Sacrococcygeal teratoma natural history, complications and prognosis
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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
Depending on the extent of the tumor invasion at the time of diagnosis, the prognosis may vary. Most of the sacrococcygeal teratomas are benign in nature and prognosis is generally regarded as good after resection. The sacrococcygeal teratoma with cystic morphology is associated with the most favorable prognosis.
Natural History, Complications, and Prognosis
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
- Found during prenatal ultrasonography, with or without maternal symptoms
- Tends 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
- Large fetal sacrococcygeal teratomas frequently produce maternal complications which necessitate non-routine, investigative ultrasonography.
Neonatal Sacrococcygeal Teratoma
- The tumor presents as a protruding mass from the sacral region and is usually mature or immature teratoma.
Sacrococcygeal Teratoma in Infants and Young Children
- The tumor presents as a palpable mass in the sacro-pelvic region compressing the bladder or rectum.[2]
- These pelvic tumors have a greater likelihood of being malignant.
Sacrococcygeal Teratoma in Older Children and Adults
- The tumor may be mistaken for a pilonidal sinus, or it may be found during a rectal exam.[1]
Complications
Maternal Complications
- Mirror syndrome (also called Ballantynes syndrome) is generalized maternal edema that mirrors the edema of the hydropic fetus and placenta.[3]
- The need for Cesarean section[4]
- Preterm labor
Fetal Complications
- Steal Syndrome: Solid and rapidly growing sacrococcygeal teratoma is highly vascularized tumor and is more likely to divert blood from circulation, leading to fetal hydrops.
- Mass effects of a large sacrococcygeal teratoma may include hip dysplasia, bowel obstruction, urinary obstruction, hydronephrosis and hydrops fetalis.[5]
- Even a small sacrococcygeal teratoma can produce complications of mass effect, if it is pre-sacral (Altman Type IV).
- Severe hydronephrosis may contribute to inadequate lung development.
- The anus may be imperforated.
- Urinary incontinence, fecal incontinence, and other chronic problems resulting from accidental damage to nerves and muscles within the pelvis[6]
Complications Related to the Treatment
- Removal of the coccyx may include additional complications.
- The most frequent complication is an unsatisfactory appearance of the surgical scar.[7]
- 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, the patient may have recurrence of the teratoma and metastatic cancer.[8]
- Bowel and bladder incontinence is observed due to soiling of the malignant cells.[9]
Prognosis
- Depending on the extent of the tumor invasion at the time of diagnosis, the prognosis may vary.
- Most of the sacrococcygeal teratomas are benign in nature and prognosis is generally regarded as good after resection of the tumor. [10]
Poor Prognostic Factors
- Ultrasound showing tumor volume to fetal weight ratio (TFR) > 0.12 before 24 weeks of gestation[11]
- Ultrasound showing TFR ≥ 0.11 before 32 weeks of gestation
- Solid tumor volume to head volume ratio > 1[12]
- Placentomegaly
- Solid and vascular tumor
- Hydrops fetalis
- High output cardiac failure[13]
Favorable Prognostic Factors
- Cystic morphology[11]
- Tumors at age < 2 months are usually benign.
References
- ↑ 1.0 1.1 Sacrococcygeal teratoma. Cancer.gov (2015)(PDQ) Sacrococcygeal Tumors in Children Accessed on November 2th, 2015
- ↑ 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.
- ↑ 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.
- ↑ Surgical experience and cosmetic outcomes in children with sacrococcygeal teratoma. PubMed (2006) 16373161&dopt=AbstractPlus PubMed Accessed on November 24th, 2015
- ↑ 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.
- ↑ Partridge EA, Canning D, Long C, Peranteau WH, Hedrick HL, Adzick NS, Flake AW (2014). "Urologic and anorectal complications of sacrococcygeal teratomas: prenatal and postnatal predictors". J. Pediatr. Surg. 49 (1): 139–42, discussion 142–3. doi:10.1016/j.jpedsurg.2013.09.042. PMID 24439598.
- ↑ Sacrococcygeal teratoma. Chop.Edu (2015) http://www.chop.edu/conditions-diseases/sacrococcygeal-teratoma-sct/about#.VnhH0bRYZFI Accessed on November 24th, 2015
- ↑ 11.0 11.1 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.
- ↑ Seung Mi Lee, Dong Hoon Suh, So Yeon Kim, Min Kyoung Kim, Sohee Oh, Sang Hoon Song, Hyun-Young Kim, Chan-Wook Park, Joong Shin Park & Jong Kwan Jun (2018). "Antenatal Prediction of Neonatal Survival in Sacrococcygeal Teratoma". Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. 37 (8): 2003–2009. doi:10.1002/jum.14553. PMID 29399854. Unknown parameter
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