Sacrococcygeal teratoma surgery: Difference between revisions

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==Management of fetal SCTs==
==Management of fetal SCTs==
===Perinatal Management===
*Perinatal intervention is only used to decrease cardiovascular complications caused by parasitic mass.<ref>{{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>
*Open fetal surgery is the option at some specialized centers.
:*Contraindication:
::*Type III or IV Altman type tumors
::*Severe placentomegaly
::*Cervical shortening
::*Maternal medical issues
*Minimally invasive in utero procedures include:
::*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>
::*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>
===Benign Sacrococcygeal Teratoma===
===Benign Sacrococcygeal Teratoma===
*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>
*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>

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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 (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.

Management of fetal SCTs

Perinatal Management

  • Perinatal intervention is only used to decrease cardiovascular complications caused by parasitic mass.[1]
  • Open fetal surgery is the option at some specialized centers.
  • Contraindication:
  • Type III or IV Altman type tumors
  • Severe placentomegaly
  • Cervical shortening
  • Maternal medical issues
  • Minimally invasive in utero procedures include:
  • Radiofrequency ablation[4][5]
  • Bladder drainage for obstructive uropathy[6][7]
  • Cyst aspiration[8]


Benign Sacrococcygeal Teratoma

  • Early complete resection is the mainstay of management of benign tumor.[9]
  • Complete excision must include removal of coccyx and early ligation of sacral vessels.


Malignant Sacrococcygeal Teratoma

Management of most fetal SCTs involves watch and wait, also known as expectant management. An often used decision tree is as follows:

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.[10][11]

In many cases, a fetus with a small SCT (under 5 or 10 cm) may be delivered vaginally.[12][13][14][15]

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, teratomas are classified according to a cancer staging system. This indicates whether chemotherapy or radiation therapy may be needed.

References

  1. 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. 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.
  10. Mazneĭkova V, Dimitrova V (1999). "[Prenatal ultrasonographic diagnosis of four cases of sacrococcygeal teratoma]". Akusherstvo i ginekologii͡a (in Bulgarian). 38 (1): 64–9. PMID 11965727.
  11. Sheil AT, Collins KA (2007). "Fatal birth trauma due to an undiagnosed abdominal teratoma: case report and review of the literature". The American journal of forensic medicine and pathology : official publication of the National Association of Medical Examiners. 28 (2): 121–7. doi:10.1097/01.paf.0000257373.91126.0d. PMID 17525561.
  12. Anteby EY, Yagel S (2003). "Route of delivery of fetuses with structural anomalies". Eur. J. Obstet. Gynecol. Reprod. Biol. 106 (1): 5–9. PMID 12475573.
  13. Ruangtrakool R, Nitipon A, Laohapensang M; et al. (2001). "Sacrococcygeal teratoma: 25 year experience". Journal of the Medical Association of Thailand = Chotmaihet thangphaet. 84 (2): 265–73. PMID 11336088.
  14. McCurdy CM, Seeds JW (1993). "Route of delivery of infants with congenital anomalies". Clinics in perinatology. 20 (1): 81–106. PMID 8458172.
  15. Kainer F, Winter R, Hofmann HM, Karpf EF (1990). "[Sacrococcygeal teratoma. Prenatal diagnosis and prognosis]". Zentralblatt für Gynäkologie (in German). 112 (10): 609–16. PMID 2205995.

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