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Intracranial teratoma: Prolonged neonatal survival after prenatal diagnosis
Intracranial teratoma: Prolonged neonatal survival after prenatal diagnosis Lawrence A. Dolkart, MD, Robert). Balcom, MD, George Eisinger, MB, BS Elmira, New York The prenatal diagnosis of an intracranial teratoma has been generally associated with either fetal death or very brief postnatal infant survival. A case is reported in which such a neonate survived for a month and was discharged home, despite the in utero diagnosis of a massive tumor. (AM J OBSTET GVNECOL 1990;162:768-9.)
Key words: Teratoma, ultrasonography, prenatal diagnosis
The prenatal diagnosis of an intracranial teratoma by ultrasonography is a rare event. Once an in utero diagnosis is established, the prognosis is bleak. A stillborn fetus or survival of a neonate for a few minutes or hours is usually expected. We present a case of a massive tumor in which the infant had an unusually prolonged survival.
Case report A 22-year-old white, gravida 3, para 1 woman had an initial ultrasonographic examination at 20 weeks' gestation to confirm gestational age; no abnormalities were detected. Her prenatal course remained normal until 33 weeks' gestation at which time a fundic size greater than expected for dates was first noted. This prompted a level II ultrasonographic examination, which showed moderate polyhydramnios, an enlarged biparietal diameter (13 em) , significant hydrocephalus, and an echogenic 6 x 8 em solid mass with cystic components occupying the right lateral ventricle (Fig. 1). Agenesis of the corpus callosum was noted, but all other major intracranial contents could be identified despite some distortion as a result of ventriculomegaly. The findings were consistent with an intracranial teratoma. No other fetal anomalies were identified. Chromosome analysis after amniocentesis revealed a normal 46,XX karyotype. The parents were advised of the poor prognosis. Neonatologists and neurosurgeons were notified of the fetal lesion, and an elective cesarean section was performed through a vertical incision after pulmonary maturity was documented (by lecithin/sphingomyelin ratio) at 37 weeks' gestation. Birth weight was 4420 gm, and Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The cranium From the Departments of Pennatology. N eonatology, and Pathology, Arnot-Ogden Memonal HospItal. Received for publicatIOn September 11, 1989; accepted September 20,
1989.
Repnnts not available.
6/1 /16822
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Fig. 1. Prenatal ultrasonographic examination of fetal head showing teratomatous mass.
was massively enlarged (Fig. 2). The infant was remarkably stable throughout her hospitalization. Full oral feedings were established within 2 weeks of birth, and an appropriate weight gain was noted. A computerized tomography head scan confirmed the intracranial mass and a markedly thinned cortical mantle. On neurologic examination the baby displayed a normal cry, moved all extremities spontaneously, and displayed good tone. However, there was no response to visual or sound stimulation and chances for higher cortical function were believed to be remote. Multiple neurosurgical opinions were obtained, and the general consensus was that the tumor was inoperable. Ventricular shunt placement was judged not to be indicated. The infant was discharged home on the 32nd day after birth. Three days later the parents noted the sudden onset of shallow respirations and poor color, and 2 hours after readmission the child died of cardio-
Fetal cranial teratoma with prolonged infant survival
Volume 162 Number 3
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Fig. 2. Massive cranial enlargement in neonate.
Fig. 3. Gross specimen of teratoma.
respiratory arrest, without resuscitative efforts as requested by the parents. Necropsy showed advanced obstructive hydrocephalus and a teratomatous mass that weighed 760 gm and measured 23 x 12 x 12 cm (Fig. 3). The tumor had destroyed the midbrain area and was considered to have been of pineal origin. Microscopic examination revealed a variety of well-differentiated tissue patterns, and a medulloblastoma component that caused diffuse malignant invasion.
the cranial anatomy could be identified on ultrasonographic examination despite the large tumor size, and the neonatal course was surprisingly prolonged, with death occurring 35 days after birth. When these tumors are diagnosed prenatally, the parents must be informed of the grave prognosis. However, obstetricians and neonatologists should be prepared for the atypical situation of survival for weeks, and even hospital discharge. Unfortunately, death has uniformly been the final outcome in these cases.
Comment
Lipman et al. 1 could document only 11 cases of fetal intracranial teratoma diagnosed by prenatal ultrasonography (in 1985), and few subsequent cases have been reported. The tumor mass is typically huge and distorts the brain anatomy beyond recognition. Almost all neonates die within minutes or hours of birth, or are stillborn, although Paes et al! described an unusual survival of 4 days. Our case is striking in that much of
REFERENCES I, Lipman SH, Pretorius DH, Rumack eM, Manco-Johnson ML. Fetal intracranial teratoma : U S . . diagnosis of three cases and a review of the literature. Radiology 1985; 157:491-4. 2. Paes BA, Desa DJ, Hunter DJS, Pirani M. Benign intracranial teratoma-prenatal diagnosis influencing early delivery. AMJ OBSTET GYNECOL 1982; 143 :600- 1.
Key words: Teratoma, ultrasonography, prenatal diagnosis
The prenatal diagnosis of an intracranial teratoma by ultrasonography is a rare event. Once an in utero diagnosis is established, the prognosis is bleak. A stillborn fetus or survival of a neonate for a few minutes or hours is usually expected. We present a case of a massive tumor in which the infant had an unusually prolonged survival.
Case report A 22-year-old white, gravida 3, para 1 woman had an initial ultrasonographic examination at 20 weeks' gestation to confirm gestational age; no abnormalities were detected. Her prenatal course remained normal until 33 weeks' gestation at which time a fundic size greater than expected for dates was first noted. This prompted a level II ultrasonographic examination, which showed moderate polyhydramnios, an enlarged biparietal diameter (13 em) , significant hydrocephalus, and an echogenic 6 x 8 em solid mass with cystic components occupying the right lateral ventricle (Fig. 1). Agenesis of the corpus callosum was noted, but all other major intracranial contents could be identified despite some distortion as a result of ventriculomegaly. The findings were consistent with an intracranial teratoma. No other fetal anomalies were identified. Chromosome analysis after amniocentesis revealed a normal 46,XX karyotype. The parents were advised of the poor prognosis. Neonatologists and neurosurgeons were notified of the fetal lesion, and an elective cesarean section was performed through a vertical incision after pulmonary maturity was documented (by lecithin/sphingomyelin ratio) at 37 weeks' gestation. Birth weight was 4420 gm, and Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The cranium From the Departments of Pennatology. N eonatology, and Pathology, Arnot-Ogden Memonal HospItal. Received for publicatIOn September 11, 1989; accepted September 20,
1989.
Repnnts not available.
6/1 /16822
768
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Fig. 1. Prenatal ultrasonographic examination of fetal head showing teratomatous mass.
was massively enlarged (Fig. 2). The infant was remarkably stable throughout her hospitalization. Full oral feedings were established within 2 weeks of birth, and an appropriate weight gain was noted. A computerized tomography head scan confirmed the intracranial mass and a markedly thinned cortical mantle. On neurologic examination the baby displayed a normal cry, moved all extremities spontaneously, and displayed good tone. However, there was no response to visual or sound stimulation and chances for higher cortical function were believed to be remote. Multiple neurosurgical opinions were obtained, and the general consensus was that the tumor was inoperable. Ventricular shunt placement was judged not to be indicated. The infant was discharged home on the 32nd day after birth. Three days later the parents noted the sudden onset of shallow respirations and poor color, and 2 hours after readmission the child died of cardio-
Fetal cranial teratoma with prolonged infant survival
Volume 162 Number 3
..', , =
, ...
"
'
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•..,.-.",:, •• t~,':'A
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769
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'" ,.:
.'
'-
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-
'
Fig. 2. Massive cranial enlargement in neonate.
Fig. 3. Gross specimen of teratoma.
respiratory arrest, without resuscitative efforts as requested by the parents. Necropsy showed advanced obstructive hydrocephalus and a teratomatous mass that weighed 760 gm and measured 23 x 12 x 12 cm (Fig. 3). The tumor had destroyed the midbrain area and was considered to have been of pineal origin. Microscopic examination revealed a variety of well-differentiated tissue patterns, and a medulloblastoma component that caused diffuse malignant invasion.
the cranial anatomy could be identified on ultrasonographic examination despite the large tumor size, and the neonatal course was surprisingly prolonged, with death occurring 35 days after birth. When these tumors are diagnosed prenatally, the parents must be informed of the grave prognosis. However, obstetricians and neonatologists should be prepared for the atypical situation of survival for weeks, and even hospital discharge. Unfortunately, death has uniformly been the final outcome in these cases.
Comment
Lipman et al. 1 could document only 11 cases of fetal intracranial teratoma diagnosed by prenatal ultrasonography (in 1985), and few subsequent cases have been reported. The tumor mass is typically huge and distorts the brain anatomy beyond recognition. Almost all neonates die within minutes or hours of birth, or are stillborn, although Paes et al! described an unusual survival of 4 days. Our case is striking in that much of
REFERENCES I, Lipman SH, Pretorius DH, Rumack eM, Manco-Johnson ML. Fetal intracranial teratoma : U S . . diagnosis of three cases and a review of the literature. Radiology 1985; 157:491-4. 2. Paes BA, Desa DJ, Hunter DJS, Pirani M. Benign intracranial teratoma-prenatal diagnosis influencing early delivery. AMJ OBSTET GYNECOL 1982; 143 :600- 1.