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'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''.
'''For patient information click [[Sacrococcygeal teratoma (patient information)|here]]'''


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{{Sacrococcygeal teratoma}}
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{{SK}} Fetiform Teratoma, Fetus in fetu


{{main|Teratoma}}
==[[Sacrococcygeal teratoma overview|Overview]]==
==Overview==
'''Sacrococcygeal teratoma''' (SCT) is a [[teratoma]] (a kind of tumor) located at the base of the [[coccyx]] (tailbone). It is thought to be a derivative of the [[primitive streak]].


==Natural history==
==[[Sacrococcygeal teratoma historical perspective|Historical Perspective]]==
SCT is seen in 1 in every 35,000 live births, and is the most common tumor presenting in newborn humans.  Most SCTs are found in babies and children, but SCTs have been reported in adults<ref>Killen DA, Jackson LM (1964) ''Sacrococcygeal teratoma in the adult'' [[Archives of Surgery]] 88(3):425-433.</ref> and the increasingly routine use of prenatal ultrasound exams has dramatically increased the number of diagnosed SCTs presenting in fetuses.  Like other teratomas, an SCT can grow very large.  Unlike other teratomas, an SCT sometimes grows larger than the rest of the fetus.


Sacrococcygeal teratomas are the most common type of [[germ cell tumor]]s (both [[benign]] and [[malignant]]) diagnosed in [[neonates]], [[infants]], and [[children]] younger than 4 years.<ref>[http://www.cancer.gov/cancertopics/pdq/treatment/extracranial-germ-cell/HealthProfessional/page6 (PDQ) Sacrococcygeal Tumors in Children]</ref>  SCTs occur more often in girls than in boys; ratios of 3:1 to 4:1 have been reported.<ref>Rescorla FJ, Sawin RS, Coran AG, et al.: Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the Childrens Cancer Group. J Pediatr Surg 33 (2): 171-6, 1998. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9498381&dopt=Abstract PUBMED Abstract]</ref>
==[[Sacrococcygeal teratoma classification|Classification]]==


Historically, sacrococcygeal teratomas present in 2 clinical patterns related to the child’s age, tumor location, and likelihood of tumor malignancy.  With the advent of routine prenatal ultrasound examinations, a third clinical pattern is emerging.
==[[Sacrococcygeal teratoma pathophysiology|Pathophysiology]]==


* '''Fetal''' tumors present during prenatal ultrasound exams, with or without maternal symptoms.  SCTs found during routine exams tend to be small and partly or entirely external.  The internal SCTs are not easily seen via ultrasound, unless they are large enough to reveal their presence by the abnomal position of the fetal [[urinary bladder]] and other organs, but large fetal SCTs frequenty produce maternal complications which necessitate non-routine, investigative ultrasounds.
==[[Sacrococcygeal teratoma causes|Causes]]==


* '''Neonatal''' tumors present at birth protruding from the sacral site and are usually mature or immature teratomas.
==[[Sacrococcygeal teratoma differential diagnosis|Differentiating Sacrococcygeal teratoma from other Diseases]]==


*Among '''infants and young children''', the tumor presents as a palpable mass in the sacropelvic region compressing the bladder or rectum.<ref>Rescorla FJ: Pediatric germ cell tumors. Semin Surg Oncol 16 (2): 144-58, 1999. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9988869&dopt=Abstract PUBMED Abstract]</ref> These pelvic tumors have a greater likelihood of being malignant. An early survey found that the rate of tumor malignancy was 48% for girls and 67% for boys older than 2 months at the time of sacrococcygeal tumor diagnosis, compared with a [[Cancer|malignant tumor]] incidence of 7% for girls and 10% for boys younger than 2 months at the time of diagnosis. The pelvic site of the [[primary tumor]] has been reported to be an adverse prognostic factor, most likely caused by a higher rate of incomplete resection.
==[[Sacrococcygeal teratoma epidemiology and demographics|Epidemiology and Demographics]]==


*In '''older children and adults''', the tumor may be mistaken for a [[pilonidal sinus]], or it may be found during a rectal exam or other evaluation.
==[[Sacrococcygeal teratoma risk factors|Risk Factors]]==


==Diagnosis==
==[[Sacrococcygeal teratoma screening|Screening]]==


During [[prenatal]] [[ultrasound]], an SCT having an external component may appear as a fluid-filled cyst or a solid mass sticking out from the fetus's buttocks.  Fetal SCTs that are entirely internal may be undetected if they are small;  detection (or at least suspicion) is possible when the fetal bladder is seen in an abnormal position, due to the SCT pushing other organs out of place.
==[[Sacrococcygeal teratoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
 
At [[birth]], the usual presentation is a visible lump or mass under the skin at the top of the buttocks crease.  If not visible, it can sometimes be felt; gently prodded, it feels somewhat like a hardboiled egg.  A small SCT, if it is entirely inside the body, may not present for years, until it grows large enough to cause pain, constipation and other symptoms of a large mass inside the [[pelvis]], or until it begins to extend out of the pelvis.  Even a relatively large SCT may be missed, if it is internal, because the bony [[pelvis]] conceals and protects it.  [[Mediastinal tumor]]s, including teratomas, are similarly concealed and protected by the [[rib cage]].
 
Some SCTs are discovered when a child begins to talk at about age 2 years and complains of their bottom hurting or feeling "poopy" when they ride in a car seat.
 
Other tumors can occur in the sacrococcygeal and/or presacral regions<ref name="pmid6366733">{{cite journal
| author = Bale PM
| title = Sacrococcygeal developmental abnormalities and tumors in children.
| journal = Perspectives in pediatric pathology
| volume = 8
| issue = 1
| pages = 9-56
| year = 1984
| pmid = 6366733
| doi =
| issn =
}}</ref> and hence must be ruled out to obtain a [[differential diagnosis]].  These include [[ependymoma|extraspinal ependymoma]]<ref name="pmid10726703">{{cite journal
| author = Aktuğ T, Hakgüder G, Sarioğlu S, Akgür FM, Olguner M, Pabuçcuoğlu U
| title = Sacrococcygeal extraspinal ependymomas: the role of coccygectomy.
| journal = J. Pediatr. Surg.
| volume = 35
| issue = 3
| pages = 515-8
| year = 2000
| pmid = 10726703
| doi =
| issn =
}}</ref>, [[neuroblastoma]] and [[rhabdomyosarcoma]].  Smaller SCTs with an external component, seen in prenatal ultrasounds or at birth, often are mistaken for [[spina bifida]].


==Diagnosis==
[[Sacrococcygeal teratoma history and symptoms|History & Symptoms]] | [[Sacrococcygeal teratoma physical examination|Physical Examination]] | [[Sacrococcygeal teratoma laboratory tests|Laboratory Tests]] | [[Sacrococcygeal teratoma pelvis x ray|Pelvis X Ray]] | [[Sacrococcygeal teratoma CT|CT]] | [[Sacrococcygeal teratoma MRI|MRI]] | [[Sacrococcygeal teratoma echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Sacrococcygeal teratoma other imaging findings|Other Imaging Findings]] | [[Sacrococcygeal teratoma other diagnostic studies|Other Diagnostic Studies]]
==Treatment==
==Treatment==
The preferred first treatment for SCT is complete surgical removal (ie, complete resection). The preferred approach to a small SCT is through the [[perineum]]; a large SCT may require an additional approach through the [[abdomen]].  Resection should include the coccyx and may also include portions of the [[sacrum]]. The surgery should include reattachment of the small muscles and ligaments formerly attached to the coccyx, in effect reconstructing the posterior perineum.
[[Sacrococcygeal teratoma medical therapy|Medical Therapy]] | [[Sacrococcygeal teratoma surgery|Surgery]] | [[Sacrococcygeal teratoma primary prevention|Primary Prevention]] | [[Sacrococcygeal teratoma secondary prevention|Secondary Prevention]] | [[Sacrococcygeal teratoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] |  [[Sacrococcygeal teratoma future or investigational therapies|Future or Investigational Therapies]]
 
SCTs are classified morphologically according to how much of them is outside or inside the body:
*Altman Type I — entirely external, sometimes attached to the body only by a narrow '''stalk'''
*Altman Type II
*Altman Type III
*Altman Type IV — entirely internal;  this is a '''presacral teratoma'''
 
The Altman Type has a significant influence on management, surgical approach, and complications of SCT.  Serial ultrasound and MRI monitoring of SCTs in fetuses [[in utero]] has demonstrated that the Altman Type can change over time.
 
Regardless of location in the body, [[teratoma]]s are classified according to a [[cancer staging]] system.  This indicates whether [[chemotherapy]] or [[radiation therapy]] may be needed.
 
==Management of fetal SCTs==
 
Management of most fetal SCTs involves [[watch and wait]], also known as [[expectant management]].  An often used [[decision tree]] is as follows:
 
*Perform detailed ultrasound exam including fetal [[echocardiogram]] and [[Doppler]] [[Data-flow analysis|flow analysis]]
**If fetal high output failure, placentomegaly, or [[hydrops]]
***If fetus not mature, perform [[pregnancy termination]] or [[fetal intervention]]
***Else fetus mature, perform emergency [[Cesarean section]]
**Else no emergent problems, perform serial [[non-stress test]]s and ultrasound [[biophysical profile]]s and plan delivery
***If emergent problems develop, return to top of decision tree
***Else if SCT over 5–10cm or [[polyhydramnios]], perform early (37 weeks gestation) [[Caesarean delivery on maternal request|elective Cesarean]] section
***Else SCT small and no complications, permit term [[spontaneous vaginal delivery]]
 
Emergent problems include maternal [[mirror syndrome]], [[polyhydramnios]], and [[preterm labor]].  Poor management decisions, including interventions that are either premature or delayed, can have dire consequences.<ref name="pmid11965727">{{cite journal
| author = Mazneĭkova V, Dimitrova V
| title = [Prenatal ultrasonographic diagnosis of four cases of sacrococcygeal teratoma]
| language = Bulgarian
| journal = Akusherstvo i ginekologii͡a
| volume = 38
| issue = 1
| pages = 64-9
| year = 1999
| pmid = 11965727
| doi =
| issn =
}}</ref><ref name="pmid17525561">{{cite journal
| author = Sheil AT, Collins KA
| title = Fatal birth trauma due to an undiagnosed abdominal teratoma: case report and review of the literature
| journal = The American journal of forensic medicine and pathology : official publication of the National Association of Medical Examiners
| volume = 28
| issue = 2
| pages = 121-7
| year = 2007
| pmid = 17525561
| doi = 10.1097/01.paf.0000257373.91126.0d
| issn =
}}</ref>
 
In many cases, a fetus with a small SCT (under 5 or 10 cm) may be delivered vaginally.<ref name="pmid12475573">{{cite journal
| author = Anteby EY, Yagel S
| title = Route of delivery of fetuses with structural anomalies
| journal = Eur. J. Obstet. Gynecol. Reprod. Biol.
| volume = 106
| issue = 1
| pages = 5-9
| year = 2003
| pmid = 12475573
| doi =
| issn =
}}</ref><ref name="pmid11336088">{{cite journal
| author = Ruangtrakool R, Nitipon A, Laohapensang M, ''et al''
| title = Sacrococcygeal teratoma: 25 year experience
| journal = Journal of the Medical Association of Thailand = Chotmaihet thangphaet
| volume = 84
| issue = 2
| pages = 265-73
| year = 2001
| pmid = 11336088
| doi =
| issn =
}}</ref><ref name="pmid8458172">{{cite journal
| author = McCurdy CM, Seeds JW
| title = Route of delivery of infants with congenital anomalies
| journal = Clinics in perinatology
| volume = 20
| issue = 1
| pages = 81-106
| year = 1993
| pmid = 8458172
| doi =
| issn =
}}</ref><ref name="pmid2205995">{{cite journal
| author = Kainer F, Winter R, Hofmann HM, Karpf EF
| title = [Sacrococcygeal teratoma. Prenatal diagnosis and prognosis]
| language = German
| journal = Zentralblatt für Gynäkologie
| volume = 112
| issue = 10
| pages = 609-16
| year = 1990
| pmid = 2205995
| doi =
| issn =
}}</ref>
 
==Complications==
 
Maternal complications of '''pregnancy''' may include [[mirror syndrome]].<ref name="pmid17465292">{{cite journal
| author = Finamore PS, Kontopoulos E, Price M, Giannina G, Smulian JC
| title = Mirror syndrome associated with sacrococcygeal teratoma: a case report
| journal = The Journal of reproductive medicine
| volume = 52
| issue = 3
| pages = 225-7
| year = 2007
| pmid = 17465292
| doi =
| issn =
}}</ref> Maternal complications of '''delivery''' may include a [[Cesarean section]] or, alternatively, a vaginal delivery with mechanical [[dystocia]].<ref name="pmid17469451">{{cite journal
| author = Nalbanski B, Markov D, Brankov O
| title = [Sacrococcygeal teratoma--a case report and literature review]
| language = Bulgarian
| journal = Akusherstvo i ginekologii͡a
| volume = 46
| issue = 2
| pages = 41-5
| year = 2007
| pmid = 17469451
| doi =
| issn =
}}</ref>


Complications of the mass effect of a teratoma in general are addressed on the [[teratoma]] page.  Complications of the mass effect of a large SCT may include [[hip dysplasia]], [[bowel obstruction]], [[urinary obstruction]], [[hydronephrosis]] and [[hydrops fetalis]].  Even a small SCT can produce complications of mass effect, if it is presacral (Altman Type IV).<ref name="pmid15750911">{{cite journal
==Case Studies==
| author = Galili O, Mogilner J
| title = Type IV sacrococcygeal teratoma causing urinary retention: a rare presentation.
| journal = J. Pediatr. Surg.
| volume = 40
| issue = 2
| pages = E18-20
| year = 2005
| pmid = 15750911
| doi = 10.1016/j.jpedsurg.2004.10.003
| issn =
}}</ref>  In the fetus, severe hydronephrosis may contribute to inadequate lung development.  Also in the fetus and newborn, the [[anus]] may be [[imperforate anus|imperforate]].


Later complications of the mass effect and/or surgery may include [[neurogenic bladder]], other forms of [[urinary incontinence]], [[fecal incontinence]], and other chronic problems resulting from accidental damage to or sacrifice of nerves and muscles within the pelvis.<ref name="pmid3439358">{{cite journal
[[Sacrococcygeal teratoma case study one|Case #1]]
| author = Engelskirchen R, Holschneider AM, Rhein R, Hecker WC, Höpner F
| title = [Sacral teratomas in childhood. An analysis of long-term results in 87 children]
| language = German
| journal = Zeitschrift für Kinderchirurgie : organ der Deutschen, der Schweizerischen und der Osterreichischen Gesellschaft für Kinderchirurgie = Surgery in infancy and childhood
| volume = 42
| issue = 6
| pages = 358-61
| year = 1987
| pmid = 3439358
| doi =
| issn =
}}</ref>  [[coccygectomy|Removal of the coccyx]] may include additional complications.  In one review of 25 patients,<ref>[http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve&db=PubMed&list_uids= 16373161&dopt=AbstractPlus PubMed]</ref> however, the most frequent complication was an unsatisfactory appearance of the surgical scar.
 
===Late effects===
Pelvic scarring may necessitate that [[Pregnancy|pregnant women]] who are SCT survivors deliver their babies by [Cesarean section].{{Fact|date=June 2007}}
 
Complications of not removing the coccyx may include both recurrence of the teratoma<ref name="Lahdenne+1993">{{cite journal
| author = Lahdenne P, Heikinheimo M, Nikkanen V, Klemi P, Siimes MA, Rapola J
| title = Neonatal benign sacrococcygeal teratoma may recur in adulthood and give rise to malignancy.
| journal = Cancer
| volume = 72
| issue = 12
| pages = 3727-31
| year = 1993
| pmid = 8252490
| doi =
| issn =
}} Synopsis:  45 survivors of infant SCT were followed up.  Two reported recurrent benign teratoma and one reported metastatic adenocarcinoma originating from the residual coccyx.  They were aged 21-43 at diagnosis.</ref> and [[Metastasis|metastatic cancer]].<ref name="Lahdenne+1993"/><ref name="Lack+1993">{{cite journal
| author = Lack EE, Glaun RS, Hefter LG, Seneca RP, Steigman C, Athari F
| title = Late occurrence of malignancy following resection of a histologically mature sacrococcygeal teratoma. Report of a case and literature review.
| journal = Arch. Pathol. Lab. Med.
| volume = 117
| issue = 7
| pages = 724-8
| year = 1993
| pmid = 8323438
| doi =
| issn =
}} Synopsis:  A 40 year old man has widely metastatic [[adenocarcinoma]] arising from the residual coccyx remaining after surgical removal of an apparently benign SCT at age 2 months.</ref>  Late malignancies usually involve incomplete excision of the coccyx and are [[adenocarcinoma]].


==See also==
==See also==
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==References==
==References==
<references/>
{{reflist|2}}
 
==External links==
*[http://www.thedoctorsdoctor.com/diseases/sacrococcygeal_teratoma.htm The Doctor's Doctor page on SCT]
* [http://groups.google.com/groups?q=(sct+OR+teratoma)+group%3Amisc.kids.pregnancy+author%3Apologirl&start=0&scoring=d&num=10&lr=&as_drrb=q&as_mind=1&as_minm=1&as_miny=2006&as_maxd=8&as_maxm=4&as_maxy=2007&safe=off& small SCT diagnosed at 20 weeks gestation, pregnancy and neonatal period]
* [http://www.sctsurvivor.com/ medium size benign SCT diagnosed via ultrasound, with complications, delivered preterm via cesarean section]
* [http://folks.harbornet.com/raneem/birthstory.html large benign SCT diagnosed via routine prenatal ultrasound, delivered near term via cesarean section]
* [http://tantinespicknmix.blogspot.com/search/label/SCT large benign SCT delivered full term vaginally, initially misdiagnosed as [[spina bifida]], correctly diagnosed at age 5 weeks, in 1962]
* [http://groups.google.com/groups?as_q=&num=10&scoring=d&as_epq=Á.G.&as_oq=Nicolas+teratoma&as_eq=&as_ugroup=alt.support.cancer&as_usubject=&as_uauthors=&lr=&as_drrb=q&as_qdr=&as_mind=1&as_minm=1&as_miny=2003&as_maxd=8&as_maxm=4&as_maxy=2007&safe=off benign (?) SCT, surveilance after neonatal surgery]
* [http://survivor-support.rare-cancer.org/child-stories/germ-cell-tumor.html SCT diagnosed at age 2½ years, by then malignant, with endodermal sinus tumor]
* [http://groups.google.com/groups?as_q=teratoma&num=10&scoring=r&as_epq=&as_oq=&as_eq=&as_ugroup=alt.support.cancer&as_usubject=&as_uauthors=ruth&lr=&as_drrb=q&as_qdr=&as_mind=1&as_minm=1&as_miny=1981&as_maxd=8&as_maxm=4&as_maxy=2007&safe=off can women born with SCT expect difficulties having children?]
 
 


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