Post on 19-Mar-2023
Journal of Pediatric Oncology, 2014, 2, 3-9 3
E-ISSN: 2309-3021/14 © 2014 Pharma Publisher
Congenital Posterior Mediastinal Teratoma with Intraspinal Extension
Mohammed Basamh1, Fahad A. Alghamdi2, Osama Rayes3 and Saleh S. Baeesa*,1
Division of Neurosurgery1, Department of Pathology
2, and Division of Pediatric Surgery
3, Faculty of Medicine,
King Abdulaziz University, Jeddah, Saudi Arabia
Abstract: Congenital teratoma is the commonest tumors of the nervous system, which are predominantly midline located. However, their spinal location is extremely rare. We present a case of a female twin newborn with huge right
side mediastinal tumor presented afterbirth with a chest infection. She underwent complete surgical resection via thoracotomy. Histopathological examination revealed immature teratoma. She had complete respiratory recovery but presented three months later with progressive paraparesis due to intrapinal tumor. She underwent thoracic laminectomy
and complete excision for what turned out histopathologically to be mature teratoma. She recovered well from surgery and received adjuvant chemotherapy over one year. Her 5-year follow up revealed a healthy child with no recurrence of the mediastinal or intraspinal teratoma on regular imaging surveillance.
We report herein a rare congenital posterior mediastinal teratoma with intraspinal extension and discuss the clinical
features, imaging studies, histopathological examination, and management.
Keywords: Congenital teratoma, Mediastinal teratoma, Spinal cord tumor, Spinal teratoma.
INTRODUCTION
Teratoma is a multipotential cell tumor consisting of
all three primitive germ cell components that further
classified, based on the degree of differentiation, into
mature, immature or malignant types based on the
degree of cellular differentiation [1]. They may originate
anywhere along the midline; however, teratoma has
predominance to sacrococcygeal, gonadal, medias-
tinal, retroperitoneal, cervicomandibular and intracra-
nial locations.
Mediastinal teratomas are commonly seen in
children and accounting 8% to 13% of all mediastinal
tumors [2] and Up to 75% of are mature type [3].
Intraspinal teratomas are extremely rare occurrence
accounting of 0.15% of adults and 4-10% of pediatric
spinal tumors (Table 1), and occur either as an
extradural extension from adjacent paraspinal origin
(Table 2) [4-7], or as an isolated intradural origin [8-14].
We aim to report a newborn presented with
congenital mediastinal immature teratoma with
extradural spinal extension of mature cystic teratoma
and discuss the management.
CASE REPORT
A newborn, monozygotic twin, girl presented since
birth with respiratory distress while feeding. She and a
healthy twin sister were a product of an uneventful
*Address correspondence to this author at the Division of Neurosurgery, Faculty of Medicine, King Abdulaziz University, P.O. Box 80215, Jeddah 21589, Kingdom of Saudi Arabia; Fax: +966-12-6408469; E-mail: sbaeesa@kau.edu.sa
pregnancy that was ended with cesarean section at the
37th
week. She was brought to a polyclinic 2 weeks
later with low-grade fever and cough. Her temperature
was 37.5oC and her physical exam revealed no
cyanosis, but with right lung basal crepitation and
decreased air entry. Her pulse Oxymetry revealed 90%
oxygen saturation and complete blood count (CBC)
was normal. Chest radiography demonstrated right
lower lung opacity, initially reported as pneumonia, and
she was discharged on oral antibiotics.
Table 1: Summary of Intraspinal Tumors Series Reported in the Literature
Author Number of
Cases Size of series
Percentage of cases
Sloof et al. 2 1,322 0.15%
Black and German
1 142 0.7%
Oi and Raimondi
2 64 3.1%
Matson 13 134 9.7%
Hendrick 3 80 3.75%
Kordas et al. 2 80 2.5%
DeSousa et al.
31 81 3.7%
Average 3.4%
Abbreviations: *Series of children,
1Authors reported 6 intraspinal teratoma
cases of 81 intraspinal tumors; 3 of 6 were originating from the sacral area that was not included.
She improved for a week but presented to our
emergency department with fever and progressive
respiratory distress. General physical examination
revealed a temperature of 38.5oC, tachypnea (34
4 Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 Basamh et al.
B/min) and tachycardia (96 B/min), with no central
cyanosis, and she had marked decreased air entry in
the right lung. The neurological exam was within
normal.
She was admitted to pediatric intensive care unit,
intubated, and started on IV antibiotics and bronchio-
lytic medications. Routine laboratory investigations
including; CBC, coagulation, renal, hepatic profiles
Table 2: Reported Cases in the Literature of Posterior Mediastinal Teratoma with Spinal Extension
Year Author Age Sex Symptoms Level Pathology Treatment Result
1961 Howard [8] 4y M Paraplegia with normal
sensations Thoracic Teratocarcinoma
Palliative decompression
Died 49 d
1981 Kolandaivelu
[9] 3.5y M
Paraplegia sensory level at T9
Thoracic, epidural
Benign teratoma GTR GR
1998 Kondo [10] 42y M Left Brown-Séquard
syndrome Thoracic, epidural
Adenocarcinoma in teratoma
GTR MR
2004 Schneider [11] NA NA NA Thoracic
Teratoma with malignant
transformation, recurred as epidural
benign teratoma
NEC + GTR, then 2
nd
resection NED
Abbreviations: NA=not available, d=days, GR= good (neurological) recovery, GTR= gross total resection, M= male, MR= minimal (neurological) recovery, NEC= neo-adjuvant chemotherapy, NED= no evidence of disease, y=years.
Figure 1: Coronal (A), Sagittal (B), and axial (C) CT scans demonstrating large tumor occupying the right hemi-thorax with
marked mass effect and shift of the right lung and mediastinal contents.
Congenital Posterior Mediastinal Teratoma with Intraspinal Extension Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 5
were within normal. Computed tomography (CT) scan
revealed a large right hemi-thorax heterogeneous mass
occupying the posterior mediastinum that measures 6.5
x 5.5 cm with areas of cystic and solid components
(Figure 1).
Serum tumor markers including serum alpha-
fetoprotein (AFP) and beta-human chorionic gonado-
tropin (B-HCG) levels were unremarkable.
The patient’s general and respiratory condition was
optimized, and then she was taken to the operating
room 48 hours later where surgery was performed
through right-side thoracotomy by pediatric surgery
team. A large posterior mediastinal capsulated reddish
brown tumor was encountered, which was adherent,
but not infiltrated, to pericardium and aorta; there was
no spinal extension. The patient tolerated the surgery
well, extubated after two days and had a smooth
complete recovery of her respiratory dysfunction and
discharged home after two weeks with a postoperative
CT scan showing no residual tumor. Histopathological
examination of the mediastinal specimen revealed
immature teratoma composed of neuroblastic tissue
with mature elements of mesodermal and ectodermal
origin (Figure 2).
Figure 2: Histopathology of the mediastinal specimen
demonstrating immature teratoma contain an immuture
neublastic tissue (A), and mature elements like respiratory
ciliated columnar epithelium and colonic glands (B).
The patient was followed up in pediatric surgery and
oncology clinics where adjuvant chemotherapy was
recommended.
During this time, reached her 4-month of age, she
was brought to the emergency department with rapidly
progressing decreased movement of her lower
extremities for 2 weeks. Her neurological examination
revealed normal mental function for age and cranial
nerves. She moved her upper extremities normally but
had flaccid paraparesis of motor grade 2 out of 5 and
absent reflexes. Urgent magnetic resonance imaging
(MRI) scan of the spine demonstrated significant cord
compression by a heterogeneous thoracic intraspinal
cystic mass extending from fourth to seventh thoracic
vertebral level (T4 to T7) that measured 3.2 x 1.4 cm,
and associated with paraspinal and ribs extension
through more than one foramina (Figure 3). MRI scan
of the brain and CT scan of the chest and abdomen
was within normal.
The patient was started on IV steroids and taken to
the operating room where thoracic laminectomy, from
T4 to T8, was performed. The tumor was located in the
epidural space extending to 2 intervertebral foraminae
but without extraforaminal extension. It was relatively
vascular encapsulated reddish-brown tumor, which was
easily dissected from the dura and completely removed
using microsurgical ultrasonic aspiration.
Histopathological examination of the spinal
specimen revealed a mature cystic teratoma composed
of three germ lines. Variable cysts were lined by
ciliated columnar epithelium, mature cartilage, vessels,
fat, stratified squamous epithelium and glial tissue
(Figure 4).
The patient had smooth postoperative recovery and
discharged in a good condition and showed
progressive neurological improvement and normal
recovery achieved after one month.
She received six cycles adjuvant chemotherapy
using modified JEB protocol (Carboplatin, Etoposide,
and Bleomycin), which were well tolerated and followed
up regularly in the clinic.
At her fifth anniversary, she remained with normal
neurological examination and development, and her
MRI scan of the spine (Figure 5) and CT scan of the
chest (Figure 6) revealed no recurrence. Worth to
mention here is that her twin has been evaluated with
head to spine MRI scans that revealed no similar or
other tumors.
6 Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 Basamh et al.
Figure 3: Sagittal (A) and axial (B) T2-WI MRI of the spine demonstrating intraspinal heterogeneous mass with marked spinal
cord compression.
Figure 4: Histopathology of the intraspinal specimen demonstrating mature teratoma contains respiratory ciliated columnar
epithelium (A), mature cartilage (B), stratified squamous epithelium (C), and adipose tissue (D).
Congenital Posterior Mediastinal Teratoma with Intraspinal Extension Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 7
Figure 5: 5-year follow up sagittal (A) and axial (B) T1-WI MRI scan demonstrating no recurrent tumor.
Figure 6: 5-year follow up axial CT (A) and axial MRI (B) scans demonstrating normal restoration of lung and mediastinal
structures and no recurrent tumor.
DISCUSSION
Teratoma is most common germ cell tumors in the
pediatrics age group [15]. The anterior mediastinum is
the commonest extragonadal site for these tumors
while it occurs in the posterior mediastinum only in 3-
8% of all mediastinal teratomas [16,17]. The posterior
mediastinal tumor may bulge unilaterally or bilaterally
from the usual midline contours to involve the spine [5].
They are also common in the sacrococcygeal spinal
region but otherwise very rare in the rest of spine [18].
Spinal teratomas are rarely occurring tumors, excluding
the relatively common sacrococcygeal form, constitute
less than 10% of all pediatrics intraspinal tumors.
Within the spinal canal, teratomas are most commonly
intradural [19] and,
particularly in children, are
associated with spina bifida [20].
Kondo et al., presented a case of 42-year-old man
who had a thoracic extradural immature teratoma [6]. It
developed over a period of more than 10 years after an
operation for a posterior mediastinal immature
teratoma. Another reported case by Schneider et al, of
an adult patient presented with epidural mature
teratoma occurring after chemotherapy and excision of
8 Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 Basamh et al.
posterior mediastinal teratoma with malignant
transformation [7]. These two cases have in common
the fact of late occurrence of the epidural tumors
following the resection of posterior mediastinal
teratomas. Furthermore, the latency for the epidural
tumor to appear clinically after their excision of the
mediastinal mass was 27 weeks and 10 years. Our
patient presented after 12 weeks of resection of the
posterior mediastinal teratoma.
CT scan is the modality of choice to detect
mediastinal teratomas in general [16], as it reveals the
extent of the heterogeneous mass containing a mixture
of soft tissues, fat, calcifications and/or teeth. However,
in cases of intraspinal teratomas, as in other spinal
tumors, magnetic resonance imaging (MRI) scan plays
a more important role because it delineate better the
tumor and surrounding neural anatomy. The
morphological appearances of intraspinal teratomas in
MRI scans are commonly oval or lobulated
heterogeneous masses, whereas extradural teratomas
were more commonly found to be dumbbell-shaped
and are commonly accompanied with vertebral body
malformation.
Tumor markers, including serum -human chorionic
gonadotropin ( -hCG) and -fetoprotien (AFP), were
applied for the diagnosis and prognosis of recurrences
of teratomas in general; however, this application was
limited in the mature teratomas as the recurrence may
have originated from non-secreting parts of the
previous lesion [7,21]. However, the diagnostic efficacy
of AFP and its role in predicting survival or altering
outcomes remains in question as we yet lacking
adequate data in this regard. Moreover, AFP since birth
is normally secreted, and its level declines gradually
and eventually plateaus between the 9th
-12th
months
[21].
An extensive sectioning and thorough histopatho-
logical examination is mandatory to avoid missing
immature or malignant tissues and preventing
misdiagnosis. This is because these immature and
malignant tissues are found in close relation to the
mature components as demonstrated in our case
[18,22].
The primary treatment for teratomas is complete
surgical excision, which may also be applied to mature
teratomas. The management of immature teratoma
remains an issue of some controversy due to its rarity,
potential missed malignant component within larger
lesions and the fact that adult patients are included in
some series in spite of their different disease course
[23]. Surgical resection of immature teratoma with
close follow-up and reserving platinum-based
chemotherapy for recurrence resulted in 98.6% 3-year
tumor free survival in a small study and seems,
therefore, a safe approach. The current standard
therapy for extragonadal nonseminatious germ cell
malignancies includes platinum-based chemotherapy
followed by resection of residual tumor [7]. The JEB
protocol was found to be more effective and less toxic
than other protocols [24]. Immature mediastinal
teratomas were observed to behave more aggressively
in adolescents and younger adults, while they are of a
benign course during infancy [25]. The combined
review of the Pediatric Oncology Group (POG) and
Children’s Cancer Study Group (CCG) reported the
grade of immaturity as the only valid risk factor for
recurrence in pediatrics immature teratoma regardless
of the site or age group [26]. This was due the strong
correlation between higher degrees of immaturity and
microscopic foci of malignant endodermal sinus
tumors.
CONCLUSION
Spinal teratomas, excluding the sacrococcygeal
location, are relatively rare. The epidural location is
even less common, with the posterior mediastinum
being an extremely rare origin. We recommend to role
out intraspinal involvement in any case with posterior
mediastinal tumor with MRI scans. Complete resection
and thorough histopathological examination are key
elements in the management of these patients. The
role of chemotherapy in immature spinal teratoma
should be further investigated.
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Received on 22-02-2014 Accepted on 24-03-2014 Published on 30-06-2014
DOI: http://dx.doi.org/10.14205/2309-3021.2014.02.01.1
© 2014 Basamh et al.; Licensee Pharma Publisher. This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.