Type 2 Sacrococcygeal Teratoma Endodermal Sinus Tumor

Home , Immature teratoma, Neoplasm, Sacrococcygeal teratoma, Teratoma

TE Herman and MJ Siegel Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO, USA

Journal of Perinatology (2011) 31, 804–806; doi:10.1038/jp.2011.30 Sacrococcygeal teratomas are classified according to the Altman classification of the American Academy of Pediatrics, Surgical Case presentation Section. By this system, the tumor in this patient corresponds to a A 3295-gram infant girl was born by elective cesarean section at 37 type 2 lesion. Type 1 is entirely external to the pelvis, type 2 has an weeks gestation to a gravida 2, para 1 mother because of a partially external component as well as a small presacral component, type 3 cystic mass detected at the base of the coccyx sonographically. The has an external component but a large internal component infant had Apgar scores of 9 at both 1 and 5 min. She was in no extending into the abdomen and type 4 is entirely pelvic without an respiratory distress. Her weight was 75th percentile, and her head external mass.1 The majority of patients have type 1 or 2 circumference (35.5 cm) was 90th percentile, consistent with good SCTFapproximately 36% of cases are type 1, 27% type 2, 18% type intrauterine growth. Platelets were normal, at 214 000/cu-mm. 3 and 18% type 4 (ref. 6). Initial radiographs of the chest and abdomen demonstrated normal It is also important to identify the giant or rapidly growing, heart size and moderate-sized caudal midline mass without hypervascular sacrococcygeal teratoma. These hypervascular calcification (Figure 1). An abdominopelvic magnetic resonance imaging (MRI) scan of the infant was performed (Figure 2). This demonstrated a partially solid, partially cystic sacrococcygeal teratoma (SCT), without hypervascularity. The tumor was predominantly external with a small presacral component. Initial alpha-fetoprotein (AFP) level was 49 912 ng mlÀ1 (normal less than 8). The human chorionic gonadotropin level was 10.3 IU lÀ1 (normal less than 5). The patient remained in stable condition and on the seventh postnatal day she was taken to the operating room, where the mass and coccyx were resected.

Denouement and discussion Histological examination of the resected tumor and coccyx demonstrated malignant teratoma with extensive endodermal sinus tumor and tumor at the resected margins. SCT is the most common neoplasm found in newborns, occurring with a striking female predominance of 4 to 6:1 (ref. 1). The tumor arises from embryonic blastema present in the human tail bud, which normally regresses by 32 to 35 days of gestation.2 Sacrococcygeal teratomas have been shown to be an interesting source of human stem cells.2 To guarantee resection of this embryonic blastema, thus reducing the risk of recurrence, coccygectomy is performed at the time of tumor resection. There is a 37% rate of recurrence of sacrococcygeal teratomas if concomitant coccygectomy is not performed.3

Correspondence: Dr TE Herman, Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 South Kings Highway Boulevard, St Louis, MO 63110, USA. E-mail: [email protected] Figure 1 Plain radiograph of the chest and abdomen shows a large pelvic soft Received 8 February 2011; accepted 6 March 2011 tissue mass, consistent with sacrococcygeal teratoma (SCT). Type 2 SCT TE Herman and MJ Siegel 805

Figure 2 Abdominal–pelvic MRI. (a) Coronal and (b) sagittal fat-suppressed, contrast-enhanced T1-weighted images. There is a large complex soft tissue mass abutting the coccyx (arrow). The tumor has external and presecral components and contains cystic elements (cystic sacrococcygeal teratoma (SCT)) and solid elements (solid SCT). The cystic part typically can contain serous or sebaceous material, whereas the solid part can contain hair, fat, and/or immature or malignant tissues. The urinary bladder (B) is anterior and superior. lesions are associated with a very high fetal mortality and a high an external component are characteristically not discovered until risk of exsanguinations at the time of surgical resection unless the after the neonatal period. The risk of malignancy in neonatally tumor is devascularized by ligation of the sacral artery.4 The diagnosed and excised SCT is very low, approximately 6% hypervascular lesions often lead to congestive heart failure from (1 of 17 neonatal lesions). Because of early tumor detection, neonates high output shunting and coagulopathy from intratumoral make up only 7% of the patients with malignant SCT.1,3 Interestingly, bleeding and consumption of clotting factors. Cross-sectional the survival of patients with malignant SCT is very poor with 80% imaging has an important role in the evaluation of SCT, mortality, except in newborns with malignant tumor. particularly of the rapidly growing hypervascular SCT. Both Tumor recurrence is thought to be due to failure to resect sonography and MRI can provide assessment of the vascularity, size completely the tumor and/or coccyx, tumor spillage or failure to and Altman grade of the tumor. Magnetic resonance angiography pathologically detect malignant elements.6 The risk of recurrence is can be used to demonstrate the course and size of the median approximately 10% for benign, mature SCT. However, some benign sacral artery, which is the usual source of blood supply to SCT. The SCT cases unfortunately have recurred with malignant elements. high-contrast resolution of MRI and its ability to identify tumor The risk of recurrence of benign SCT with immature elements is margins and vessels makes it preferable to computed tomography 33%. The risk of recurrence with malignant elements is 18%. The for assessing the tumor extent. lower risk of recurrence of malignant teratoma compared with The mortality in giant hypervascular SCT is particularly high in benign, immature teratoma may be due to the use of adjuvant the presence of placentomegaly and hydrops. If the affected fetus is chemotherapy in some cases. The AFP level is an important marker a singleton, with normal chromosomes, less than 30 weeks of sacrococcygeal teratoma.3 After resection of SCT, the AFP level gestation and has high-output congestive heart failure from a type should fall gradually during the months after resection, and attain 1 or 2 SCT, then fetal debulking can be successfully performed and normal levels by approximately 9 months.7 can be life saving.5 Post-surgical problems include fecal soling, constipation, However, in patients who do not have rapidly growing, neurogenic bladder and urinary tract infections. The risk depends hypervascular SCT, the prognosis depends upon tumor pathology, upon the adequacy of surgical reconstruction of the pelvic ﬂoor, recurrence and functional impairment post resection. In the buttock and sphincter preservation. Currently, the risk of post- neonate without congestive heart failure or coagulopathy, the operative functional impairment at major pediatric surgical centers prognosis is usually good, with 80% of lesions being benign mature is low, with constipation reported as the most common problem.8 teratoma. Yolk sac tumor or endodermal sinus tumor is the most This patient will be followed up with frequent AFP level common malignant element in SCT.3 Malignant tumors are more measurements and intermittently obtained pelvic MRI. often found in older patients (beyond 1 years of age), in solid Chemotherapy will be initiated if there is evidence of tumor tumors and in intrapelvic tumors.3 The risk of malignancy is recurrence based on failure of AFP levels to fall or recurrent mass understandably high in type 4 SCT, because these tumors without accumulation.

Journal of Perinatology Type 2 SCT TE Herman and MJ Siegel 806

Conﬂict of interest 4 Kaneyama K, Yamataka A, Kobayashi H, Lane GL, Itoh S, Kinoshita K et al. Giant highly The authors declare no conﬂict of interest. vascular sacrococcygeal teratoma; report of its excision using the ligaSure vessel sealing system. J Pediatr Surg 2004; 39: 1791–1793. 5 Hedrick HL, Flake AW, Crombleholme TM, Howell LJ, Johnson MP, Wilson RD et al. Sacrococcygeal teratoma: prenatal assessment, fetal intervention and outcome. J Pediatr References Surg 2004; 39: 430–438. 6 DeBacker A, Madern GC, Hakvoort-Cammel FGAJ, Haentjens P, Oosterhuis JW, Hazebrok 1 Gabra JP, Jesudason EC, McDowell HP, Pizer BL, Losty PD. Sacrococcygeal teratoma – a FWJ. Study of factors associated with recurrence in children with sacrococcygeal 25 year experience in a UK regional center. J Pediatr Surg 2006; 41: 1513–1516. teratoma. J Pediatr Surg 2006; 41: 173–181. 2 Busch C, Bareiss PM, Sinnberg T, Just L, Wehrmann M, Fuchs J et al. Isolation of three 7 Barreto MWG, Silva LV, Barini R, Oliveira-Filho AG, Sbragia L. Alpha-fetoprotein stem cell lines from human sacrococcygeal teratomas. J Pathol 2009; 217: 589–596. following neonatal resection of sacrococcygeal teratoma. Pediatr Hematol Oncol 2006; 3 Ein SH, Mancer K, Debo Adenyemi S. Malignant sacrococcygeal teratoma – endodermal 23: 287–291. sinus, yolk sac tumor – in infants and children: 32 year review. J Pediatr Surg 1985; 8 Draper H, Chitayat D, Ein SH, Langer JC. Long-term functional results following 20: 473–477. resection of neonatal sacrococcygeal teratoma. Pediatr Surg Int 2009; 25: 243–246.

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