Sacrococcygeal teratoma surgery: Difference between revisions

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==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]].
[[Perinatal period|Perinatal]] [[Surgery|surgical]] intervention is used to decrease [[cardiovascular]] [[Complication (medicine)|complications]] caused by the large sacrococcygeal teratoma. Early complete [[resection]] is the mainstay of management of [[benign tumor]]. Complete [[Surgery|surgical]] [[excision]] in [[malignant]] sacrococcygeal teratoma is followed by [[Chemotherapy|platinum based chemotherapy]].


==Management of fetal SCTs==
==Surgery==
===Benign Sacrococcygeal Teratoma===
===Perinatal Management===
*Early complete resection is the mainstay of management of benign tumor.<ref>{{cite journal |vauthors=Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, Weetman R, Cullen J, Rogers P, Vinocur C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP |title=Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study |journal=J. Clin. Oncol. |volume=17 |issue=7 |pages=2137–43 |year=1999 |pmid=10561269 |doi= |url=}}</ref>
[[Perinatal period|Perinatal]] intervention is only used to decrease [[cardiovascular]] [[Complication (medicine)|complications]] caused by the parasitic mass.<ref name="tt">{{cite journal |vauthors=Roybal JL, Moldenhauer JS, Khalek N, Bebbington MW, Johnson MP, Hedrick HL, Adzick NS, Flake AW |title=Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas |journal=J. Pediatr. Surg. |volume=46 |issue=7 |pages=1325–32 |year=2011 |pmid=21763829 |doi=10.1016/j.jpedsurg.2010.10.020 |url=}}</ref>
*Complete excision must include removal of coccyx and early ligation of sacral vessels.<ref>{{cite journal |vauthors=Ein SH, Mancer K, Adeyemi SD |title=Malignant sacrococcygeal teratoma--endodermal sinus, yolk sac tumor--in infants and children: a 32-year review |journal=J. Pediatr. Surg. |volume=20 |issue=5 |pages=473–7 |year=1985 |pmid=3903096 |doi= |url=}}</re
====Open Fetal Surgery====  
*Open [[Fetus|fetal]] [[surgery]] is the option at some specialized centers.
*[[Contraindication]] to open [[Fetus|fetal]] [[surgery]]:
:*Type III or IV Altman type [[Tumor|tumors]]
:*Severe [[placentomegaly]]
:*[[Cervix|Cervical]] shortening
:*Maternal [[medical conditions]]
====Minimally Invasive in Utero Procedures====
*[[Laser ablation]]<ref>{{cite journal |vauthors=Makin EC, Hyett J, Ade-Ajayi N, Patel S, Nicolaides K, Davenport M |title=Outcome of antenatally diagnosed sacrococcygeal teratomas: single-center experience (1993-2004) |journal=J. Pediatr. Surg. |volume=41 |issue=2 |pages=388–93 |year=2006 |pmid=16481257 |doi=10.1016/j.jpedsurg.2005.11.017 |url=}}</ref><ref>{{cite journal |vauthors=Hecher K, Hackelöer BJ |title=Intrauterine endoscopic laser surgery for fetal sacrococcygeal teratoma |journal=Lancet |volume=347 |issue=8999 |pages=470 |year=1996 |pmid=8618503 |doi= |url=}}</ref>
*[[Radiofrequency ablation]]<ref>{{cite journal |vauthors=Lam YH, Tang MH, Shek TW |title=Thermocoagulation of fetal sacrococcygeal teratoma |journal=Prenat. Diagn. |volume=22 |issue=2 |pages=99–101 |year=2002 |pmid=11857611 |doi= |url=}}</ref><ref>{{cite journal |vauthors=Paek BW, Jennings RW, Harrison MR, Filly RA, Tacy TA, Farmer DL, Albanese CT |title=Radiofrequency ablation of human fetal sacrococcygeal teratoma |journal=Am. J. Obstet. Gynecol. |volume=184 |issue=3 |pages=503–7 |year=2001 |pmid=11228510 |doi=10.1067/mob.2001.110446 |url=}}</ref>
*[[Urinary bladder|Bladder]] drainage for [[obstructive uropathy]]<ref>{{cite journal |vauthors=Wilson RD, Hedrick H, Flake AW, Johnson MP, Bebbington MW, Mann S, Rychik J, Liechty K, Adzick NS |title=Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome |journal=Fetal. Diagn. Ther. |volume=25 |issue=1 |pages=15–20 |year=2009 |pmid=19122459 |doi=10.1159/000188056 |url=}}</ref><ref>{{cite journal |vauthors=Scrimgeour EM, Brown P |title=BSE and potential risks to slaughtermen |journal=Vet. Rec. |volume=129 |issue=17 |pages=390–1 |year=1991 |pmid=1746122 |doi= |url=}}</ref>
*[[Cyst]] [[aspiration]]<ref>{{cite journal |vauthors=Lee MY, Won HS, Hyun MK, Lee HY, Shim JY, Lee PR, Kim A |title=Perinatal outcome of sacrococcygeal teratoma |journal=Prenat. Diagn. |volume=31 |issue=13 |pages=1217–21 |year=2011 |pmid=22024911 |doi=10.1002/pd.2865 |url=}}</ref>
===Delivery Option===
'''Fetus with High-risk Sacrococcygeal Teratoma'''<ref name="tt">{{cite journal |vauthors=Roybal JL, Moldenhauer JS, Khalek N, Bebbington MW, Johnson MP, Hedrick HL, Adzick NS, Flake AW |title=Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas |journal=J. Pediatr. Surg. |volume=46 |issue=7 |pages=1325–32 |year=2011 |pmid=21763829 |doi=10.1016/j.jpedsurg.2010.10.020 |url=}}</ref>
*Early [[Childbirth|delivery]] by [[Caesarean section|cesarean section]] after 28 weeks of [[gestation]]
'''Fetus with Low-risk Sacrococcygeal Teratoma'''<ref name="tt">{{cite journal |vauthors=Roybal JL, Moldenhauer JS, Khalek N, Bebbington MW, Johnson MP, Hedrick HL, Adzick NS, Flake AW |title=Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas |journal=J. Pediatr. Surg. |volume=46 |issue=7 |pages=1325–32 |year=2011 |pmid=21763829 |doi=10.1016/j.jpedsurg.2010.10.020 |url=}}</ref>
*[[Childbirth|Delivery]] by [[Caesarean section|cesarean section]] after 36 weeks of [[gestation]]
'''Fetus with Small Tumor''' '''(< 5cm)'''<ref>{{cite journal |vauthors=Okada T, Sasaki F, Cho K, Honda S, Naito S, Hirokata G, Todo S |title=Management and outcome in prenatally diagnosed sacrococcygeal teratomas |journal=Pediatr Int |volume=50 |issue=4 |pages=576–80 |year=2008 |pmid=18937757 |doi=10.1111/j.1442-200X.2008.02703.x |url=}}</ref>
*[[Vagina|Vaginal]] [[Childbirth|delivery]]


*If complete resection can't be achieved with first surgery, a second surgery must be performed.<ref>{{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>
===Postnatal Management===
====Benign Sacrococcygeal Teratoma====
Early complete [[resection]] is the mainstay of management of [[benign tumor]].<ref name="txchemo">{{cite journal |vauthors=Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, Weetman R, Cullen J, Rogers P, Vinocur C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP |title=Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study |journal=J. Clin. Oncol. |volume=17 |issue=7 |pages=2137–43 |year=1999 |pmid=10561269 |doi= |url=}}</ref>
*Complete [[excision]] must include removal of [[coccyx]] and early ligation of [[Sacrum|sacral]] [[Blood vessel|blood vessels]].<ref>{{cite journal |vauthors=Ein SH, Mancer K, Adeyemi SD |title=Malignant sacrococcygeal teratoma--endodermal sinus, yolk sac tumor--in infants and children: a 32-year review |journal=J. Pediatr. Surg. |volume=20 |issue=5 |pages=473–7 |year=1985 |pmid=3903096 |doi= |url=}}</ref>
*If complete [[resection]] can not be achieved with first [[surgery]], a second [[surgery]] must be performed.<ref>{{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>


 
====Malignant Sacrococcygeal Teratoma====
===Malignant Sacrococcygeal Teratoma===
*Complete [[Surgery|surgical]] [[excision]] in [[malignant]] sacrococcygeal teratoma is followed by [[Chemotherapy|platinum based chemotherapy]].  
 
*Most widely used combination of [[chemotherapy]] is [[Bleomycin]], [[etoposide]], [[cisplatin]] or [[carboplatin]].
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>
==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.
 
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.


==References==
==References==
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Latest revision as of 21:16, 7 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

Perinatal surgical intervention is used to decrease cardiovascular complications caused by the large sacrococcygeal teratoma. Early complete resection is the mainstay of management of benign tumor. Complete surgical excision in malignant sacrococcygeal teratoma is followed by platinum based chemotherapy.

Surgery

Perinatal Management

Perinatal intervention is only used to decrease cardiovascular complications caused by the parasitic mass.[1]

Open Fetal Surgery

Minimally Invasive in Utero Procedures

Delivery Option

Fetus with High-risk Sacrococcygeal Teratoma[1]

Fetus with Low-risk Sacrococcygeal Teratoma[1]

Fetus with Small Tumor (< 5cm)[9]

Postnatal Management

Benign Sacrococcygeal Teratoma

Early complete resection is the mainstay of management of benign tumor.[10]

Malignant Sacrococcygeal Teratoma

References

  1. ↑ 1.0 1.1 1.2 Roybal JL, Moldenhauer JS, Khalek N, Bebbington MW, Johnson MP, Hedrick HL, Adzick NS, Flake AW (2011). "Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas". J. Pediatr. Surg. 46 (7): 1325–32. doi:10.1016/j.jpedsurg.2010.10.020. PMID 21763829.
  2. ↑ Makin EC, Hyett J, Ade-Ajayi N, Patel S, Nicolaides K, Davenport M (2006). "Outcome of antenatally diagnosed sacrococcygeal teratomas: single-center experience (1993-2004)". J. Pediatr. Surg. 41 (2): 388–93. doi:10.1016/j.jpedsurg.2005.11.017. PMID 16481257.
  3. ↑ Hecher K, Hackelöer BJ (1996). "Intrauterine endoscopic laser surgery for fetal sacrococcygeal teratoma". Lancet. 347 (8999): 470. PMID 8618503.
  4. ↑ Lam YH, Tang MH, Shek TW (2002). "Thermocoagulation of fetal sacrococcygeal teratoma". Prenat. Diagn. 22 (2): 99–101. PMID 11857611.
  5. ↑ Paek BW, Jennings RW, Harrison MR, Filly RA, Tacy TA, Farmer DL, Albanese CT (2001). "Radiofrequency ablation of human fetal sacrococcygeal teratoma". Am. J. Obstet. Gynecol. 184 (3): 503–7. doi:10.1067/mob.2001.110446. PMID 11228510.
  6. ↑ Wilson RD, Hedrick H, Flake AW, Johnson MP, Bebbington MW, Mann S, Rychik J, Liechty K, Adzick NS (2009). "Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome". Fetal. Diagn. Ther. 25 (1): 15–20. doi:10.1159/000188056. PMID 19122459.
  7. ↑ Scrimgeour EM, Brown P (1991). "BSE and potential risks to slaughtermen". Vet. Rec. 129 (17): 390–1. PMID 1746122.
  8. ↑ Lee MY, Won HS, Hyun MK, Lee HY, Shim JY, Lee PR, Kim A (2011). "Perinatal outcome of sacrococcygeal teratoma". Prenat. Diagn. 31 (13): 1217–21. doi:10.1002/pd.2865. PMID 22024911.
  9. ↑ Okada T, Sasaki F, Cho K, Honda S, Naito S, Hirokata G, Todo S (2008). "Management and outcome in prenatally diagnosed sacrococcygeal teratomas". Pediatr Int. 50 (4): 576–80. doi:10.1111/j.1442-200X.2008.02703.x. PMID 18937757.
  10. ↑ Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, Weetman R, Cullen J, Rogers P, Vinocur C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP (1999). "Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study". J. Clin. Oncol. 17 (7): 2137–43. PMID 10561269.
  11. ↑ Ein SH, Mancer K, Adeyemi SD (1985). "Malignant sacrococcygeal teratoma--endodermal sinus, yolk sac tumor--in infants and children: a 32-year review". J. Pediatr. Surg. 20 (5): 473–7. PMID 3903096.
  12. ↑ 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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