Sacrococcygeal teratoma pathophysiology
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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
- 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.[1]
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:
- Myelomeningocoele[4]
- Vertebral anomalies
- Bladder outlet obstruction
- Hydronephrosis
- Rectal stenosis or atresia
- Intraocular teratoma[5]
- Hydrops fetalis
- Cardiomegaly due to vascular shunting and high output cardiac failure
- Tethered spinal cord[6]
Gross Pathology
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![Gross Image of Sacrococcygeal teratoma [Case courtesy of Dr Saeed Soltany Hosn, Radiopaedia.org, rID: 33220]](/images/5/51/Sacrococcygeal-teratoma-6.jpg)
Gross Image of Sacrococcygeal teratoma [Case courtesy of Dr Saeed Soltany Hosn, Radiopaedia.org, rID: 33220]
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![Gross Image of Sacrococcygeal teratoma [Image courtesy of Sinha S, Sarin YK, Deshpande VP]](/images/4/43/5-261-1-PB.gif)
Gross Image of Sacrococcygeal teratoma [Image courtesy of Sinha S, Sarin YK, Deshpande VP]
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![Gross Image of Excised Sacrococcygeal teratoma[Image courtesy of Sinha S, Sarin YK, Deshpande VP]](/images/3/3f/5-262-1-PB.gif)
Gross Image of Excised Sacrococcygeal teratoma[Image courtesy of Sinha S, Sarin YK, Deshpande VP]
![Gross Image of Sacrococcygeal teratoma [Case courtesy of Dr Saeed Soltany Hosn, Radiopaedia.org, rID: 33220]](/index.php/File:Sacrococcygeal-teratoma-6.jpg)
![Gross Image of Sacrococcygeal teratoma [Image courtesy of Sinha S, Sarin YK, Deshpande VP]](/index.php/File:5-261-1-PB.gif)
![Gross Image of Excised Sacrococcygeal teratoma[Image courtesy of Sinha S, Sarin YK, Deshpande VP]](/index.php/File:5-262-1-PB.gif)
Microscopic Pathology
Sacrococcygeal teratoma can be divided into following two types depending on the microscopic pathology: [7]
Mature Teratoma
Immature Teratoma
- Malignant
- Consists of small fraction of incompletely differentiated tissue
- They have elevated tumor markers including yolk sac component secreting alpha fetoprotein or primitive neuroectodermal tumor (PNET).
References
- ↑ Sacrococcygeal teratoma. Hindawi (2015)http://www.hindawi.com/journals/criog/2012/131369/ Accessed on December 15th, 2015
- ↑ 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.
- ↑ 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.
- ↑ Sacrococcygel Teratoma. Radiopedia (2015) http://radiopaedia.org/articles/sacrococcygeal-teratoma Accessed on December 15, 2015
- ↑ 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) - ↑ 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) - ↑ 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.