754 Postgrad Med J 2000;76:754–759 Postgrad Med J: first published as 10.1136/pgmj.76.902.754 on 1 December 2000. Downloaded from Sacrococcygeal teratoma in the perinatal period R Tuladhar, S K Patole, J S Whitehall Teratomas are formed when germ cell tumours tissues are less commonly identified.12 An ocu- arise from the embryonal compartment. The lar lens present as lentinoids (lens-like cells), as name is derived from the Greek word “teratos” well as a completely formed eye, have been which literally means “monster”. The ending found within sacrococcygeal teratomas.10 11 “-oma” denotes a neoplasm.1 Parizek et al reported a mature teratoma containing the lower half of a human body in Incidence one of fraternal twins.13 Sacrococcygeal teratoma is the most common congenital tumour in the neonate, reported in Size approximately 1/35 000 to 1/40 000 live Size of a sacrococcygeal teratoma (average 8 births.2 Approximately 80% of aVected infants cm, range 1 to 30 cm) does not predict its bio- are female—a 4:1 female to male preponder- logical behaviour.8 Altman et al have defined ance.2 the size of sacrococcygeal teratomas as follows: The first reported case was inscribed on a small, 2 to 5 cm diameter; moderate, 5 to 10 Chaldean cuneiform tablet dated approxi- cm diameter; large, > 10 cm diameter.14 mately 2000 BC.3 In the modern era, the first large series of infants and children with sacro- Site coccygeal teratomas was reported by Gross et The sacrococcygeal region is the most com- al in 1951.4 mon location. Less common sites are the mediastinum, testes, retroperitoneum, brain, Embryonic origin head and neck, vagina, stomach, and pineal Sacrococcygeal teratomas have tissues derived region.815 Sacrococcygeal teratomas may con- from ectoderm, mesoderm, and endoderm.2 tinue to grow posteriorly to form an external Although their embryonic origin is still uncer- protrusion, or dissect anteriorly, distorting tain, they are believed to arise early in gestation regional organs (rectum, vagina, and bladder) (at around the late second or early third week) without invading them.814 from the totipotential cells of Hensen’s node (also called the primitive knot), a remnant of Extent the primitive streak in the coccygeal region.5–7 The American Academy of Pediatrics’ surgical The primitive streak appears as a linear section (APPSS) classification helps in grading thickening in the ectoderm at the caudal edge the extent of sacrococcygeal teratomas, as of the bilaminar embryonic disc. It usually follows16: diminishes in size, eventually disappearing Type I—Predominantly external with mini- http://pmj.bmj.com/ after undergoing degenerative changes. As the mal presacral component. mesoderm rapidly proliferates, the primitive Type II—Present externally but with signifi- streak comes to lie more and more caudally, cant intrapelvic extension. where the remnant of Hensen’s node descends Type III—Apparent externally but predomi- to the tip or anterior surface of coccyx.5–7 nantly a pelvic mass extending into the abdomen. Types Type IV—Presacral with no external presen- on September 29, 2021 by guest. Protected copyright. Sacrococcygeal teratomas may be classified as tation. benign (mature) and malignant or immature (composed of embryonic elements).8 Mature Histology teratomas are most common in neonates Sacrococcygeal teratomas are graded histologi- (68%) and older children (73%). Immature cally as follows17: teratomas are cystic, whereas malignant tu- Grade 0—Tumour contains only mature tis- mours are solid. Over 50% of sacrococcygeal sue. teratomas have calcification and ossification.8 Grade 1—Tumour contains rare foci of Keslar et al reported that 69 (62%) of the 96 immature tissues. sacrococcygeal teratomas in their series were Grade 2—Tumour contains moderate quanti- Department of composed of both solid and cystic elements.8 ties of immature tissues. Neonatology, Kirwan Hospital for Women, The cysts may be filled with serous fluid, Grade 3—Tumour contains large quantities Queensland 4817, mucoid, or sebaceous material and lined by of immature tissue with or without Australia true epithelium. Ein et al found cystic tumours malignant yolk sac elements. R Tuladhar that were filled with cerebrospinal fluid from Grading of sacrococcygeal teratomas, unlike S K Patole choroid plexus present in the tumour mass.6 that of ovarian teratomas, does not seem to J S Whitehall Virtually any tissue can be present in a correlate directly with prognosis.8 8 10–13 Correspondence to: sacrococcygeal teratoma. Neuroglial tis- Dr Patole sue, skin, respiratory and enteric epithelium, Fetal diagnosis [email protected] cartilage, smooth muscel, and striated muscle Though described as early as the 19th century, 8 Submitted 19 January 2000 are the most common elements found. Bone, it was not until the advent of antenatal Accepted 19 April 2000 pancreatic tissue, choroid plexus, and adrenal ultrasonography that sacrococcygeal teratomas www.postgradmedj.com Perinatal sacrococcygeal teratoma 755 Postgrad Med J: first published as 10.1136/pgmj.76.902.754 on 1 December 2000. Downloaded from anomalies other than imperforate anus have been described.614 Vogl and Riel reported a case with anorectal malformation, sacral dys- plasia, and a presacral mass (Currarino’s triad).22 Lahdenne et al reported vertebral abnormalities in 80% of their 45 patients with benign sacrococcygeal teratoma.23 Perinatal management The perinatal management of sacrococcygeal teratoma requires an interdisciplinary liaison between obstetricians, neonatologists, and pae- diatric surgeons. The high perinatal/neonatal mortality and morbidity rates associated with sacrococcygeal teratoma are attributed to preterm delivery and to complications such as Figure 1 Antenatal ultrasound (saggital section) at 22 malignant invasion, haemorrhage into the weeks of gestation, revealing a large sacrococcygeal teratoma tumour, obstruction of umbilical flow, high (courtesy of Sinh Le and the Department of Radiology, output cardiac failure, hydrops fetalis, and Kirwan Hospital for Women). bladder outlet obstruction.19 24–28 Some of these complications can be detected prenatally and were diagnosed in utero18 (fig 1). The majority treated appropriately.29 Garcia et al reported a of sacrococcygeal teratomas present between case of decompression of a cystic grade IV sac- the 22nd and the 34th week of gestation. Most rococcygeal teratoma in utero through an are benign, resectable after birth without amniotic catheter to alleviate hydronephrosis significant morbidity, and rarely associated and potential renal damage.30 Bond et al postu- with other congenital anomalies. There is thus lated that the tumour acts as an arteriovenous the potential to lead a normal life without dis- fistula, resulting in high output cardiac failure ability.18 The diagnosis of sacrococcygeal ter- with placentomegaly and non-immune hy- atoma on routine sonograms is associated with drops. If this were true then intervention to a greater than expected incidence of prenatal control this circulatory steal may be the only and perinatal complications.19 Close antenatal way to salvage severely aVected fetuses.26 Close follow up is needed to optimise patient follow up with repeated ultrasound scans is counselling and treatment in the presence of a necessary to monitor an increase in tumour completely solid tumour and the onset of poly- size, extension into adjacent structures, tumour hydramnios. A poor outcome is usually corre- vascularity, and evidence of cardiac failure.18 lated with placentomegaly, cardiomegaly, or Volume reduction amniocentesis and tocolysis non-immune hydrops fetalis.19 may be required to treat symptomatic polyhy- dramnios and prevent preterm delivery. Bar- Associated malformations ring the development of hydrops or maternal The incidence of various congenital malforma- complications, delivery should be delayed to http://pmj.bmj.com/ tions associated with sacrococcygeal teratoma allow for fetal maturation. If hydrops develops, ranges from 5% to 26%.4620Of these, anorec- close maternal–fetal observation is needed. tal and genital malformations are of prime Maternal corticosteroids are advisable to accel- concern. The association of sacrococcygeal erate fetal pulmonary maturation.31 Termina- teratoma with anorectal malformations was tion of pregnancy may have to be considered in described as early as 1935.21 During the third cases with bad prognostic signs (for example, week of embryonic life, genital folds unite to cardiac failure, placentomegaly, marked hy- on September 29, 2021 by guest. Protected copyright. form the genital tubercle by migration of mes- drops) or with associated major congenital enchymal cells from the primitive streak region anomalies. Kum et al described the following around the cloacal membrane. During the prerequisites for consideration of fetal inter- fourth and seventh weeks, the cloaca is subdiv- vention in preterm pregnancy18: ided by the urorectal septum to form the + accurate prenatal diagnosis and well anorectal canal and the primitive urogenital defined natural history to allow the confi- sinus.21 A growing sacrococcygeal teratoma dent diagnosis of a correctable lesion that could thus encroach between the layers of the will otherwise prevent fetal survival; cloacal membrane and prevent descent and + absence of other life threatening or debili- fusion of the urorectal septum to the cloacal tating anomalies; membrane, resulting in a high anorectal + ability to perform the procedure without malformation with a rectourethral