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
Pathogenesis
Sacrococcygeal teratomas originate 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.[1]
Genetics
Development of Sacrococcygeal teratoma is associated with gain of 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:
- Bladder outlet obstruction
- Hydronephrosis
- Rectal stenosis or atresia
- Hydrops fetalis
- Cardiomegaly due to vascular shunting and high output cardiac failure
Gross Pathology
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Gross Image of Sacrococcygeal teratoma
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Gross Image of Sacrococcygeal teratoma
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Gross Image of Excised Sacrococcygeal teratoma
Microscopic Pathology
- Sacrococcygeal teratoma can be divided into three types depending on the microscopic pathology, as follow:
- Mature teratoma:may consist of fully differentiated somatic tissue.[4]
- Immature teratoma: may consist of small fraction of incompletely differentiated tissue.
- Malignant teratoma:
- Around 20% of Sacrococcygeal teratomas are malignant.
- 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:[5][6]
| Grade | Microscopic Features |
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Grade 0 |
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Grade I |
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Grade II |
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Grade III |
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References
- ↑ http://www.hindawi.com/journals/criog/2012/131369/
- ↑ 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.
- ↑ 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.