Sacrococcygeal teratoma natural history, complications and prognosis: Difference between revisions
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===Fetal Complications=== | ===Fetal Complications=== | ||
====Pre-Surgical==== | ====Pre-Surgical==== | ||
*Steal Syndrome: Rapidly growing and solid sacrococcygeal teratoma is highly vascularized tumor and are more likely to divert blood from circulation, leading to [[fetal hydrops]]. | |||
*Mass effect of a large sacrococcygeal teratoma may include [[hip dysplasia]], [[bowel obstruction]], [[urinary obstruction]], [[hydronephrosis]] and [[hydrops fetalis]].<ref name="pmid15750911">{{cite journal | *Mass effect of a large sacrococcygeal teratoma may include [[hip dysplasia]], [[bowel obstruction]], [[urinary obstruction]], [[hydronephrosis]] and [[hydrops fetalis]].<ref name="pmid15750911">{{cite journal | ||
| author = Galili O, Mogilner J | | author = Galili O, Mogilner J | ||
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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.
Natural History
Sacrococcygeal teratomas are the most common type of germ cell tumors (both benign and malignant) diagnosed in neonates, infants, and children younger than 4 years.[1]
Fetal Sacrococcygeal Teratoma
- Presents during prenatal ultrasound exams, with or without maternal symptoms
- Found during routine exams tend to be small and partly or entirely external
- The internal Sacrococcygeal teratomas are not easily seen via ultrasound, unless they are large enough to reveal their presence by the abnormal position of the fetal urinary bladder and other organs, but large fetal SCTs frequently produce maternal complications which necessitate non-routine, investigative ultrasounds.
Neonatal Sacrococcygeal Teratoma
- Presents at birth protruding from the sacral site and is usually mature or immature teratoma
Infants and Young Children Sacrococcygeal Teratoma
- The tumor presents as a palpable mass in the sacropelvic region compressing the bladder or rectum.[2]
- These pelvic tumors have a greater likelihood of being malignant.
Older Children and Adults Sacrococcygeal Teratoma
- The tumor may be mistaken for a pilonidal sinus, or it may be found during a rectal exam or other evaluation.
Complications
Maternal Complications
- Mirror syndrome (also called Ballantynes syndrome) is generalized maternal edema that 'mirrors' the edema of the hydropic fetus and placenta.[3]It can occur with immune or non-immune hydrous fetalis.[4]It presents with peripheral edema leading to rapid weight gain, and pre-eclampsia like symptoms.[5]
- [Cesarean section]] or, alternatively, a vaginal delivery with mechanical dystocia.[6]
Fetal Complications
Pre-Surgical
- Steal Syndrome: Rapidly growing and solid sacrococcygeal teratoma is highly vascularized tumor and are more likely to divert blood from circulation, leading to fetal hydrops.
- Mass effect of a large sacrococcygeal teratoma may include hip dysplasia, bowel obstruction, urinary obstruction, hydronephrosis and hydrops fetalis.[7]
- Even a small SCT can produce complications of mass effect, if it is presacral (Altman Type IV).
- Severe hydronephrosis may contribute to inadequate lung development.
- The anus may be imperforate.
- Urinary incontinence, fecal incontinence, and other chronic problems resulting from accidental damage to nerves and muscles within the pelvis.[8]
Post-Surgical
- Removal of the coccyx may include additional complications.
- The most frequent complication was an unsatisfactory appearance of the surgical scar.[9]
- Pelvic scarring may necessitate that pregnant women who are sacrococcygeal teratoma survivors deliver their babies by [Cesarean section].
- If coccyx is not removed during surgery, patient may have recurrence of the teratoma[10] and metastatic cancer.[10][11]
Prognosis
Imaging studies may be used to study prognostic factors of sacrococcygeal teratoma.
Poor Prognostic Factors
- Ultrasound showing tumor volume to fetal weight ratio (TFR) >.12 before 24 weeks of gestation[12]
- Ultrasound showing TFR ≥0.11 before 32 weeks of gestation
- Solid tumor volume to head volume ratio >1[13]
Favorable Prognostic Factors
- Cystic morphology[12]
- Tumors at age < 2 months are usually benign.
References
- ↑ (PDQ) Sacrococcygeal Tumors in Children
- ↑ Rescorla FJ: Pediatric germ cell tumors. Semin Surg Oncol 16 (2): 144-58, 1999. PUBMED Abstract
- ↑ 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.
- ↑ Finamore PS, Kontopoulos E, Price M, Giannina G, Smulian JC (2007). "Mirror syndrome associated with sacrococcygeal teratoma: a case report". J Reprod Med. 52 (3): 225–7. PMID 17465292.
- ↑ van Selm M, Kanhai HH, Gravenhorst JB (1991). "Maternal hydrops syndrome: a review". Obstet Gynecol Surv. 46 (12): 785–8. PMID 1780115.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 16373161&dopt=AbstractPlus PubMed
- ↑ 10.0 10.1 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.
- ↑ 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.
- ↑ 12.0 12.1 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.
- ↑ Sy ED, Filly RA, Cheong ML, Clifton MS, Cortes RA, Ohashi S, Takifuji K, Ventura DE, Grethel EJ, Wagner AJ, Gress EA, Ball RH, Lee H, Harrison MR (2009). "Prognostic role of tumor-head volume ratio in fetal sacrococcygeal teratoma". Fetal. Diagn. Ther. 26 (2): 75–80. doi:10.1159/000238113. PMID 19752518.