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Sacrococcygeal teratoma: Prenatal diagnosis and management
Sacrococcygeal teratoma: Prenatal diagnosis and management Susan J. Gross, M.D., Ronald J. Benzie, M.B., Ch.B., Mathew Sermer, M.D., Martin B. Skidmore, M.B., and Stephanie R. Wilson, M.D. Toronto, Ontario, Canada Although sacrococcygeal teratoma is a rare and potentially malignant tumor, 10 cases were documented during a 5-year period at the University of Toronto Perinatal Complex. Diagnosis was made in the six ca~es in which prenatal ultrasound examination was performed. One patient with twins elected to terminate the pregnancy at 19 weeks. In three of the cases diagnosed prenatally, serial ultrasound was performed. There was a 75% cesarean section rate. In all cases diagnosed prenatally, the large tumor size affected the mode of delivery. In the four cases without prenatal diagnosis, two infants were delivered vaginally, and two were delivered abdominally for obstetric reasons. There was one case of neonatal morbidity where tumor vascularity and rupture resu.lted in hypovolemic shock. All tumors were resected and found to be benign. A plan of management is recommended and, with appropriate obstetric and pediatric care, a good outcome can be anticipated in most cases. (AM J OssTET GvNECOL 1987;156:393-6.)
Key words: Teratoma, sacrococcygeal, pregnancy Sa~rococcygeal teratoma is a congenital tumor arising from all three germ cell layers. It has an incidence of one to two per 40,000 deliveries' with only isolated reports in the literature. The importance of neonatal diagnosis is twofold. Ten percent of these tumors may be malignant at the time of delivery.2 They may grow to a large size, affecting fetal outcome and labor management. The objectives of this study were to examine the prenatal diagnosis and the antepartum and intrapartum management with subsequent outcome.
Methods Cases of sacrococcygeal teratoma during a 5-year period (from 1980 through 1985) from four tertiary referral centers forming the University of Toronto Perinatal Complex were studied retrospectively. Six cases, including one set of twins, originated from the complex itself, while four were referred from outlying hospitals. Prenatal assessment, diagnosis, fetal weight and sex, size of tumor, mode of delivery, and outcome were documented. A diagnosis of sacrococcygeal teratoma required histologic confirmation. Results A total of IO cases were recorded, with one set of female twins and a female: male ratio of 9: l. In six of the cases, ultrasonographic diagnosis of sacrococcygeal
From the University of Toronto Perinatal Complex (Toronto General Hospital, Women's College Hospital, Mount Sinai Hospital, and The Hospital for Sick Children). Presented at the Forty-second Annual Meeting of The Society of Obstetricians and Gynaecologists of Canada, Charlottetown, Prince Edward Island, Canada, June 23-27, 1986. Reprint requests: Dr. R. J. Benzie, Ottawa General Hospital, 501 Smyth, Ottawa, Ontario, Canada KIH 8L6.
teratoma was made, a large-for-dates uterus being the indication for the ultrasound examination. No other phenotypic abnormalities were identified although there were three cases of polyhydramnios. Because of prenatal diagnosis of sacrococcygeal teratoma in both twins, one patient requested and obtained termination of pregnancy at 19 weeks (Figs. l and 2). The remaining four cases were diagnosed at the time of delivery. The maternal serum o:-fetoprotein and amniotic fluid o:-fetoprotein and acetylcholinesterase had not been tested. There was a 50% (four of eight) prematurity rate. The overall average gestational age was 35.5 ± 4 weeks. There was a 75% (six of eight) cesarean section rate. In the four cases where prenatal diagnosis was made by ultrasound, the presence of a large tumor (average volume 2340 ± 1026 cm 3 ) affected the mode of delivery. In none of these cases was the infant delivered immediately after the diagnosis was made. In three of them, serial ultrasound examinations were performed and the fetus was delivered abdominally because of an abnormal nonstress test, premature rupture of the membranes, and polyhydramnios, respectively. In the four cases without prenatal diagnosis, two infants were delivered vaginally. The average volume of these tumors was relatively small (average volume 317 ± 197 cm'). The remaining two infants were delivered by cesarean section for obstetric reasons (transverse lie and failure to progress, respectively, Table I). Significant morbidity was seen in one case. Because of the large size and vascularity of the tumor, the infant developed hypovolemic shock during the resection operation resulting in subsequent pre-renal failure. Fig. 3 illustrates the size and vascularity of the teratoma. The infant received appropriate management and was normal at follow-up 2 years later. 393
394
Gross et al.
February 1987 Am J Obstet Gynecol
Fig. I. Long-axis ultrasound of twin A, showing caudal unilocular cystic mass.
Fig. 2. Long-axis ultrasound of twin B, showing large caudal multilocular cystic mass.
There was one neonatal death secondary to prematurity and one death at 3 1/2 years of age secondary to malignant endodermal sinus tumor, apparently unrelated to the original lesion. The tumor was resected during the first week of life in surviving neonates and all the tumors were benign. No associated anomalies were found prenatally by ultrasound or at subsequent operation.
Comment Our series was consistent with some known features of sacrococcygeal teratoma. It is more common in female infants (75% to 80%) 2 and there is concordance
between twins. 3 As with many congenital malformations, sacrococcygeal teratoma is associated with polyhydramnios. 4 None of the tumors in our series, however, were malignant. No associated anomalies of the lower vertebrae, anorectum, or genitourinary system were documented. With respect to prenatal diagnoses, while none of the cases were tested for elevated amniotic fluid a-fetoprotein and acetylcholinesterase levels, these enzymes have been found to be elevated in sacrococcygeal teratoma, especially in the second trimester. 5 Although elevation of these enzymes would not likely have affected the diagnosis in these cases, further study in this area is
Sacrococcygeal teratoma 395
Volume 156 Number 2
Fig. 3. Female newborn infant with sacrococcygeal teratoma, 32 weeks' gestation, after cesarean section.
Table I. Summary of cases of sacrococcygeal teratoma Case No. I
2 3 4 5 6 7 8 9 JO
Birth weight Fetal sex
(gm)
F F (Twin A) F (Twin B) F F F F M F F
5000 2300 4600 2070 3960 5460 2910 3160
Gestational age at diagnosis (wk)
Gestational age at delivery (wk)
23 24 19 ·19 30 27 40 40 37 38
36 34 19 19 32 27 40 40 37 38
warranted. As seen in our series, where no ultrasonographic errors were made, careful ultrasonography can be a very accurate method of prenatal diagnosis and assessment. The important differential diagnosis of meningomyelocele and to a lesser extent hemangioma should not be forgotten. We therefore recommend that, while a rare entity, sacrococcygeal teratoma should be suspected in a largefor-dates patient, and ultrasound examination should be performed in order to make the diagnosis prenatally. These patients should be evaluated by serial ultrasonography to assess tumor size. Appropriate counseling at the time of diagnosis is essential, not only in order to discuss possible termination but also to adequately educate and prepare parents for the impending labor, delivery, and management. The literature documents different modes of management: (1) vaginal delivery,' (2) cesarean section, 1 (3) cesarean section after partial delivery because of dystocia,6 (4) vaginal drainage of tumor followed by vaginal
Prenatal diagnosis
Ultrasonographic Ultrasonographic Ultrasonographic Ultrasonographic Ultrasonographic Ultrasonographic
Tumor size at birth (cm)
Mode of delivery
27 by 15 by 8 14 by JO by 10 1.5 by 1.5 6 (diameter) 25 by I I by 13 5 by 5 by 5 IO by 7 by 7 17 by 15 by 17 5 (diameter) 6 by 4 by 5
Cesarean section Cesarean section Elective abortion Elective abortion Cesarean section Cesarean section Vaginal Cesarean section Cesarean section Vaginal
delivery,7 and (5) abdominal drainage of tumor followed by vaginal delivery. 8 While mode of delivery has been individualized in the literature, tumor size is critical in this decision for two reasons. In our two cases of vaginal delivery, the tumors were small enough for delivery; however, a large tumor can cause serious obstetric complications such as abnormal lie and dystocia. More importantly, as seen in our one case of hypotensive shock, a large sacrococcygeal teratoma can be highly vascular, thereby causing exsanguination or rupture on aspiration. The literature documents cases of neonatal death after rupture of sacrococcygeal teratoma during vaginal delivery." On the basis of the above literature review and our own series of cases, we feel it reasonable to suggest that, except for very small tumors (
Gross et al.
be obtained with good outcome anticipated in most cases. We would like to thank Dr. Yung and Dr. Claessens for providing cases for this study. REFERENCES 1. Salaymel MT. Giant sacrococcygeal teratoma in the newborn. Int Surg 1971 ;56:56. 2. Seeds JW, Mittelstaedt CA, Cefalo RC, Parker TF. Prenatal diagnosis of a sacrococcygeal teratoma-an anechoic caudal mass.JCU 1982;10:193. 3. Lees RF, Williamson BRJ, Brenbridge ANAG, Buschi AJ, Teja K. Sonography of benign sacral teratoma in utero. Radiology 1980;134:717. 4. Horger EO, McCarter LM. Prenatal diagnosis of sacrococcygeal teratoma. AM J 0BSTET GYNECOL 1979; r34:228.
February 1987 Am J Obstet Gynecol
5. Brock DJH, Richmond DH, Liston WA. Normal secondtrimester amniotic fluid alphafetoprotein and acetylcholinesterase associated with fetal sacrococcygeal teratoma. Prenat Diagn 1983;3:343. 6. Musci MC, Clark MJ, Ayres RE, Finkel MA. Management of dystocia caused by a large sacrococcygeal teratoma. Obstet Gynecol 1982;62: 105. 7. Weiss DB, Wajntraub G, Abulafia Y, Schiller M. Vaginal surgical intervention for a sacro-coccygeal teratoma obstructing labour. Acta Obstet Gynecol Scand 1976;55:183. 8. Tanaree P. Delivery obstructed by sacrococcygeal teratoma. AM j 0BSTET GYNECOL 1982; 142:239. 9. Guigiaro A, Boario U, Fraceso G, Freni G. Three cases of intrapartum rupture of sacrococcygeal teratoma [in Italian]. Minerva Pediatr 1977;29:1517.
Human chorionic gonadotropin, prolactin, estriol, and dehydroepiandrosterone sulfate concentrations in cord blood of premature and term newborn infants: Relationship to the sex of the neonate Basil Ho Yuen, M.B., Ch.B., and Everett K. Mincey, Ph.D. Vancouver, British Columbia, Canada Although the composition of human chorionic gonadotropin, prolactin, estriol, and dehydroepiandrosterone sulfate in the umbilical cord blood has been studied, less information is available on the effect of fetal sex on the cord blood concentrations. We assayed these hormones in cord blood from 405 newborn infants delivered between 23 and 43 weeks of gestation. In pooled data for both sexes, human chorionic gonadotropin showed a declining trend from the beginning to the end of the sampling interval. By contrast, in 398 newborn infants of known sex, prolactin, estriol, and dehydroepiandrosterone sulfate levels increased during the same period. Sex differences were observed for human chorionic gonadotropin only; female newborn infants had higher cord blood concentrations throughout the sampling period, with the differences becoming statistically significant between 29 and 36 weeks. (AM J OssrET GvNECOL 1987; . 156:396-400.)
Key words: Newborn infants, sex, chorionic gonadotropin, prolactin, estriol, dehydroepiandrosterone sulfate, cord blood Human chorionic gonadotropin, prolactin, estriol, and dehydroepiandrosterone sulfate are important
From the Gynaecologic Endocrine Laboratory, Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynaecology, Faculty of Medicine, The University of British Columbia, and Metropolitan Biomedical Laboratories. Presented at the Forty-second Annual Meeting of The Society of Obstetricians and Gynaecologists of Canada, Charlottetown, Prince Edward Island, Canada, June 23-27, 1986. Reprint requests: Dr. B. Ho Yuen, Department of Obstetrics and Gynaecology, The University of British Columbia, 4490 Oak St., Vancouver, British Columbia, Canada V6H 3V5.
396
polypeptide and steroid hormones produced in the fetal-placental unit. Although their composition in the umbilical cord blood has been studied, less information is available on the effect of fetal sex on the cord blood concentrations.'· 2 In the present study, we measured the levels of these hormones in the cord blood of newborn infants from mid pregnancy to term to determine whether there were any sex differences in these levels.
Material and methods At the time of delivery, mixed umbilical cord blood was collected into tubes containing ethylenediamine-
Key words: Teratoma, sacrococcygeal, pregnancy Sa~rococcygeal teratoma is a congenital tumor arising from all three germ cell layers. It has an incidence of one to two per 40,000 deliveries' with only isolated reports in the literature. The importance of neonatal diagnosis is twofold. Ten percent of these tumors may be malignant at the time of delivery.2 They may grow to a large size, affecting fetal outcome and labor management. The objectives of this study were to examine the prenatal diagnosis and the antepartum and intrapartum management with subsequent outcome.
Methods Cases of sacrococcygeal teratoma during a 5-year period (from 1980 through 1985) from four tertiary referral centers forming the University of Toronto Perinatal Complex were studied retrospectively. Six cases, including one set of twins, originated from the complex itself, while four were referred from outlying hospitals. Prenatal assessment, diagnosis, fetal weight and sex, size of tumor, mode of delivery, and outcome were documented. A diagnosis of sacrococcygeal teratoma required histologic confirmation. Results A total of IO cases were recorded, with one set of female twins and a female: male ratio of 9: l. In six of the cases, ultrasonographic diagnosis of sacrococcygeal
From the University of Toronto Perinatal Complex (Toronto General Hospital, Women's College Hospital, Mount Sinai Hospital, and The Hospital for Sick Children). Presented at the Forty-second Annual Meeting of The Society of Obstetricians and Gynaecologists of Canada, Charlottetown, Prince Edward Island, Canada, June 23-27, 1986. Reprint requests: Dr. R. J. Benzie, Ottawa General Hospital, 501 Smyth, Ottawa, Ontario, Canada KIH 8L6.
teratoma was made, a large-for-dates uterus being the indication for the ultrasound examination. No other phenotypic abnormalities were identified although there were three cases of polyhydramnios. Because of prenatal diagnosis of sacrococcygeal teratoma in both twins, one patient requested and obtained termination of pregnancy at 19 weeks (Figs. l and 2). The remaining four cases were diagnosed at the time of delivery. The maternal serum o:-fetoprotein and amniotic fluid o:-fetoprotein and acetylcholinesterase had not been tested. There was a 50% (four of eight) prematurity rate. The overall average gestational age was 35.5 ± 4 weeks. There was a 75% (six of eight) cesarean section rate. In the four cases where prenatal diagnosis was made by ultrasound, the presence of a large tumor (average volume 2340 ± 1026 cm 3 ) affected the mode of delivery. In none of these cases was the infant delivered immediately after the diagnosis was made. In three of them, serial ultrasound examinations were performed and the fetus was delivered abdominally because of an abnormal nonstress test, premature rupture of the membranes, and polyhydramnios, respectively. In the four cases without prenatal diagnosis, two infants were delivered vaginally. The average volume of these tumors was relatively small (average volume 317 ± 197 cm'). The remaining two infants were delivered by cesarean section for obstetric reasons (transverse lie and failure to progress, respectively, Table I). Significant morbidity was seen in one case. Because of the large size and vascularity of the tumor, the infant developed hypovolemic shock during the resection operation resulting in subsequent pre-renal failure. Fig. 3 illustrates the size and vascularity of the teratoma. The infant received appropriate management and was normal at follow-up 2 years later. 393
394
Gross et al.
February 1987 Am J Obstet Gynecol
Fig. I. Long-axis ultrasound of twin A, showing caudal unilocular cystic mass.
Fig. 2. Long-axis ultrasound of twin B, showing large caudal multilocular cystic mass.
There was one neonatal death secondary to prematurity and one death at 3 1/2 years of age secondary to malignant endodermal sinus tumor, apparently unrelated to the original lesion. The tumor was resected during the first week of life in surviving neonates and all the tumors were benign. No associated anomalies were found prenatally by ultrasound or at subsequent operation.
Comment Our series was consistent with some known features of sacrococcygeal teratoma. It is more common in female infants (75% to 80%) 2 and there is concordance
between twins. 3 As with many congenital malformations, sacrococcygeal teratoma is associated with polyhydramnios. 4 None of the tumors in our series, however, were malignant. No associated anomalies of the lower vertebrae, anorectum, or genitourinary system were documented. With respect to prenatal diagnoses, while none of the cases were tested for elevated amniotic fluid a-fetoprotein and acetylcholinesterase levels, these enzymes have been found to be elevated in sacrococcygeal teratoma, especially in the second trimester. 5 Although elevation of these enzymes would not likely have affected the diagnosis in these cases, further study in this area is
Sacrococcygeal teratoma 395
Volume 156 Number 2
Fig. 3. Female newborn infant with sacrococcygeal teratoma, 32 weeks' gestation, after cesarean section.
Table I. Summary of cases of sacrococcygeal teratoma Case No. I
2 3 4 5 6 7 8 9 JO
Birth weight Fetal sex
(gm)
F F (Twin A) F (Twin B) F F F F M F F
5000 2300 4600 2070 3960 5460 2910 3160
Gestational age at diagnosis (wk)
Gestational age at delivery (wk)
23 24 19 ·19 30 27 40 40 37 38
36 34 19 19 32 27 40 40 37 38
warranted. As seen in our series, where no ultrasonographic errors were made, careful ultrasonography can be a very accurate method of prenatal diagnosis and assessment. The important differential diagnosis of meningomyelocele and to a lesser extent hemangioma should not be forgotten. We therefore recommend that, while a rare entity, sacrococcygeal teratoma should be suspected in a largefor-dates patient, and ultrasound examination should be performed in order to make the diagnosis prenatally. These patients should be evaluated by serial ultrasonography to assess tumor size. Appropriate counseling at the time of diagnosis is essential, not only in order to discuss possible termination but also to adequately educate and prepare parents for the impending labor, delivery, and management. The literature documents different modes of management: (1) vaginal delivery,' (2) cesarean section, 1 (3) cesarean section after partial delivery because of dystocia,6 (4) vaginal drainage of tumor followed by vaginal
Prenatal diagnosis
Ultrasonographic Ultrasonographic Ultrasonographic Ultrasonographic Ultrasonographic Ultrasonographic
Tumor size at birth (cm)
Mode of delivery
27 by 15 by 8 14 by JO by 10 1.5 by 1.5 6 (diameter) 25 by I I by 13 5 by 5 by 5 IO by 7 by 7 17 by 15 by 17 5 (diameter) 6 by 4 by 5
Cesarean section Cesarean section Elective abortion Elective abortion Cesarean section Cesarean section Vaginal Cesarean section Cesarean section Vaginal
delivery,7 and (5) abdominal drainage of tumor followed by vaginal delivery. 8 While mode of delivery has been individualized in the literature, tumor size is critical in this decision for two reasons. In our two cases of vaginal delivery, the tumors were small enough for delivery; however, a large tumor can cause serious obstetric complications such as abnormal lie and dystocia. More importantly, as seen in our one case of hypotensive shock, a large sacrococcygeal teratoma can be highly vascular, thereby causing exsanguination or rupture on aspiration. The literature documents cases of neonatal death after rupture of sacrococcygeal teratoma during vaginal delivery." On the basis of the above literature review and our own series of cases, we feel it reasonable to suggest that, except for very small tumors (
Gross et al.
be obtained with good outcome anticipated in most cases. We would like to thank Dr. Yung and Dr. Claessens for providing cases for this study. REFERENCES 1. Salaymel MT. Giant sacrococcygeal teratoma in the newborn. Int Surg 1971 ;56:56. 2. Seeds JW, Mittelstaedt CA, Cefalo RC, Parker TF. Prenatal diagnosis of a sacrococcygeal teratoma-an anechoic caudal mass.JCU 1982;10:193. 3. Lees RF, Williamson BRJ, Brenbridge ANAG, Buschi AJ, Teja K. Sonography of benign sacral teratoma in utero. Radiology 1980;134:717. 4. Horger EO, McCarter LM. Prenatal diagnosis of sacrococcygeal teratoma. AM J 0BSTET GYNECOL 1979; r34:228.
February 1987 Am J Obstet Gynecol
5. Brock DJH, Richmond DH, Liston WA. Normal secondtrimester amniotic fluid alphafetoprotein and acetylcholinesterase associated with fetal sacrococcygeal teratoma. Prenat Diagn 1983;3:343. 6. Musci MC, Clark MJ, Ayres RE, Finkel MA. Management of dystocia caused by a large sacrococcygeal teratoma. Obstet Gynecol 1982;62: 105. 7. Weiss DB, Wajntraub G, Abulafia Y, Schiller M. Vaginal surgical intervention for a sacro-coccygeal teratoma obstructing labour. Acta Obstet Gynecol Scand 1976;55:183. 8. Tanaree P. Delivery obstructed by sacrococcygeal teratoma. AM j 0BSTET GYNECOL 1982; 142:239. 9. Guigiaro A, Boario U, Fraceso G, Freni G. Three cases of intrapartum rupture of sacrococcygeal teratoma [in Italian]. Minerva Pediatr 1977;29:1517.
Human chorionic gonadotropin, prolactin, estriol, and dehydroepiandrosterone sulfate concentrations in cord blood of premature and term newborn infants: Relationship to the sex of the neonate Basil Ho Yuen, M.B., Ch.B., and Everett K. Mincey, Ph.D. Vancouver, British Columbia, Canada Although the composition of human chorionic gonadotropin, prolactin, estriol, and dehydroepiandrosterone sulfate in the umbilical cord blood has been studied, less information is available on the effect of fetal sex on the cord blood concentrations. We assayed these hormones in cord blood from 405 newborn infants delivered between 23 and 43 weeks of gestation. In pooled data for both sexes, human chorionic gonadotropin showed a declining trend from the beginning to the end of the sampling interval. By contrast, in 398 newborn infants of known sex, prolactin, estriol, and dehydroepiandrosterone sulfate levels increased during the same period. Sex differences were observed for human chorionic gonadotropin only; female newborn infants had higher cord blood concentrations throughout the sampling period, with the differences becoming statistically significant between 29 and 36 weeks. (AM J OssrET GvNECOL 1987; . 156:396-400.)
Key words: Newborn infants, sex, chorionic gonadotropin, prolactin, estriol, dehydroepiandrosterone sulfate, cord blood Human chorionic gonadotropin, prolactin, estriol, and dehydroepiandrosterone sulfate are important
From the Gynaecologic Endocrine Laboratory, Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynaecology, Faculty of Medicine, The University of British Columbia, and Metropolitan Biomedical Laboratories. Presented at the Forty-second Annual Meeting of The Society of Obstetricians and Gynaecologists of Canada, Charlottetown, Prince Edward Island, Canada, June 23-27, 1986. Reprint requests: Dr. B. Ho Yuen, Department of Obstetrics and Gynaecology, The University of British Columbia, 4490 Oak St., Vancouver, British Columbia, Canada V6H 3V5.
396
polypeptide and steroid hormones produced in the fetal-placental unit. Although their composition in the umbilical cord blood has been studied, less information is available on the effect of fetal sex on the cord blood concentrations.'· 2 In the present study, we measured the levels of these hormones in the cord blood of newborn infants from mid pregnancy to term to determine whether there were any sex differences in these levels.
Material and methods At the time of delivery, mixed umbilical cord blood was collected into tubes containing ethylenediamine-