SPECIAL FEATURE SECTION EDITOR: WALTER W. TUNNESSEN, JR, MD Picture of the Month Alexander K. C. Leung, MBBS; C. Pion Kao, MD FEMALE INFANT was born to a gravida 3, para 1, 25-year-old woman at 36 weeks’ gestation. The infant was delivered by cesarean section. A huge mass was attached to the infant’s lower back (Figure). From the Department of Pediatrics, University of Calgary and Alberta Children’s Hospital, Calgary. Figure. (REPRINTED) ARCH PEDIATR ADOLESC MED/ VOL 154, MAR 2000 WWW.ARCHPEDIATRICS.COM 309 ©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Denouement and Discussion Sacrococcygeal Teratoma A huge sacrococcygeal teratoma is attached to the infant’s lower back. The high-output cardiac failure, placentomegaly, fetal growth cephalic portion of the tumor is necrotic. retardation, and hydrops fetalis.7 Severe dystocia with neo- natal death may occur if the tumor is unsuspected prena- acrococcygeal teratomas, although rare, are the most tally and the infant is delivered vaginally. Approximately frequently recognized neoplasm in fetuses,1 with an 15% of infants with these tumors have associated congen- incidence of approximately 1 in 35 000 to 40 000 ital anomalies such as anorectal malformations, sacral bone S 1 8 live births. More common in female than male infants (4: defects, and genitourinary abnormalities. 12), a familial predisposition is present in some cases.3 The incidence of twins in the immediate family varies from 14% DIAGNOSTIC EVALUATION to 50%. A lateral radiograph of the abdomen may show anterior PATHOGENESIS displacement of the rectum and possible calcification within the tumor. Abdominal and pelvic ultrasonogra- Teratomas are congenital tumors composed of tissues de- phy, computed tomography scanning, and magnetic reso- rived from all 3 embryonic germinal layers. Postsacral tu- nance imaging help assess internal extension of the mors are thought to arise from multipotential embryonic tumor, involvement of other organs, and existence of meta- cells that occur in greatest concentration in the area of the static lesions. Serum a1-fetoprotein levels are elevated in 70% primitive knot (Hensen node) and presacral tumors from of children with malignant tumors, with normal levels in incomplete migration of germ cells from the yolk sac to the children with benign tumors.9 urogenital ridge.3 The female gonads of the fetus termi- nate their differentiation at 10 weeks’ gestation in contrast TREATMENT AND PROGNOSIS to 7 weeks’ for the male. The multipotential cells, there- fore, remain longer in the female gonads and are more prone The treatment of choice is complete resection of the tu- to disturbances, which may account for the higher inci- mor and coccyx. Failure to remove the coccyx will re- dence of sacrococcygeal teratomas in females.4 Sacrococ- sult in local recurrence in 30% of cases.10 Malignant re- cygeal teratomas may be the result of twinning or abortive currences have been reported in patients with previously attempts at twinning, which may account for the higher benign tumors.11 If the tumor is not resectable, chemo- incidence in monozygotic twins. therapy may shrink it and render it resectable. The prog- nosis is excellent for children whose tumors are ex- MANIFESTATIONS AND COMPLICATIONS cised, along with the coccyx, soon after birth, with a cure rate of 90%.11 Patients with malignant sacrococcygeal tu- Clinically, the tumor most commonly presents as a mass mors have a poor prognosis (mortality rate .90%). protruding between the coccyx and the anus, is usually cov- Accepted for publication September 10, 1999. ered with normal intact skin. Rarely, the skin may be necrotic Reprints: Alexander K. C. Leung, MBBS, Alberta Chil- owing to compromise in blood supply. Sacrococcygeal tera- dren’s Hospital, 1820 Richmond Rd SW, Calgary, Alberta, tomas almost always arise from the tip of the coccyx and vary Canada T2T 5C7. greatly in the amount of external vs internal tissue exten- sions. About 1% are intrapelvic with no external presenta- REFERENCES tion and can only be diagnosed by rectal examination.5 They 1. Havranek P, Hedlund H, Rubenson A, et al. Sacrococcygeal teratoma in Sweden can be classified according to location into 4 types: type I, between 1978 and 1989. J Pediatr Surg. 1992;27:916-918. predominantly external with only a minimal presacral com- 2. Azizkhan RG, Caty MG. Teratomas in childhood. Curr Opin Pediatr. 1996;8:287-292. ponent; type II, external but with considerable intrapelvic 3. Castleberry RP, Cushing B, Perlman E, Hawkins EP. Sacrococcygeal tumors. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 3rd extension; type III, apparent externally but the predominant ed. Philadelphia, Pa: Lippincott-Raven Publishers; 1997:921-945. mass is pelvic and extends into the abdomen; and type IV, 4. Leung AK, Rubin SZ, Seagram GF, Hwang WS. Sacrococcygeal teratoma. Aust Paediatr J. 1985;21:123-125. presacral with no external presentation. 5. Liu KK, Lee KH, Ku KW. Sacrococcygeal teratoma in children: a diagnostic chal- Approximately 2% to 10% of sacrococcygeal teratomas lenge. AustNZJSurg. 1994;64:102-105. are malignant before the affected infant is age 2 months, 50% 6. Reinberg Y, Long R, Manivel JG, Resnick J, Simonton S, Gonzalez R. Urological aspects of sacrococcygeal teratoma in children. J Urol. 1993;150:948-949. are malignant by age 1 year, and almost 100% are malignant 7. Robertson FM, Crombleholme TM, Frantz ID III, Shepard BA, Bianchi DW, D’Alton by age 5 years.6 Malignant transformation is most likely to ME. Devascularization and staged resection of giant sacrococcygeal teratoma in occur between age 4 months and 5 years.6 A tumor with a the premature infant. J Pediatr Surg. 1995;30:309-311. 8. Areci RJ, Weinstein HJ. Neoplasia. In: Avery GB, Fletcher MA, MacDonald MG, large presacral component may cause obstruction of the rec- eds. Neonatology: Pathophysiology and Management of the Newborn. Philadel- tum, colon, or urinary tract.5 Occasionally, it may cause phia, Pa: JB Lippincott Co; 1994:1211-1228. 9. Broadbent VA. Malignant disease in the neonate. In: Roberton NR, ed. Textbook venous or lymphatic obstruction and paresis or paralysis of of Neonatology. Edinburgh, Scotland: Churchill Livingstone Inc; 1986:689-695. the lower limbs, especially if it is malignant. 10. Matthay KK. Congenital malignant disorders. In: Taeusch HW, Ballard RA, Avery Patients with large tumors early in gestation have a ME, eds. Shaffer and Avery’s Diseases of the Newborn. 6th ed. Philadelphia, Pa: 7 WB Saunders Co; 1991:1025-1048. mortality rate higher than 50%. It has been suggested that 11. Belik R, Shandling B, Pope M, Thorner P, Weitzman S, Ein SH. Malignant benign arteriovenous shunting through a large tumor results in neonatal sacrococcygeal teratoma. J Pediatr Surg. 1993;28:1158-1160. (REPRINTED) ARCH PEDIATR ADOLESC MED/ VOL 154, MAR 2000 WWW.ARCHPEDIATRICS.COM 310 ©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021.