Sacrococcygeal teratoma overview
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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
Historical Perspective
Sacrococcygel teratoma was first described by Stanley in 1842. In 1863, first successful surgery was performed on Sacrococcygeal teratoma by Dr. W. Blizard. Between 1950s and 1960s, germ cell theory of the origin of Sacrococcygeal teratoma was introduced.[1]
Classification
Sacrococcygeal teratoma is classified into four different categories according to American Academy of Pediatrics Surgery classification based upon the anatomical location of the tumor.[2][3] Subtypes of sacrococcygeal teratoma have different staging systems based on the size and growth of the tumor, lymph node involvement, and the presence of metastasis.[4]
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.[5] Development of Sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.[6][7] The pathophysiology of sacrococcygeal teratoma depends on the histological subtype.
Causes
Development of sacrococcygeal teratoma is associated with gain of chromosomes 1q32-qter regions and losses of the 6q24-qter and 18q21-qter regions.[6][7]
Differential Diagnosis
Sacrococcygeal teratoma must be differentiated from endodermal sinus tumor, ependymoma, fibromatosis, ganglioneuroma, giant cell tumor of the sacrum, leiomyoma, meningomyelocele, ovarian teratoma, neuroblastoma, retrorectal hamartoma, intracanalicular epidermoid tumor, rhabdomyosarcoma, paraganglioma, dermal sinus stalk ascending towards the conus modulars, and hydromelia.[8]
Epidemiology & Demographics
Sacrococcygeal teratoma is one of the most common congenital tumors.[9] The incidence of Sacrococcygeal teratoma is estimated to be 1 in 35,000 births.[9]
Risk Factors
There are no established risk factors for sacrococcygeal teratoma.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for sacrococcygela teratoma.[10]
Natural History, Complications, and Prognosis
Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Most of the sacrococcygeal teratomas are benign in nature and prognosis is generally regarded as good after resection.[11] The sacrococcygeal teratoma with cystic morphology is associated with the most favorable prognosis.[12]
Diagnosis
=History and Symptoms
The hallmark of sacrococcygeal teratoma is a protruding mass at the sacrococcygeal region. It is important to obtain the history about the onset, and the progression of the symptoms. Most common symptoms of sacrococcygeal teratoma are due to compression caused by the large tumor mass.[13]
Physical Examination
References
- ↑ Olson JS. The History of Cancer, An Annotated Bibliography. ABC-CLIO; 1989.
- ↑ Puri P, Höllwarth ME. Pediatric Surgery, Diagnosis and Management. Springer Science & Business Media; 2009.
- ↑ Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.
- ↑ DeVita VT, Lawrence TS, Rosenberg SA. DeVita, Hellman, and Rosenberg's Cancer, Principles & Practice of Oncology. Lippincott Williams & Wilkins; 2008.
- ↑ http://www.hindawi.com/journals/criog/2012/131369/
- ↑ 6.0 6.1 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.
- ↑ 7.0 7.1 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.
- ↑ Myers LB, Bulich LA. Anesthesia for Fetal Intervention and Surgery. PMPH-USA; 2005.
- ↑ 9.0 9.1 Waldenström's macroglobulinemia. Cincinnati Children's (2015)http://www.cincinnatichildrens.org/service/f/fetal-care/conditions/sct/default/ Accessed on November 24, 2015
- ↑ Recommendations. US preventive services task force(2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=sacrococcygeal+teratoma Accessed on November 25, 2015
- ↑ http://www.chop.edu/conditions-diseases/sacrococcygeal-teratoma-sct/about#.VnhH0bRYZFI
- ↑ Shue E, Bolouri M, Jelin EB, Vu L, Bratton B, Cedars E, Yoke L, Byrne F, Hirose S, Feldstein V, Miniati D, Lee H (2013). "Tumor metrics and morphology predict poor prognosis in prenatally diagnosed sacrococcygeal teratoma: a 25-year experience at a single institution". J. Pediatr. Surg. 48 (6): 1225–31. doi:10.1016/j.jpedsurg.2013.03.016. PMID 23845611.
- ↑ https://rarediseases.org/rare-diseases/sacrococcygeal-teratoma/