Fetal Brain Tumors: A Review of 154 Cases

ABSTRACT

The purpose of this literature review is to describe the various types of brain tumors that occur in the fetus, their locations, initial findings, pathology, and outcome. Data are presented that show which patients are likely to survive or benefit from treatment compared with those who are unlikely to respond. An analysis is performed on patients with fetal brain tumors that were detected prenatally by imaging studies or discovered directly after birth. The review revealed 154 fetuses presenting with brain tumors. Teratoma was the leading neoplasm. Next was astrocytoma followed by craniopharyngioma, primitive neuroectodermal tumor, choroid plexus papilloma, meningeal tumors, and ependymoma. The most common presenting findings were macrocephaly and an intracranial mass. Overall hydrocephalus was next, followed by stillbirth. Most tumors were detected during the third trimester of pregnancy. Over a third of the affected fetuses were stillborn. Many tumors were inoperable as they occupied much of the intracranial cavity and involved large areas of the brain. Overall survival rate was 23/154, or 15%. Generally fetal brain tumors are associated with a poor prognosis.

Fetal brain tumors - congenital brain tumors - intrauterine death

REFERENCES

• 1 Buetow P C, Smirniotopoulos J G, Done S. Congenital brain tumors: a review of 45 cases.  AJR Am J Roentgenol. 1990;  155 587-593
• 2 Rickert C H. Neuropathology and prognosis of foetal brain tumours.  Acta Neuropathol. 1999;  98 567-576
• 3 Cavalheiro S, Moron A F, Hisaba W et al.. Fetal hydrocephalus—prenatal treatment.  Childs Nerv Syst. 2003;  19 561-573
• 4 Carstensen H, Juhler M, Bøgeskov L et al.. A report of nine newborns with congenital brain tumours.  Childs Nerv Syst. 2006;  22 1427-1431
• 5 Cassart M, Bosson N, Garel C et al.. Fetal intracranial tumors: a review of 27 cases.  Eur Radiol. 2008;  18 2060-2066
• 6 Sherer D M, Woods Jr J R, Abramowicz J S et al.. Prenatal sonographic assessment of early, rapidly growing fetal cervical teratoma.  Prenat Diagn. 1993;  13 1079-1084
• 7 Schlembach D, Bornemann A, Rupprecht T et al.. Fetal intracranial tumors detected by ultrasound: a report of two cases and review of the literature.  Ultrasound Obstet Gynecol. 1999;  14 407-418
• 8 Isaacs Jr H. I. Perinatal brain tumors: a review of 250 cases.  Pediatr Neurol. 2002;  27 249-261
• 9 Isaacs Jr H. II. Perinatal brain tumors: a review of 250 cases.  Pediatr Neurol. 2002;  27 333-342
• 10 Wienk M A, van Geijn H P, Copray F JA et al.. Prenatal diagnosis of fetal tumors by ultrasonography.  Obstet Gynecol Surv. 1990;  45 639-653
• 11 Jänisch W, Schreiber D, Martin H, Gerlach H. Primary intracranial tumors as cause of death in the fetus and infant.  Zentralbl Allg Pathol. 1984;  129 75-89
• 12 Jellinger K, Sunder-Plassmann M. Connatal intracranial tumours.  Neuropadiatrie. 1973;  4 46-63
• 13 Volpe J J. Brain tumors and vein malformations. In: Volpe JJ Neurology of the Newborn, 3rd ed. Philadelphia; WB Saunders Co 1995: 795-807
• 14 Isaacs Jr H. Brain tumors. In: Isaacs H Jr Tumors of the Fetus and Newborn. Vol. 35 in the Series Major Problems in Pathology. Philadelphia; W.B. Saunders 1997: 187-228
• 15 Storr U, Rupprecht T, Bornemann A et al.. Congenital intracerebral teratoma: a rare differential diagnosis in newborn hydrocephalus.  Pediatr Radiol. 1997;  27 262-264
• 16 Bare J B, Abramowsky C R, Hayes L L, Shehata B M. Congenital immature teratoma of the central nervous system: three case reports with literature review.  Fetal Pediatr Pathol. 2007;  26 109-118
• 17 Bolat F, Kayaselcuk F, Tarim E et al.. Congenital intracranial teratoma with massive macrocephaly and skull rupture.  Fetal Diagn Ther. 2008;  23 1-4
• 18 Alagappan A, Shattuck K E, Rowe T, Hawkins H. Massive intracranial immature teratoma with extracranial extension into oral cavity, nose, and neck.  Fetal Diagn Ther. 1998;  13 321-324
• 19 Vinters H V, Murphy J, Wittmann B et al.. Intracranial teratoma: antenatal diagnosis at 31 weeks' gestation by ultrasound.  Acta Neuropathol. 1982;  58 233-236
• 20 Paes B A, deSa D J, Hunter D J et al.. Benign intracranial teratoma—prenatal diagnosis influencing early delivery.  Am J Obstet Gynecol. 1982;  143 600-601
• 21 Oi S, Tamaki N, Kondo T et al.. Massive congenital intracranial teratoma diagnosed in utero.  Childs Nerv Syst. 1990;  6 459-461
• 22 Nanda A, Schut L, Sutton L N. Congenital forms of intracranial teratoma.  Childs Nerv Syst. 1991;  7 112-114
• 23 Rostad S, Kleinschmidt-DeMasters B K. Two massive congenital intracranial immature teratomas with neck extension.  Teratology. 1985;  32 163-169
• 24 Washburne J F, Magann E F, Chauhan S P, Fratkin J D, Morrison J C. Massive congenital intracranial teratoma with skull rupture at delivery.  Am J Obstet Gynecol. 1995l;  173 226-228
• 25 DiGiovanni L M, Sheikh Z. Prenatal diagnosis, clinical significance and management of fetal intracranial teratoma: a case report and literature review.  Am J Perinatol. 1994;  11 420-422
• 26 Horton D, Pilling D W. Early antenatal ultrasound diagnosis of fetal intracranial teratoma.  Br J Radiol. 1997;  70 1299-1301
• 27 Odell J M, Allen J K, Badura R J, Weinberger E. Massive congenital intracranial teratoma: a report of two cases.  Pediatr Pathol. 1987;  7 333-340
• 28 Wakai S, Arai T, Nagai M. Congenital brain tumors.  Surg Neurol. 1984;  21 597-609
• 29 Burger P C, Scheithauer B W. Tumors of the central nervous system. Atlas of Tumor Pathology, Third series, Fasicle 10. Washington, D.C.; Armed Forces Institute of Pathology 1994
• 30 Bigner DB, McLendon RE, Bruner JM Russell and Rubinsteins' Pathology of Tumors of the Nervous System. 6th ed., Vols. I and II. London; Arnold 1998
• 31 Kleihues P, Cavenee W K. Pathology and Genetics of Tumors of the Nervous System. Lyon; International Agency for Research on Cancer, WHO 1997
• 32 McConachie N S, Twining P, Lamb M P. Case report: antenatal diagnosis of congenital glioblastoma.  Clin Radiol. 1991;  44 121-122
• 33 Dören M, Tercanli S, Gullotta F, Holzgreve W. Prenatal diagnosis of a highly undifferentiated brain tumor—a case report and review of the literature.  Prenat Diagn. 1997;  17 967-971
• 34 Leins A M, Kainer F, Weis S. Sonography and neuropathology of a congenital brain tumor: report of a rare incident.  Ultrasound Obstet Gynecol. 2001;  17 245-247
• 35 Thankamony A, Harlow F H, Ponnampalam J et al.. Congenital brain tumour mimicking fetal intracranial haemorrhage.  J Obstet Gynaecol. 2007;  27 314-317
• 36 Sabet L M. Congenital glioblastoma multiforme associated with congestive heart failure.  Arch Pathol Lab Med. 1982;  106 31-34
• 37 Heckel S, Favre R, Gasser B et al.. Prenatal diagnosis of a congenital astrocytoma: a case report and literature review.  Ultrasound Obstet Gynecol. 1995;  5 63-66
• 38 Winters J L, Wilson D, Davis D G. Congenital glioblastoma multiforme: a report of three cases and a review of the literature.  J Neurol Sci. 2001;  188 13-19
• 39 Mirkin L D, Ey E H, Chaparro M. Congenital subependymal giant-cell astrocytoma: case report with prenatal ultrasonogram.  Pediatr Radiol. 1999;  29 776-780
• 40 Isaacs Jr H. Brain tumors. In: Isaacs H Jr Tumors of the Fetus and Infant: An Atlas. New York; Springer-Verlag 2002: 199-240
• 41 Anteby S O, Cohen H, Sadovsky E. Dystocia caused by fetal intracranial tumor.  Obstet Gynecol. 1974;  43 50-53
• 42 Riboni G, De Simoni M, Leopardi O, Molla R. Ultrasound appearance of a glioblastoma in a 33-week fetus in utero.  J Clin Ultrasound. 1985;  13 345-346
• 43 Sylvestre G, Sherer D M. Prenatal sonographic findings associated with malignant astrocytoma following normal early third-trimester ultrasonography.  Am J Perinatol. 1998;  15 581-584
• 44 Lee D Y, Kim Y M, Yoo S J et al.. Congenital glioblastoma diagnosed by fetal sonography.  Childs Nerv Syst. 1999;  15 197-201
• 45 Geraghty A V, Knott P D, Hanna H M. Prenatal diagnosis of fetal glioblastoma multiforme.  Prenat Diagn. 1989;  9 613-616
• 46 Roosen N, Deckert M D, Nikola N et al.. Congenital anaplastic astrocytoma with favorable prognosis: case report.  J Neurosurg. 1988;  69 604-609
• 47 Kamitomo M, Sameshima H, Uetsuhara K et al.. Fetal glioblastoma: rapid growth during the third trimester.  Fetal Diagn Ther. 1998;  13 339-342
• 48 Weber F, Mori Y. Congenital craniopharyngioma.  Helv Paediatr Acta. 1976;  31 261-270
• 49 Jänisch W, Flegelo H G. Craniopharyngioma in a fetus.  Zentralbl Allg Pathol. 1989;  135 65-69
• 50 Palo P, Penttinen M, Kalimo H. Early ultrasound diagnosis of fetal intracranial tumors.  J Clin Ultrasound. 1994;  22 447-450
• 51 Gass H H. Large calcified craniopharyngioma and bilateral subdural hematomata present at birth; survey of neonatal brain tumors.  J Neurosurg. 1956;  13 514-519
• 52 Tabaddor K, Shulman K, Dal Canto M C. Neonatal craniopharyngioma.  Am J Dis Child. 1974;  128 381-383
• 53 Snyder J R, Lustig-Gillman I, Milio L et al.. Antenatal ultrasound diagnosis of an intracranial (craniopharyngioma).  J Clin Ultrasound. 1986;  14 304-306
• 54 Yamada H, Haratake J, Narasaki T et al.. Embryonal craniopharyngioma. Case report of the morphogenesis of a craniopharyngioma.  Cancer. 1995;  75 2971-2977
• 55 Arai T, Ohno K, Takada Y et al.. Neonatal craniopharyngioma and inference of tumor inception time: case report and review of the literature.  Surg Neurol. 2003;  60 254-259
• 56 Lonjon M, Dran G, Casagrande F et al.. Prenatal diagnosis of a craniopharyngioma: a new case with radical surgery and review.  Childs Nerv Syst. 2005;  21 177-180
• 57 Bailey W, Freidenberg G R, James H E et al.. Prenatal diagnosis of a craniopharyngioma using ultrasonography and magnetic resonance imaging.  Prenat Diagn. 1990;  10 623-629
• 58 Hurst R W, McIlhenny J, Park T S et al.. Neonatal craniopharyngioma: CT and ultrasonographic features.  J Comput Assist Tomogr. 1988;  12 858-861
• 59 Müller-Scholden J, Lehrnbecher T, Müller H L et al.. Radical surgery in a neonate with craniopharyngioma. report of a case.  Pediatr Neurosurg. 2000;  33 265-269
• 60 Bonnin J M, Rubenstein L J, Palmer N F et al.. The association of embryonal tumors originating in the kidney and in the brain: a report of seven cases.  Cancer. 1984;  54 2137-2146
• 61 Becker L E, Hinton D. Primitive neuroectodermal tumors of the central nervous system.  Hum Pathol. 1983;  14 538-550
• 62 Dehner L P. Peripheral and central primitive neuroectodermal tumors. A nosologic concept seeking a consensus.  Arch Pathol Lab Med. 1986;  110 997-1005
• 63 Rorke L B. The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors.  J Neuropathol Exp Neurol. 1983;  42 1-15
• 64 Vandenberg S R, Herman M M, Rubinstein L J. Embryonal central neuroepithelial tumors: current concepts and future challenges.  Cancer Metastasis Rev. 1987;  5 343-365
• 65 Girschick H J, Klein R, Scheurlen W G, Kühl J. Cytogenetic and histopathologic studies of congenital supratentorial primitive neuroectodermal tumors: a case report.  Pathol Oncol Res. 2001;  7 67-71
• 66 Inwald D, Kempley S, Hird M. Congenital primitive neuroectodermal tumour presenting as obstructed labour.  Arch Dis Child Fetal Neonatal Ed. 1998;  78 F222-F224
• 67 Ehret M, Jacobi G, Hey A et al.. Embryonal brain neoplasms in the neonatal period and early infancy.  Clin Neuropathol. 1987;  6 218-223
• 68 McComb J G, Davis R L, Isaacs Jr H. Extraneural metastatic medulloblastoma during childhood.  Neurosurgery. 1981;  9 548-551
• 69 Mitchell C S, Wood B P, Shimada H. Neonatal disseminated primitive neuroectodermal tumor.  AJR Am J Roentgenol. 1994;  162 1160
• 70 Suresh S, Indrani S, Vijayalakshmi S et al.. Prenatal diagnosis of cerebral neuroblastoma by fetal brain biopsy.  J Ultrasound Med. 1993;  12 303-306
• 71 Mitchell D, Rojiani A M, Richards D et al.. Congenital CNS primitive neuroectodermal tumor: case report and review of the literature.  Pediatr Pathol Lab Med. 1995;  15 949-956
• 72 Shah N C, Ray A, Bartels U et al.. Diffuse intrinsic brainstem tumors in neonates. Report of two cases.  J Neurosurg Pediatrics. 2008;  1 382-385
• 73 Müller T, Girschick G, Mark K et al.. Fetal intracranial tumors detected by ultrasound.  Ultrasound Obstet Gynecol. 2001;  18 290-291
• 74 Taboada D, Froufe A, Alonso A et al.. Congenital medulloblastoma. Report of two cases.  Pediatr Radiol. 1980;  9 5-10
• 75 Mondkar J, Kalgutkar A, Nalavade Y et al.. Congenital primary cerebral neuroblastoma.  Indian Pediatr. 1994;  31 698-702
• 76 Asai M, Kitamura H, Yanagibashi T et al.. Case of acrania associated with congenital medulloblastoma.  Eur J Obstet Gynecol Reprod Biol. 1998;  81 115-117
• 77 Eberhart C G, Brat D J, Cohen K J et al.. Pediatric neuroblastic brain tumors containing abundant neuropil and true rosettes.  Pediatr Dev Pathol. 2000;  3 346-352
• 78 Molina C P, Hawkins H, Campbell G et al.. Case of the month: January 1999—Fetus with echogenic mass in third ventricle.  Brain Pathol. 1999;  9 605-606
• 79 Yamada T, Takeuchi K, Masuda Y et al.. Prenatal imaging of congenital cerebral primitive neuroectodermal tumor.  Fetal Diagn Ther. 2003;  18 137-139
• 80 Lippa C, Abroms I F, Davidson R et al.. Congenital choroid plexus papilloma of the fourth ventricle.  J Child Neurol. 1989;  4 127-130
• 81 Gertz H J, Unger M, Lobeck H et al.. Histological and immunocytochemical findings in a case of fetal choroid plexus papilloma.  Zentralbl Allg Pathol. 1990;  136 719-722
• 82 Adra A M, Mejides A A, Salman F A et al.. Prenatal sonographic diagnosis of a third ventricle choroid plexus papilloma.  Prenat Diagn. 1994;  14 865-867
• 83 Welch K, Strand R, Bresnan M et al.. Congenital hydrocephalus due to villous hypertrophy of the telencephalic choroid plexuses. Case report.  J Neurosurg. 1983;  59 172-175
• 84 Romano F, Bratta F G, Caruso G et al.. Prenatal diagnosis of choroid plexus papillomas of lateral ventricle. A report of two cases.  Prenat Diagn. 1996;  16 567-571
• 85 Asai A, Hoffman H J, Hendrick E B et al.. Primary intracranial neoplasms in the first year of life.  Childs Nerv Syst. 1989;  5 230-233
• 86 Nakamura Y, Becker L E. Meningeal tumors of infancy and childhood.  Pediatr Pathol. 1985;  3 341-358
• 87 Solitare G B, Krigman M R. Congenital intracranial neoplasm. A case report and review of the literature.  J Neuropathol Exp Neurol. 1964;  23 280-292
• 88 van Vliet M AT, Bravenboer B, Kock H CLV, Teepen J L. Congenital meningeal sarcoma—a case report.  J Perinat Med. 1983;  11 249-254
• 89 Witzke R, Hori A, Rahlf G. Meningiomas in a male fetus from a hormonally dysequilibrated mother.  Childs Nerv Syst. 1985;  1 359-362
• 90 Madsen C, Schrøder H D. Congenital intracranial meningioma. A case report and literature review.  APMIS. 1993;  101 923-925
• 91 Drut R, Sapia S, Gril D et al.. Nonimmune hydrops fetalis, hydramnios, microcephaly, and intracranial meningeal hemangioendothelioma.  Pediatr Pathol. 1993;  13 9-13
• 92 Kudo H, Oi S, Tamaki N et al.. Ependymoma diagnosed in the first year of life in Japan in collaboration with the International Society for Pediatric Neurosurgery.  Childs Nerv Syst. 1990;  6 375-378
• 93 Comi A M, Backstrom J W, Burger P C et al.. Clinical and neuroradiologic findings in infants with intracranial ependymomas. Pediatric Oncology Group.  Pediatr Neurol. 1998;  18 23-29
• 94 Rubinstein L J. The definition of the ependymoblastoma.  Arch Pathol. 1970;  90 35-45
• 95 Lorentzen M, Hägerstrand I. Congenital ependymoblastoma.  Acta Neuropathol. 1980;  49 71-74
• 96 Vinchon M, Soto-Ares G, Riffaud L et al.. Supratentorial ependymoma in children.  Pediatr Neurosurg. 2001;  34 77-87
• 97 Heye N, Iglesias J, Huber S, Lobeck H. Congenital ependymoma. Case report and immunohistochemical studies.  Zentralbl Allg Pathol. 1989;  135 43-49
• 98 Rickert C H, Göcke H, Paulus W. Fetal ependymoma associated with Down's syndrome.  Acta Neuropathol. 2002;  103 78-81
• 99 Mazewski C M, Hudgins R J, Reisner A et al.. Neonatal brain tumors: a review.  Semin Perinatol. 1999;  23 286-298
• 100 Jooma R, Kendall B, Hayward R. Intracranial tumors in neonates: a report of seventeen cases.  Surg Neurol. 1984;  21 165-170
• 101 Raisanen J M, Davis R L. Congenital brain tumors.  Pathology (Phila). 1993;  2 103-116