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 name = pat> Sacrococcygeal teratoma. Hindawi (2015)http://www.hindawi.com/journals/criog/2012/131369/ Accessed on December 15th, 2015</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.
Sacrococcygeal teratoma originates from the [[Pluripotency|pluripotent]] [[Cell (biology)|cells]] in primitive knot or Hensen's node, which is the primary organizer of [[Embryo|embryonic]] development, located on the [[anterior]] surface of the [[sacrum]] or [[coccyx]] by the 2nd or 3rd [[Gestation|gestational]] week. Development of sacrococcygeal teratoma is associated with gain of [[Chromosome|chromosomes]] ''1q32-qter'' regions and loss of the ''6q24-qter'' and ''18q21-qter'' regions. The [[pathophysiology]] of sacrococcygeal teratoma depends on the [[Histology|histological]] subtype.


==Pathogenesis==
== Pathophysiology ==
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 name = pat> Sacrococcygeal teratoma. Hindawi (2015)http://www.hindawi.com/journals/criog/2012/131369/ Accessed on December 15th, 2015</ref>
 
===Pathogenesis===
* Sacrococcygeal teratoma originates from the [[Pluripotency|pluripotent]] [[Cell (biology)|cells]] in primitive knot or Hensen's node, which is the primary organizer of [[Embryo|embryonic]] development, located on the [[Anatomical terms of location|anterior]] surface of the [[sacrum]] or [[coccyx]] by the 2nd or 3rd [[Gestation|gestational]] week.<ref name="pat">Sacrococcygeal teratoma. Hindawi (2015)http://www.hindawi.com/journals/criog/2012/131369/ Accessed on December 15th, 2015</ref>


==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 [[Chromosome|chromosomes]] ''1q32-qter'' regions and loss 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 name = path>Sacrococcygel Teratoma. Radiopedia (2015) http://radiopaedia.org/articles/sacrococcygeal-teratoma Accessed on December 15, 2015</ref>
*[[Spina bifida|Myelomeningocoele]]<ref name="path">Sacrococcygel Teratoma. Radiopedia (2015) http://radiopaedia.org/articles/sacrococcygeal-teratoma Accessed on December 15, 2015</ref>
*[[Vertebral anomalies]]
*[[Vertebral anomalies]]
*[[Bladder outlet obstruction]]
*[[Bladder outlet obstruction]]
*[[Hydronephrosis]]
*[[Hydronephrosis]]
*Rectal stenosis or atresia
*[[Rectum|Rectal]] [[stenosis]] or [[atresia]]
*[[Ocular|Intraocular]] [[teratoma]]<ref>{{Cite journal
| author = [[Kristen E. Zhelnin]], [[Grant M. Gebhard]], [[David M. Mirsky]], [[Scott Cn Oliver]], [[Mark A. Lovell]], [[Csaba Galambos]], [[Timothy M. Crombleholme]] & [[Emily A. McCourt]]
| title = Pediatric Intraocular Immature Teratoma Associated With Sacrococcygeal Teratoma
| journal = [[Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society]]
| volume = 20
| issue = 3
| pages = 240–244
| year = 2017
| month = June
| doi = 10.1177/1093526616686233
| pmid = 28521629
}}</ref>
*[[Hydrops fetalis]]
*[[Hydrops fetalis]]
*[[Cardiomegaly]] due to vascular shunting and high output cardiac failure
*[[Cardiomegaly]] due to vascular shunting and high output cardiac failure
*Tethered [[spinal cord]]<ref>{{Cite journal
| author = [[Tugba Sarac Sivrikoz]], [[Recep Has]], [[Aytul Corbacioglu Esmer]], [[Ibrahim Kalelioglu]], [[Atil Yuksel]] & [[Orhun Cig Taskin]]
| title = Prenatal diagnosis of tethered spinal cord associated with sacrococcygeal teratoma
| journal = [[Journal of clinical ultrasound : JCU]]
| volume = 44
| issue = 8
| pages = 506–509
| year = 2016
| month = October
| doi = 10.1002/jcu.22344
| pmid = 26892808
}}</ref>


==Gross Pathology==
==Gross Pathology==
<gallery>  
<gallery>  
Image:Sacrococcygeal-teratoma-6.jpg|Gross Image of Sacrococcygeal teratoma
Image:Sacrococcygeal-teratoma-6.jpg|Gross Image of Sacrococcygeal teratoma [[https://radiopaedia.org Case courtesy of Dr Saeed Soltany Hosn, Radiopaedia.org, rID: 33220]]
Image:5-261-1-PB.gif|Gross Image of Sacrococcygeal teratoma
Image:5-261-1-PB.gif|Gross Image of Sacrococcygeal teratoma [[http://www.jneonatalsurg.com/ojs/index.php/jns/article/view/5/57‘’here’’ [[Image courtesy of Sinha S, Sarin YK, Deshpande VP]]]]
Image:5-262-1-PB.gif|Gross Image of Excised Sacrococcygeal teratoma
Image:5-262-1-PB.gif|Gross Image of Excised Sacrococcygeal teratoma[[http://www.jneonatalsurg.com/ojs/index.php/jns/article/view/5/57‘’here’’ [[Image courtesy of Sinha S, Sarin YK, Deshpande VP]]]]
</gallery>
</gallery>


==Microscopic Pathology==
==Microscopic Pathology==
Sacrococcygeal teratoma can be divided into following three types depending on the microscopic pathology: <ref name = "micro">{{cite journal |vauthors=Calaminus G, Schneider DT, Bökkerink JP, Gadner H, Harms D, Willers R, Göbel U |title=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 |journal=J. Clin. Oncol. |volume=21 |issue=5 |pages=781–6 |year=2003 |pmid=12610174 |doi= |url=}}</ref>
Sacrococcygeal teratoma can be divided into following two types depending on the [[microscopic]] [[pathology]]: <ref name="micro">{{cite journal |vauthors=Calaminus G, Schneider DT, Bökkerink JP, Gadner H, Harms D, Willers R, Göbel U |title=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 |journal=J. Clin. Oncol. |volume=21 |issue=5 |pages=781–6 |year=2003 |pmid=12610174 |doi= |url=}}</ref>
===Mature Teratoma===
===Mature Teratoma===
*[[Benign]]
*[[Benign]]
*Consist of fully differentiated somatic tissue
*Consists of fully differentiated [[somatic]] [[Tissue (biology)|tissue]]
 
===Immature Teratoma===  
===Immature Teratoma===  
*[[Malignant]]
*[[Malignant]]
*Consist of small fraction of incompletely differentiated tissue
*Consists of small fraction of incompletely differentiated [[Tissue (biology)|tissue]]
*They have elevated tumor markers including yolk sac component secreting [[alpha fetoprotein]] or [[primitive neuroectodermal tumor]] [[(PNET)]].
*They have elevated [[Tumor marker|tumor markers]] including [[yolk sac]] component secreting [[alpha fetoprotein]] or [[primitive neuroectodermal tumor|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:<ref name = "aa">{{cite journal | authors=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"
|valign=top|
|+
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Grade}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Microscopic Features}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Grade 0'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Tumor contains only mature tissue.
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
'''Grade I'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Tumor contains rare foci of immature tissues.
*Less than 10% tissue is Immature
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
'''Grade II'''
| style="padding: 5px 5px; background: #F5F5F5;" |
* Tumor contains moderate quantities of immature tissues.
* Upto 10-50% tissue in immature
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
'''Grade III'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Tumor contains large quantities of immature tissue with or without malignant yolk sac elements.
*More than 50% tissue is immature
|}


==References==
==References==
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Latest revision as of 19:07, 6 May 2019

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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 the 2nd or 3rd gestational week. Development of sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and loss of the 6q24-qter and 18q21-qter regions. The pathophysiology of sacrococcygeal teratoma depends on the histological subtype.

Pathophysiology

Pathogenesis

Genetics

  • Development of Sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and loss of the 6q24-qter and 18q21-qter regions.[2][3]

Associated Conditions

Following conditions are associated with sacrococcygeal teratoma:

Gross Pathology

Microscopic Pathology

Sacrococcygeal teratoma can be divided into following two types depending on the microscopic pathology: [7]

Mature Teratoma

Immature Teratoma

References

  1. ↑ Sacrococcygeal teratoma. Hindawi (2015)http://www.hindawi.com/journals/criog/2012/131369/ Accessed on December 15th, 2015
  2. ↑ 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. ↑ 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.
  4. ↑ Sacrococcygel Teratoma. Radiopedia (2015) http://radiopaedia.org/articles/sacrococcygeal-teratoma Accessed on December 15, 2015
  5. ↑ Kristen E. Zhelnin, Grant M. Gebhard, David M. Mirsky, Scott Cn Oliver, Mark A. Lovell, Csaba Galambos, Timothy M. Crombleholme & Emily A. McCourt (2017). "Pediatric Intraocular Immature Teratoma Associated With Sacrococcygeal Teratoma". Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society. 20 (3): 240–244. doi:10.1177/1093526616686233. PMID 28521629. Unknown parameter |month= ignored (help)
  6. ↑ Tugba Sarac Sivrikoz, Recep Has, Aytul Corbacioglu Esmer, Ibrahim Kalelioglu, Atil Yuksel & Orhun Cig Taskin (2016). "Prenatal diagnosis of tethered spinal cord associated with sacrococcygeal teratoma". Journal of clinical ultrasound : JCU. 44 (8): 506–509. doi:10.1002/jcu.22344. PMID 26892808. Unknown parameter |month= ignored (help)
  7. ↑ 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.

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