcygeal teratoma is associated with gain of chromosomes ''1q32-qter'' regions and losses of the ''6q24-qter'' and ''18q21-qter'' regions.<ref name = "aa">{{cite journal |vauthors=Harms D, Zahn S, Göbel U, Schneider DT |title=Pathology and molecular biology of teratomas in childhood and adolescence |journal=Klin Padiatr |volume=218 |issue=6 |pages=296–302 |year=2006 |pmid=17080330 |doi=10.1055/s-2006-942271 |url=}}</ref><ref name = "aaa">{{cite journal |vauthors=Veltman I, Veltman J, Janssen I, Hulsbergen-van de Kaa C, Oosterhuis W, Schneider D, Stoop H, Gillis A, Zahn S, Looijenga L, Göbel U, van Kessel AG |title=Identification of recurrent chromosomal aberrations in germ cell tumors of neonates and infants using genomewide array-based comparative genomic hybridization |journal=Genes Chromosomes Cancer |volume=43 |issue=4 |pages=367–76 |year=2005 |pmid=15880464 |doi=10.1002/gcc.20208 |url=}}</ref> The pathophysiology of sacrococcygeal teratoma depends on the histological subtype. | ||
==Pathogenesis== | ==Pathogenesis== | ||
| Line 10: | Line 10: | ||
==Genetics== | ==Genetics== | ||
Development of Sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.<ref name = "aa">{{cite journal |vauthors=Harms D, Zahn S, Göbel U, Schneider DT |title=Pathology and molecular biology of teratomas in childhood and adolescence |journal=Klin Padiatr |volume=218 |issue=6 |pages=296–302 |year=2006 |pmid=17080330 |doi=10.1055/s-2006-942271 |url=}}</ref><ref name = "aaa">{{cite journal |vauthors=Veltman I, Veltman J, Janssen I, Hulsbergen-van de Kaa C, Oosterhuis W, Schneider D, Stoop H, Gillis A, Zahn S, Looijenga L, Göbel U, van Kessel AG |title=Identification of recurrent chromosomal aberrations in germ cell tumors of neonates and infants using genomewide array-based comparative genomic hybridization |journal=Genes Chromosomes Cancer |volume=43 |issue=4 |pages=367–76 |year=2005 |pmid=15880464 |doi=10.1002/gcc.20208 |url=}}</ref> | Development of Sacrococcygeal teratoma is associated with gain of chromosomes ''1q32-qter'' regions and losses of the ''6q24-qter'' and ''18q21-qter'' regions.<ref name = "aa">{{cite journal |vauthors=Harms D, Zahn S, Göbel U, Schneider DT |title=Pathology and molecular biology of teratomas in childhood and adolescence |journal=Klin Padiatr |volume=218 |issue=6 |pages=296–302 |year=2006 |pmid=17080330 |doi=10.1055/s-2006-942271 |url=}}</ref><ref name = "aaa">{{cite journal |vauthors=Veltman I, Veltman J, Janssen I, Hulsbergen-van de Kaa C, Oosterhuis W, Schneider D, Stoop H, Gillis A, Zahn S, Looijenga L, Göbel U, van Kessel AG |title=Identification of recurrent chromosomal aberrations in germ cell tumors of neonates and infants using genomewide array-based comparative genomic hybridization |journal=Genes Chromosomes Cancer |volume=43 |issue=4 |pages=367–76 |year=2005 |pmid=15880464 |doi=10.1002/gcc.20208 |url=}}</ref> | ||
==Associated Conditions== | ==Associated Conditions== | ||
Following conditions are associated with sacrococcygeal teratoma: | Following conditions are associated with sacrococcygeal teratoma: | ||
*[[Myelomeningocoele]]<ref>http://radiopaedia.org/articles/sacrococcygeal-teratoma</ref> | *[[Myelomeningocoele]] <ref>http://radiopaedia.org/articles/sacrococcygeal-teratoma</ref> | ||
*[[Vertebral anomalies]] | *[[Vertebral anomalies]] | ||
*Bladder outlet obstruction | *[[Bladder outlet obstruction]] | ||
*Hydronephrosis | *[[Hydronephrosis]] | ||
*Rectal stenosis or atresia | *[[Rectal stenosis]] or atresia | ||
*Hydrops fetalis | *[[Hydrops fetalis]] | ||
*Cardiomegaly due to vascular shunting and high output cardiac failure | *[[Cardiomegaly]] due to vascular shunting and high output cardiac failure | ||
==Gross Pathology== | ==Gross Pathology== | ||
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*Malignant | *Malignant | ||
*Consist of small fraction of incompletely differentiated tissue | *Consist of small fraction of incompletely differentiated tissue | ||
*They have elevated tumor markers including yolk sac component secreting alpha fetoprotein or primitive neuroectodermal tumor (PNET). | *They have elevated tumor markers including yolk sac component secreting [[alpha fetoprotein]] or [[primitive neuroectodermal tumor]] [[(PNET)]]. | ||
==Grading Based Upon Microscopic Features== | ==Grading Based Upon Microscopic Features== | ||
According to Gonzalez-Crussi System, | According to Gonzalez-Crussi System, sacrococcygeal teratoma is graded on a scale from 0-3, based on the histology:<ref name = "cs">Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.</ref><ref name = "ad">{{cite journal |vauthors=Harms D, Zahn S, Göbel U, Schneider DT |title=Pathology and molecular biology of teratomas in childhood and adolescence |journal=Klin Padiatr |volume=218 |issue=6 |pages=296–302 |year=2006 |pmid=17080330 |doi=10.1055/s-2006-942271 |url=}}</ref> | ||
{| style="border: 0px; font-size: 90%; margin: 3px; width: 800px" | {| style="border: 0px; font-size: 90%; margin: 3px; width: 800px" | ||
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'''Grade 0''' | '''Grade 0''' | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Tumor contains only mature tissue | *Tumor contains only mature tissue. | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | ||
'''Grade I''' | '''Grade I''' | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Tumor contains rare foci of immature tissues | *Tumor contains rare foci of immature tissues. | ||
*Less than 10% tissue is Immature | *Less than 10% tissue is Immature | ||
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[[Category:Pediatric cancers]] | [[Category:Pediatric cancers]] | ||
[[Category:Mature chapter]] | [[Category:Mature chapter]] | ||
{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
Revision as of 04:33, 25 December 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]
Overview
Sacrococcygeal teratoma originates from the pluripotent cells in primitive knot or [[Hensen's node[[, which is the primary organizer of embryonic development, located on the anterior surface of the sacrum or coccyx by 2rd or 3rd gestational week.[1] Development of sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.[2][3] The pathophysiology of sacrococcygeal teratoma depends on the histological subtype.
Pathogenesis
Sacrococcygeal teratoma originates from the pluripotent cells in primitive knot or Hensen's node, which is the primary organizer of embryonic development, located on the anterior surface of the sacrum or coccyx by 2rd or 3rd gestational week.[4]
Genetics
Development of Sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.[2][3]
Associated Conditions
Following conditions are associated with sacrococcygeal teratoma:
- Myelomeningocoele [5]
- Vertebral anomalies
- Bladder outlet obstruction
- Hydronephrosis
- Rectal stenosis or atresia
- Hydrops fetalis
- Cardiomegaly due to vascular shunting and high output cardiac failure
Gross Pathology
-
Gross Image of Sacrococcygeal teratoma
-
Gross Image of Sacrococcygeal teratoma
-
Gross Image of Excised Sacrococcygeal teratoma
Microscopic Pathology
Sacrococcygeal teratoma can be divided into following three types depending on the microscopic pathology: [6]
Mature Teratoma
- Benign
- Consist of fully differentiated somatic tissue
Immature Teratoma
- Malignant
- Consist of small fraction of incompletely differentiated tissue
- They have elevated tumor markers including yolk sac component secreting alpha fetoprotein or primitive neuroectodermal tumor (PNET).
Grading Based Upon Microscopic Features
According to Gonzalez-Crussi System, sacrococcygeal teratoma is graded on a scale from 0-3, based on the histology:[7][8]
| Grade | Microscopic Features |
|---|---|
|
Grade 0 |
|
|
Grade I |
|
|
Grade II |
|
|
Grade III |
|
References
- ↑ http://www.hindawi.com/journals/criog/2012/131369/
- ↑ 2.0 2.1 Harms D, Zahn S, Göbel U, Schneider DT (2006). "Pathology and molecular biology of teratomas in childhood and adolescence". Klin Padiatr. 218 (6): 296–302. doi:10.1055/s-2006-942271. PMID 17080330.
- ↑ 3.0 3.1 Veltman I, Veltman J, Janssen I, Hulsbergen-van de Kaa C, Oosterhuis W, Schneider D, Stoop H, Gillis A, Zahn S, Looijenga L, Göbel U, van Kessel AG (2005). "Identification of recurrent chromosomal aberrations in germ cell tumors of neonates and infants using genomewide array-based comparative genomic hybridization". Genes Chromosomes Cancer. 43 (4): 367–76. doi:10.1002/gcc.20208. PMID 15880464.
- ↑ http://www.hindawi.com/journals/criog/2012/131369/
- ↑ http://radiopaedia.org/articles/sacrococcygeal-teratoma
- ↑ Calaminus G, Schneider DT, Bökkerink JP, Gadner H, Harms D, Willers R, Göbel U (2003). "Prognostic value of tumor size, metastases, extension into bone, and increased tumor marker in children with malignant sacrococcygeal germ cell tumors: a prospective evaluation of 71 patients treated in the German cooperative protocols Maligne Keimzelltumoren (MAKEI) 83/86 and MAKEI 89". J. Clin. Oncol. 21 (5): 781–6. PMID 12610174.
- ↑ Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.
- ↑ Harms D, Zahn S, Göbel U, Schneider DT (2006). "Pathology and molecular biology of teratomas in childhood and adolescence". Klin Padiatr. 218 (6): 296–302. doi:10.1055/s-2006-942271. PMID 17080330.