Cervical and oral teratoma in the fetus: A systematic review of etiology, pathology, diagnosis, treatment and prognosis

Article (PDF Available)inArchives of Gynecology 282(4):355-61 · October 2010with133 Reads
DOI: 10.1007/s00404-010-1500-7 · Source: PubMed
Abstract
The aim of the study was to produce a systematic review about etiology, pathology, diagnosis, prognosis and clinical management regarding oral and cervical teratomas. A systematic review of Pubmed/Medline using the following keywords was made: epignathus, cervical teratoma, fetus, oral teratoma, prenatal diagnosis, prognosis, treatment, ultrasound. The following clinical conclusions can be reached: (1) teratomas are rare, usually benign congenital tumors which recognized multifactorial etiology; (2) prenatal ultrasound diagnosis can be made early in pregnancy (15-16 weeks); (3) 3D ultrasound and MRI may enhance the accuracy of the antenatal diagnosis (location, extension and intracranial spread) and may aid in the selection of patients requiring treatment; (4) prenatal karyotype and search for associated abnormalities is mandatory in all teratomas; (5) delivery should involve elective Cesarean section with ex utero intrapartum treatment procedure or resection of the tumor mass, which may be performed on placental support operation on placental support procedure to increase the chances of postnatal survival.
This article was published in the above mentioned Springer issue.
The material, including all portions thereof, is protected by copyright;
all rights are held exclusively by Springer Science + Business Media.
The material is for personal use only;
commercial use is not permitted.
Unauthorized reproduction, transfer and/or use
may be a violation of criminal as well as civil law.
ISSN 0932-0067, Volume 282, Number 4
MATERNO-FETAL MEDICINE
Cervical and oral teratoma in the fetus: a systematic review
of etiology, pathology, diagnosis, treatment and prognosis
Gabriele Tonni
C. De Felice
G. Centini
C. Ginanneschi
Received: 27 January 2010 / Accepted: 26 April 2010 / Published online: 15 May 2010
Ó Springer-Verlag 2010
Abstract
Introduction The aim of the study was to produce a
systematic review about etiology, pathology, diagnosis,
prognosis and clinical management regarding oral and
cervical teratomas.
Materials and methods A systematic review of Pubmed/
Medline using the following keywords was made: epig-
nathus, cervical teratoma, fetus, oral teratoma, prenatal
diagnosis, prognosis, treatment, ultrasound.
Conclusion The following clinical conclusions can be
reached: (1) teratomas are rare, usually benign congenital
tumors which recognized multifactorial etiology; (2) pre-
natal ultrasound diagnosis can be made early in pregnancy
(15–16 weeks); (3) 3D ultrasound and MRI may enhance
the accuracy of the antenatal diagnosis (location, extension
and intracranial spread) and may aid in the selection of
patients requiring treatment; (4) prenatal karyotype and
search for associated abnormalities is mandatory in all
teratomas; (5) delivery should involve elective Cesarean
section with ex utero intrapartum treatment procedure or
resection of the tumor mass, which may be performed on
placental support operation on placental support procedure
to increase the chances of postnatal survival.
Keywords Cervical teratoma Epignathus
Ex utero intrapartum treatment procedure (EXIT)
Operation on placental support (OOPS)
Introduction
Incidence etiology
Cervical teratomas (Fig. 1) represent 3–5% of all terato-
mas, and their incidence may range from 1 in 20,000 to 1 in
40,000 [1] or from 1 in 35,000 to 1 in 200,000 live births
[2], with a female-to-male ratio of 3:1 [3]. In a series of
1,253 teratomas of different types [48], only 6 (0.47%)
were classified as epignathus [9].
Oral teratoma or epignathus (Figs. 2, 3) is a congenital
tumor occurring in the sphenoid region on the palate or
pharynx, which is also called Rathke’s pouch [10], and
may rarely be found in the nasal cavity of the hard palate.
Oral teratoma or epignathus may be due to abnormal
migration of primordial cells settling in the mediastinum or
hypothalamic regions. Teratomas or epignathi have multi-
factorial etiology, such as chromosomal abnormalities;
examples include trisomy 13 [11], ring X-chromosome
mosaicism with inactive ring X-chromosome [12, 13],
gonosomal pentasomy 49, XXXXY karyotype [14], gene
mutations (e.g. HLXB9) [15] or abnormalities in early
embryonic development [16]. Teratoma or epignathus may
G. Tonni (&)
Prenatal Diagnostic Service, Prenatal Diagnostic Unit,
Division of Obstetrics and Gynecology, Guastalla Civil Hospital,
AUSL Reggio Emilia, Via Donatori Sangue, 1,
42016 Guastalla (Reggio Emilia), Italy
e-mail: Tonni.Gabriele@ausl.re.it
C. De Felice
Neonatal Intensive Care Unit, Policlinic Hospital ‘Le Scotte’’,
University of Siena, Siena, Italy
G. Centini
Prenatal Diagnostic Unit,
Department of Obstetrics and Gynecology,
Policlinic Hospital ‘Le Scotte’’, University of Siena,
Siena, Italy
C. Ginanneschi
Human Pathology and Oncology, Pathologic Anatomy Section,
Policlinic Hospital ‘‘Le Scotte’’, University of Siena, Siena, Italy
123
Arch Gynecol Obstet (2010) 282:355–361
DOI 10.1007/s00404-010-1500-7
Author's personal copy