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 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 .10 11 “-oma” denotes a .1 Parizek et al reported a mature teratoma containing the lower half of a human body in Incidence one of fraternal twins.13 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 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 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 .5–7 nantly a pelvic mass extending into the . 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 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 . 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 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, , 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 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 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 fistula.21 An increased risk to the mother’s life or her anterior location of the tumour could thus future fertility. result in the absence of rectum and anus.21 The The combination of fetal hydrops and sacro- physical presence of a teratoma could also pre- coccygeal teratoma is considered to be lethal.32 vent fusion of the genital folds, resulting in a Nakayama et al, however, reported two such bifid scrotum and hypospadias.21 cases who survived after preterm delivery by Other associated anomalies include spinal .32 They found that the dysraphism, sacral agenesis, dislocation of the combination of sacrococcygeal teratoma and hips caused by a large tumour, and menin- hydrops had a particular eVect on pulmonary gocele.8 Rarely cardiac anomalies such as a function and fluid balance. Removal of tumour ventricular septal defect or gastrointestinal led to an immediate improvement in respira-

www.postgradmedj.com 756 Tuladhar, Patole, Whitehall Postgrad Med J: first published as 10.1136/pgmj.76.902.754 on 1 December 2000. Downloaded from

tory function. They proposed the following labour.18 Robertson et al reported devasculari- tumour related factors contributing to respira- sation of a sacrococcygeal teratoma to decrease tory insuYciency: the chance of spontaneous haemorrhage in a + the increased blood volume represented by preterm neonate39; antenatal ultrasound had tumour circulation may have exceeded the confirmed the highly vascular nature of capacity of baby’s lungs to arterialise blood; tumour, with several large feeding vessels aris- + venous blood from the tumour may have ing from the internal iliac arteries. Both O contributed to a low mixed venous P 2 and internal iliac arteries and the small middle sac- pulmonary vasoconstriction; excision may ral artery were ligated, after stabilisation of res- have removed the source of desaturated piratory status.39 acidaemic blood and relieved pulmonary Delivery should be conducted in a tertiary vasoconstriction; hospital. The size of a sacrococcygeal teratoma + fetal ascites and abdominal distension may have embarrassed lung development in determines the mode of delivery. Non-vascular tumours and those less than 10 cm in diameter utero, resulting in reduced lung volumes; 18 + ascites raised the diaphragm after birth and may be delivered vaginally. However, Gross et paracentesis thus improved pulmonary al recommended caesarean delivery in all function. fetuses with sacrococcygeal teratomas of more than 5 cm diameter, to minimise the risk of Investigations rupture and haemorrhage.40 Tumour size has Sacrococcygeal teratomas should be excised been more than 10 cm in almost all reported soon after delivery to avoid tumour ulceration cases with diYcult deliveries.14 41–43 Teratomas and haemorrhage and to reduce the risk of should be well protected after delivery because malignant change.18 The following investiga- erosion of the surface may precipitate bleeding tions are recommended before surgery: before surgical excision is undertaken.44 45 + x rays of spine, abdomen, chest, and Blood loss in large and even benign tumours to detect evidence of tumour or metasta- has been reported to be substantial, sometimes 33 34 sis ; equalling the patient’s blood volume.46 Most + ultrasound to define the extent, nature reviews report mortality ranging from around (cystic or solid) of the mass, the presence 5% to 9% following exsanguinating haemor- and size of solid elements or calcification, rhage.14 34 47 48 and to recognise intrapelvic extension35; Grosfeld et al reported disseminated intra- computed tomography to delineate the + vascular coagulation in a neonate who suVered bony pelvic structures and to identify small tumour rupture and haemorrhage during areas of intrapelvic extension, but this 34 involves radiation of the gonads; it is best delivery. The aetiology of such clotting abnormalities is probably complex and multi- performed following administration of a 45 contrast agent835; factorial. Extensive disruption of large, defec- +magnetic resonance imaging (MRI) also tive tumour endothelium during labour and delineates bony and muscular pelvic struc- delivery could precipitate fulminant dissemi- tures and can characterise the nature of the nated intravascular coagulation. Trauma to the http://pmj.bmj.com/ mass and detect intrapelvic extension836; sacrococcygeal teratoma during delivery may Hata et al reported facilitation of antenatal cause entry of tissue thromboplastin into the diagnosis of sacrococcygeal teratoma by blood stream, resulting in activation of the combined use of Doppler sonography and coagulation cascade.45 Good vascular access MRI37; and adequate supply of blood products is thus + angiography to demonstrate the main necessary before surgery.

blood vessels supplying sacrococcygeal During surgery, precautions must be taken on September 29, 2021 by guest. Protected copyright. teratomas—the middle and lateral sacral to prevent hypothermia, which is easily precipi- and gluteal branches of the internal iliac tated because of the large surface area and the artery and a branch from the profundus vascularity of the tumour.44 The treatment of femoris artery; as hypervascularity, irregu- choice for sacrococcygeal teratoma is early sur- larly encased vessels, arteriovenous shunt- gical resection with complete excision of the ing, and serpentine dilated veins may be coccyx, because microscopic nests of neoplas- seen in malignant as well as in benign tic cells are commonly found in or immediately tumours, angiography is not conclusive in adjacent to the coccyx.515A recurrence rate of distinguishing benign from malignant dis- ease8; 37% was reported when the coccyx was not removed completely.51 Patients with malignant + Gross et al have discussed the role of myelography in evaluating the intraspinal sacrococcygeal teratomas are managed after extension of sacrococcygeal teratomas.38 surgery with irradiation if residual is present, and always with combination chemo- 115 Management at delivery therapy. The management of sacrococcygeal teratomas depends on fetal gestational age and wellbeing, Postoperative complications the presence of associated anomalies, and The main postoperative complication is wound tumour vascularity assessed by colour flow because of the proximity to the anus Doppler.18 Highly vascular tumours should be of the surgical site and the skin flaps that may delivered by caesarean section to avoid risk of be needed.859 Bladder dysfunction may oc- haemorrhage during the second stage of cur.83447

www.postgradmedj.com Perinatal sacrococcygeal teratoma 757 Postgrad Med J: first published as 10.1136/pgmj.76.902.754 on 1 December 2000. Downloaded from

Follow up/recurrence went early surgery with complete resection. The recurrence rate of sacrococcygeal terato- Seven were long term survivors. Two died from mas varies between 7.5% and 22%.48 49 In con- non-disease-related causes. One child whose trast to previous reports,14 50, Bilik et al found a lesion was observed after birth presented with higher recurrence rate for primary sacrococcy- metastatic disease at 10 months of age and geal teratomas with larger mean diameters underwent incomplete resection at that time. (11.1 (3.2) cm with recurrence v 7.9 (4.4) cm She died despite adjuvant treatment. Three with no recurrence (mean (SD)); p = 0.07).49 other patients presenting between seven and 30 They concluded that despite multiple histo- months of age without evidence of metastatic logical sections in large primary tumours disease underwent complete resection and reported as benign, these large tumours may were long term survivors.9 well harbour undetectable small foci of malig- Schropp et al reported their experience with nant endodermal sinus cells. sacrococcygeal teratomas in children over four Serum á fetoprotein in the first month of life decades (1950 to 1990).54 Of 73 teratomas, 57 and immunohistochemical markers are not (78%) were benign and 16 (22%) malignant. reported to be of prognostic significance.50 There were five recurrences in children with However, after total resection of primary benign disease, only one of which did not have sacrococcygeal teratoma together with the coc- an initial . The average age at cyx, a raised serum á fetoprotein has been presentation of benign sacrococcygeal terato- found to be a reliable marker of recurrence of maswas20daysv468 days in those with poorly diVerentiated yolk sac tumours.51 Bilik et malignant disease. Seven of the 16 children al reported markedly raised serum á fetopro- with malignant sacrococcygeal teratomas were tein in malignant recurrent compared with long term survivors. The average survival was benign sacrococcygeal teratomas.49 As none of 9.3 months in the nine children who died the variables except tumour size was found to (range 1 to 28 months). Only two of 10 cases be a reliable predictor of recurrence, these (diagnosed between 1976 and 1991) undergo- investigators concluded that meticulous rou- ing chemotherapy and radiotherapy for malig- tine physical examination is essential every nant disease died with disease (80% survival). three to six months for at least the first three Three deaths occurred in those with benign postoperative years to detect recurrence.49 sacrococcygeal teratomsa (95% survival). One They also emphasised the importance of regu- neonate died in the immediate postoperative lar serum á fetoprotein measurements, prompt period because of complications (rupture of radiographic investigations to detect possible hepatic subcapsular haematoma and dissemi- recurrences (for example where there is raised nated intravascular coagulation with sepsis).54 á fetoprotein but clinically undetectable Maturation from malignant to benign sacro- tumour), and the need to regard every coccygeal teratoma after chemotherapy (pseu- recurrence as potentially malignant.49 doretroconversion) has been reported.54 55 The potential for late occurrence of malig- nancy in mature sacrococcygeal teratoma is well documented.52 53 Lack et al reported Sequelae of sacrococcygeal teratoma infiltration of virtually the entire coccyx with an The type of the teratoma and the surgical http://pmj.bmj.com/ 40 years after the resection at approach both contribute to functional seque- two months of age of a congenital mature lae. Tumours with a large presacral component teratoma.52 Lahdenne et al followed up 45 may present with lower extremity weakness or patients (aged 4–43 years, mean 21.5 years) for paralysis, constipation, abdominal distension, detection of late recurrences.53 All had been and urinary tract symptoms secondary to blad- operated on in infancy for benign sacrococcy- der outlet obstruction.56–58 Isolated cases of

geal teratomas. Three recurrences (two benign lower extremity weakness or paralysis and on September 29, 2021 by guest. Protected copyright. and one malignant) were diagnosed 21 to 43 bladder dysfunction have been described post- years after the initial diagnosis and operative operatively, particularly in association with treatment; coccygectomy had not been per- malignant lesions. Many of the previously formed as a primary procedure in patients with reported large series did not report long term malignant recurrence. Rescorla et al reported faecal and .34 47 56 59–63 En- recurrent disease in nine of 80 patients (11%) gelskirchen et al reported electromanometri- with mature teratomas, at between six and 34 cally detected rectal and bladder dysfunction in months after resection.9 Three had metastatic 40% of children following excision of sacrococ- disease. Two were long term survivors after cygeal teratoma, although none had overt surgery alone. Six were alive at a mean follow incontinence.63 Malone et al reported on 27 up time of 114 months after chemotherapy (5) patients followed up for a mean period of five and pelvic irradiation (1). One patient with years (range 2–12).64 Eleven (41%) had some metastatic disease was lost to follow up. This form of functional impairment. Faecal and uri- series also included cases of immature ter- nary incontinence were present in nine. Two atoma and endodermal sinus tumour (EST). had lower extremity weakness because of Of 24 children with immature teratoma sciatic nerve palsy.64 Tumours with large followed up for an average of 39 months, one intrapelvic extensions requiring an abdomino- (4.2%) had a rising á fetoprotein level 6 peritoneal approach for resection were associ- months after the initial procedure and required ated with a higher incidence (67%) of func- resection. The child was free of disease 33 tional sequelae. It was concluded that it was months after the second procedure. Of the 13 impossible to determine retrospectively neonates and infants with EST, nine under- whether dysfunction was caused by the tumour

www.postgradmedj.com 758 Tuladhar, Patole, Whitehall Postgrad Med J: first published as 10.1136/pgmj.76.902.754 on 1 December 2000. Downloaded from

itself or by the surgery.64 Rintala et al evaluated 24 Gergely RZ, Eden R, Schinfrin BS. Antenatal diagnosis of congenital sacral teratoma. J Reprod Med 1975;24:229–31. quality of life and faecal continence in 26 adult 25 Kohga S, Nambu T, Tanaka K. of placenta patients (mean age 30 years) who had and sacrococcygeal teratoma. Virchows Arch A 1980;386: 223–9. undergone surgery for benign sacrococcygeal 26 Bond SJ, Harrison MR, Schmidt KG. Death due to teratoma in infancy.65 Good faecal continence high-output cardiac failure in fetal sacrococcygeal teratoma. J Pediatr Surg 1990;25:1287–91. was reported by 88% of these patients. 27 Inou M, Kubota A, Hasegawa T, et al. Antenatal diagnosis of However, only 27% had completely normal sacrococcygeal teratoma with hydrops fetalis. Eur J Pediatr bowel habits. Some faecal soiling was present Surg 1994;4:125–7. 28 Zaninovic AC, Westra SJ, Hall TR, et al. Congenital bladder in 27%. No correlation between the severity of rupture and urine ascites secondary to a sacrococcygeal ter- anorectal malfunction and the degree of atoma. Pediatr Radiol.1992;22:509–11. 29 Elchalal U, Ben-Shachar I, Nadjari M, et al. Prenatal intrapelvic extension of the tumour was found. diagnosis of acute bladder distension associated with fetal Other health problems, including urinary sacrococcygeal teratomaa case report. Prenat Diagn 1995; 15:1160–4. incontinence, were reported by 13 patients 30 Garcia AM, Morgan WM, Bruner JP. In utero decompres- (50%). All control subjects (26 healthy patients sion of a cystic grade IV sacrococcygeal teratoma. Fetal Diagn Ther 1998;13:305–8. of similar age and sex distribution) had good 31 Chisholm CA, Heider AL, Kuller JA, et al. Prenatal diagno- faecal continence; 77% had completely normal sis and perinatal management of fetal sacrococcygeal 65 teratoma. Am J Perinatol 1999;16:47–50. bowel habits. Hypergonadotropic hypogo- 32 Nakayama DK, Killian A, Hill LM, et al. The newborn with nadism and sperm abnormalities have been hydrops and sacrococcygeal teratoma. J Pediatr Surg 1991;26:1435–8. reported in men born with benign sacrococcy- 33 Mahour GH, Wooley MM, Trivedi SN. Sacrococcygeal geal teratomas.66 Such patients may have teratoma: a 33 year experience. J Pediatr Surg 1975;10:183– 8. Leydig cell dysfunction, abnormal spermato- 34 Grosfeld JL, Ballantine TVN, Lowe D. Benign and genesis, or both. The association of these with malignant teratomas in children: analysis of 85 patients. sacrococcygeal teratomas may reflect a con- Surgery 1976;80:297–305. 66 35 Feldman M, Byrne P, Johnson MA, et al. Neonatal genital germ cell defect. sacrococcygeal teratoma: multiimaging modality assess- ment. Am J Obstet Gynecol 1990;163:675–82. 36 Cohen MD. Clinical utility of magnetic resonance imaging 1 Arceci RJ, Weinstein HJ. Neoplasia. In: Avery GB, Fletcher in pediatrics. Am J Dis Child 1986;140:947–56. MA, MacDonald MG, eds. Neonatology, pathophysiology and 37 Hata K, Hata T, Kitao M. Antenatal diagnosis of sacrococ- management of the newborn. Philadelphia: J B Lippincott, cygeal teratoma facilitated by combined use of Doppler 1994:1219–20. sonography and MR imaging. Am J Roentgenol l991;156: 2 Winderl LM, Silverman RK. Prenatal identification of a 1115–16. completely cystic internal sacrococcygeal teratoma (type 38 Gross RE, Clatworthy HW, Meeker IA. Sacrococcygeal ter- IV). Ultrasound Obstet Gynecol 1997;9:425–8. atoma in infants and children: report of 40 cases. Surg 3 Ballantyne JW. Teratologie. Williams and Nougate, 1874. Gynecol Obstet 1951;92:309–18. 4 Gross RE, Clatworthy HW, Meeker IA. Sacrococcygeal ter- 39 Robertson FM, Crombleholme TM, Frantz ID. Devascu- atoma in infants and children: report of 40 cases. Surg larization and staged resection of giant sacrococcygeal Gynecol Obstet 1951;92:341–52. teratoma in the preterm . J Pediatr Surg 1995;30:309– 5 Izant RJ, Filston HC. Sacrococcygeal teratomas: analysis of 11. 43 cases .Am J Surg 1975;130:617–21. 40 Gross SJ, Benzie RJ, Sermer M, et al. Sacrococcygeal 6 Ein SH, Adeyemi SD, Mancer K. Benign sacrococcygeal teratoma: prenatal diagnosis and management. Am J Obstet teratoma in infants and children: a 25 year review. Ann Surg Gynecol 1987;156:393–6. 1980;191:382–4. 41 Viller MR. Dystocia caused by sacrococcygeal teratoma. 7 Moazam F, Talbert JL. Congenital anorectal malformations: Two case reports. Obstet Gynecol 1969;34:783. harbingers of sacrococcygeal teratomas. Arch Surg 1985; 42 Weiss DB, Wajntraub G, Abulafla Y. Vaginal surgical inter- 120:856–9. vention for sacrococcygeal teratoma obstructing labour. 8 Keslar PJ, Buck JL, Suarez ES. Germ cell tumors of the sac- Acta Obstet Gynecol Scand 1976;55:183–6. rococcygeal region: radiologic-pathologic correlation. 43 SchiVer MA, Greenberg E. Sacrococcygeal teratoma in

Radiographics 1994;14:607–22. labour and the newborn. Am J Obstet Gynecol 1956;72: http://pmj.bmj.com/ 9 Rescorla FJ, Robert SS, Arnold GC, et al. Long-term 1054–8. outcome for infants and children with sacrococcygeal 44 Teitelbaum D, Teich S, Cassidy S, et al. Highly vascularized teratoma: a report from the Children’s Group. J sacrococcygeal teratoma: description of this atypical variant Pediatr Surg 1998;33:171–6. and its operative management. J Pediatr Surg 1994;29:98– 10 Juric-Lekic G, Trosic M, Svajger A. Lentinoids within 101. sacrococcygeal teratoma: origin by transdiVerentiation? 45 Murphy JJ, Blair GK, Fraser GC. Coagulopathy associated Hum Pathol 1993;24:227–9. with large sacrococcygeal teratoma. J Pediatr Surg 1992;27: 11 Consolato S, Volker E, Bernhard B. Huge fetal sacrococcy- 1308–10. geal teratoma with a completely formed eye and intratu- 46 Smith B, Passaro E, Clatworthy HW. The vascular anatomy moral DNA ploidy heterogeneity. Pediatr Dev Pathol 1999;2: of sacrococcygeal teratoma; its significance in surgical man- 50–7. agement. Surgery 1961;49:534–9. 12 Valdisserri RO, Yunis EJ. Sacrococcygeal teratomas. Cancer 47 Noseworthy J, Jack EE, Kosakewich HPW. Sacrococcygeal on September 29, 2021 by guest. Protected copyright. 1981;48:217–21. germ cell tumors in childhood: an updated experience with 13 Parizek J, Nemecek S, Pospisilova B, et al. Mature sacrococ- 118 patients. J Pediatr Surg 1981;16:358–64. cygeal teratoma containing the lower half of a human body. 48 Dewan PA, Davidson PM, Campbell PE. Sacrococcygeal Child Nerv Syst 1992;8:108–10. teratoma: has chemotherapy improved survival? J Pediatr 14 Altman RP, Randolph JG, Lilly JR. Sacrococcygeal ter- Surg 1987;22:274–7. atoma. American Academy of Pediatrics Surgical Section 49 Bilik R, Shandling B, Pope M, et al. Malignant benign neo- survey.1973. J Pediatr Surg 1974;9:389–98. natal sacrococcygeal teratoma. J Pediatr Surg 1993;28:1158– 15 Brinker MR, Sheldin RG, Moynihan PC. Sacrococcygeal 60. teratoma in children. J La State Med Soc 1989;141:26–31. 50 Lahdenne P, Heikinheimo M, Periko M. Cell diVerentiation 16 Murphy JJ, Blair GK, Fraser GC. Coagulopathy associated in a sacrococcygeal teratoma. An immunohistochemical and with large sacrococcygeal teratomas. J Pediatr Surg 1992;27: follow up study. Pathol Res Pract 1990;186:336–43. 1308–10. 51 Crussi FG, Winkler RF, Mirkin DL. Sacrococcygeal 17 Graf JL, Housely HT, Albanese CT, et al. A surprising histo- teratoma in infant and children. Arch Pathol Lab Med 1978; logical evolution of preterm sacrococcygeal teratoma. J 102:420–5. Pediatr Surg 1998;33:177–9. 52 Lack EE, Glaun RS, Hefter LG, et al. Late occurrence of 18 Kum CK, Wong YC, Prabhakaran K. Management of fetal following resection of a histologically mature sacrococcygeal teratoma. Ann Acad Med 1993;22:377–80. sacrococcygeal teratoma. Arch Pathol Lab Med 1993;117: 19 Holterman AX, Filiatrault D, Lallier M, et al. The natural 724–7. history of sacrococcygeal teratomas diagnosed through rou- 53 Lahdenne P, Heikinheimo M, Nikkanen V, et al. Neonatal tine obstetric sonogram: a single institution experience. J benign sacrococcygeal teratoma may recur in adulthood and Pediatr Surg 1998;33:899–903. give rise to malignancy. Cancer 1993;15:3727–31. 20 Carney JA, Thompson DP, Johnson CL. Teratomas in 54 Schropp KP, Lobe TE, Rao B. Sacrococcygeal teratoma: the children: clinical and pathologic aspects. J Pediatr Surg experience of four decades. J Pediatr Surg 1992;27:1075–9. 1972;7:271–86. 55 Cranston PE, Smith EE, Hamrick-Turner J Emergence of 21 Subbarao P, Bhatnagar V, Mitra DK. The association of mature teratoma following treatment of sacrococcygeal sacrococcygeal teratoma with high anorectal and genital endodermal sinus tumour: CT and MR imaging with malformations. Aust NZ J Surg 1994;64:214–15. pathological correlation. Pediatr Radiol 1994;24:239–40. 22 Vogl D, Riel KA. Prasakraler Tumor mit sakraler Menin- 56 Leung AKC, Rubin SZ, Seagram GF, et al. Sacrococcygeal gozele. Z Kinderchir 1988;43:361–4. teratoma. Aust Paediatr J 1985;21:123–5. 23 Lahdenne P, Heikinheimo M, Jaaskelainen J, et al. Vertebral 57 Whalen TV, Mahour GH, Landing BH, et al. Sacrococcy- abnormalities associated with congenital sacrococcygeal ter- geal teratomas in infants and children. Am J Surg 1985;150: atomas. J Pediatr Orthop 1991;11:603–7. 373–5.

www.postgradmedj.com Perinatal sacrococcygeal teratoma 759 Postgrad Med J: first published as 10.1136/pgmj.76.902.754 on 1 December 2000. Downloaded from

58 Ashcraft KW, Holder TM. Hereditary presacral teratoma. J 63 Engelskirchen R, Holschneider AM, Rhein R, et al. Pediatr Surg 1974;9:691–7. Sacrococcygeal teratomas in children. An analysis of long 59 Tapper D, Lack EE. Teratomas in infancy and childhood. A term results in 87 children. Z Kinderchir 1987;42:358–61. 54 year experience at the Children’s Hospital Medical Cen- 64 Malone PS, Spitz L, Kiely EM, et al. The functional sequelae tre. Ann Surg 1983;198:398–410. of sacrococcygeal teratoma J Pediatr Surg 1990;25:679–80. 60 Gwinn JL, Dockerry MB, Kennedy RLJ. Presacral terato- 65 Rintala R, Lahdenne P, Lindahl H, et al. Anorectal function mas in infancy and childhood. Pediatrics 1955;16:239–49. in adults operated for a benign sacrococcygeal teratoma. J 61 Billmire DF, Grosfeld JL. Teratomas in childhood: an analy- Pediatr Surg 1993;28:1165–7. sis of 142 cases. J Pediatr Surg 1986;21:548–51. 66 Lahdenne P, Dunkel L, Heikinheimo M, et al. Hypergona- 62 Kirk D, Lister J. Urinary complications of sacrococcygeal dotropic hypogonadism and sperm abnormalities with teratoma. Z Kinderchir 1976;18:294–304. benign sacrococcygeal teratomas. J Androl 1991;12:226–30. http://pmj.bmj.com/ on September 29, 2021 by guest. Protected copyright.

www.postgradmedj.com