Sacrococcygeal teratoma: Difference between revisions

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==Lab tests==
==Lab tests==
==[[Sacrococcygeal teratoma echocardiography or ultrasound|Echocardiography or Ultrasound]]==
==[[Sacrococcygeal teratoma echocardiography or ultrasound|Echocardiography or Ultrasound]]==
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]].


==Treatment==
==Treatment==

Revision as of 18:59, 13 January 2012

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Natural history, Complications, and Prognosis

Differentiating Sacrococcygeal teratoma from other Diseases

Diagnosis

History & Symptoms

Physical Examination

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Echocardiography or Ultrasound

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.

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:

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.[1][2]

In many cases, a fetus with a small SCT (under 5 or 10 cm) may be delivered vaginally.[3][4][5][6]

Complications

Maternal complications of pregnancy may include mirror syndrome.[7] Maternal complications of delivery may include a Cesarean section or, alternatively, a vaginal delivery with mechanical dystocia.[8]

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).[9] In the fetus, severe hydronephrosis may contribute to inadequate lung development. Also in the fetus and newborn, the anus may be 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.[10] Removal of the coccyx may include additional complications. In one review of 25 patients,[11] however, the most frequent complication was an unsatisfactory appearance of the surgical scar.

Late effects

Pelvic scarring may necessitate that pregnant women who are SCT survivors deliver their babies by [Cesarean section].[citation needed]

Complications of not removing the coccyx may include both recurrence of the teratoma[12] and metastatic cancer.[12][13] Late malignancies usually involve incomplete excision of the coccyx and are adenocarcinoma.

See also

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ McCurdy CM, Seeds JW (1993). "Route of delivery of infants with congenital anomalies". Clinics in perinatology. 20 (1): 81–106. PMID 8458172.
  6. ↑ 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.
  7. ↑ Finamore PS, Kontopoulos E, Price M, Giannina G, Smulian JC (2007). "Mirror syndrome associated with sacrococcygeal teratoma: a case report". The Journal of reproductive medicine. 52 (3): 225–7. PMID 17465292.
  8. ↑ Nalbanski B, Markov D, Brankov O (2007). "[Sacrococcygeal teratoma--a case report and literature review]". Akusherstvo i ginekologii͡a (in Bulgarian). 46 (2): 41–5. PMID 17469451.
  9. ↑ Galili O, Mogilner J (2005). "Type IV sacrococcygeal teratoma causing urinary retention: a rare presentation". J. Pediatr. Surg. 40 (2): E18–20. doi:10.1016/j.jpedsurg.2004.10.003. PMID 15750911.
  10. ↑ Engelskirchen R, Holschneider AM, Rhein R, Hecker WC, Höpner F (1987). "[Sacral teratomas in childhood. An analysis of long-term results in 87 children]". Zeitschrift für Kinderchirurgie : organ der Deutschen, der Schweizerischen und der Osterreichischen Gesellschaft für Kinderchirurgie = Surgery in infancy and childhood (in German). 42 (6): 358–61. PMID 3439358.
  11. ↑ 16373161&dopt=AbstractPlus PubMed
  12. ↑ 12.0 12.1 Lahdenne P, Heikinheimo M, Nikkanen V, Klemi P, Siimes MA, Rapola J (1993). "Neonatal benign sacrococcygeal teratoma may recur in adulthood and give rise to malignancy". Cancer. 72 (12): 3727–31. PMID 8252490. 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.
  13. ↑ Lack EE, Glaun RS, Hefter LG, Seneca RP, Steigman C, Athari F (1993). "Late occurrence of malignancy following resection of a histologically mature sacrococcygeal teratoma. Report of a case and literature review". Arch. Pathol. Lab. Med. 117 (7): 724–8. PMID 8323438. 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.

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