2014, Volume 30, Number 3, Page(s) 220-224
Congenital Mature Cystic Teratoma of the Lateral Neck Presenting as Cystic Hygroma: A Rare Case Report with Literature Review
Thin Thin WIN1, Nur Hidayati Mohamad FAKARUL RAZY1, Suzina Sheikh AB HAMID2, Anusha BALAS UBRAMANIAN2, Ganesh RAMALINGGAM2
1Department of Pathology, Sains Malaysia University, School of Medical Sciences, KELANTAN, MALAYSIA
2Department of Otorhinolaryngology - Head and Neck Surgery, Sains Malaysia University, School of Medical Sciences, KELANTAN, MALAYSIA
Keywords: Teratoma, Immature, Cystic lymphangioma, Head and neck neoplasms
Teratoma of the neck is a rare extra-gonadal teratoma. Although it
can be seen in adult, most of the cases present in fetal life or early
childhood. Grading and classification of teratoma is important for
management and prognosis. Decision making between mature and
immature teratoma is sometime challenging if it is not composed of
extensive unusual neuroectodermal tissue component. We reported
a rare case of huge congenital mature cystic teratoma composted
of various neuroectodermal tissues in an 8-month-old boy; it was
located at the lateral neck and mimicked congenital cystic hygroma.
A literature review was done to grade and classify the tumour.
Teratomas are germ cell tumours that originate in
totipotential cells and are composed of tissues arising
from more than one germ cell layers1
. They are most
commonly located in the sacrococcygeal region, followed by
the ovaries, testis, anterior mediastinum, retroperitoneum,
and finally the head and neck, which account for less than
Teratoma of the neck is uncommon location. Most of them
present as midline involvement and they have relationship
with thyroid gland4. Many congenital cervical teratoma
can be mistaken as cystic hygroma4. We report a rare
case of huge congenital mature cystic teratoma in an
8-month-old baby boy; it was located at the lateral neck
and mimicked congenital cystic hygroma. Due to some
uncommon histological findings, we reviewed the literature
to classify and grade the tumour.
An 8-month-old boy was admitted to head and neck surgical
unit with huge cystic left lateral neck mass presented since
birth. The mass was clinically diagnosed as cystic hygroma.
The cystic mass was noticed since antenatal period and the
baby was delivered via elective caesarean section. The mass
was very huge and bigger than the baby's head. However,
there were no breathing difficulties and the baby was able
to suck well without chocking. Weight gain was normal.
Imaging findings also suggested cystic hygroma and it was
treated with sclerotherapy 3 times. However, the cystic mass
failed to reduce in size. At the age of 8 months, surgical
excision was planned after Magnetic Resonance Imaging
(MRI) scan was performed. MRI showed mixed intensity
with mainly fluid component (Figure 1
huge cystic mass was seen at the left neck region extending
from the left ear lobe to the left supraclavicular region inferiorly and cervical vertebra posteriorly. It also extended
intra-orally and caused thinning of buccal mucosa. It
measured 14x18 cm in diameter. After aspiration of 5.4 liters
of straw color fluid, the mass was excised. Few enlarged
lymph nodes measuring around 1 x 1 cm were seen at the
left parapharyngeal area and were also excised. All the
specimens were sent for histopathological examination.
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|Figure 1: T2-weighted MRI demonstrating a mixed-intensity
lesion with mainly fluid component.
On gross pathological examination, it was a cystic mass
measuring 15x12x6 cm in diameter and 5-8 mm in wall
thickness. It contained yellowish gelatinous material. Serial
cut sections showed partly solid and partly cystic with
variegated appearance. Solid areas measured about 3x4x3
cm in diameter (Figure 2).
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|Figure 2: Cystic tumor mass was cut opened to see inner wall.
Lower part of the specimen was composed of solid gray white
Histopathologically, the cyst wall was partly lined by stratified squamous epithelium and partly by respiratory
typed pseudostratified ciliated columner epithelium
(Figure 3A). Subepithelial tissue showed many large foci of
mature neuroglial tissue (Figure 3B). Other mature tissue
components such as choroid plexus epithelium (Figure
3C), mucus secreting glands, pancreatic acini, salivary
glands, adipose tissue, skeletal muscle, bony trabeculae and
cartilages were seen in the cyst wall. Most of the solid area
was composed of mature neuroglial tissue with astrocytes,
oligodendrocytes and numerous psammomatous
calcifications (Figure 4A). Foci of mature ependymal cells
forming tubules and rosettes were also seen (Figure 4B).
Some of the foci of cartilages showed immature cartilage
without a clear cut margin which accounted for less than
10% of sample tissue (Figure 4C). However, immature
neuroglial tissue components were not seen. Sections from
one enlarged cervical lymph node showed foci of mature
neroglial tissue within a lymphoid architecture. Based on
the above histopathological features, the cystic mass was
diagnosed as mature teratoma (Grade 1) and the cervical
lymph node was diagnosed as nodal gliomatosis.
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|Figure 3: Sections from cystic wall. A) Cyst wall of the tumor was
partly lined by squamous epithelium and partly by respiratory
typed ciliated epithelium (H&E x100). B) Cyst wall of the tumor
was lined by respiratory typed ciliated epithelium and mature
neuroglial tissue beneath the epithelium (H&E x100). C) Cyst
wall of the tumor was partly lined by choroid plexus epithelium
in papillary configuration (H&E x100).
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|Figure 4: Sections from solid area. A) Foci of mature neuroglial
tissue composed of oligodendrocyts and psammomatous
calcification (H&E x200). B) Foci of mature ependymal cells in
neuroectodermal area forming tubules and rosettes (H&E x200).
C) Foci of immature cartilage without clear cut boundaries (H&E
Teratomas of the head and neck are interesting because
of their obscure origin, bizarre microscopic appearance,
unpredictable behavior and often dramatic clinical
. Cervical teratomas are uncommon.
Until 2009, only 217 cases of cervical teratomas have been
reported in the world literature and ninety percent of the
cases are diagnosed in the pediatric population6
. In the
vast majority of cases, the tumour presents clinically as a
large neck mass noted at birth4
. Most of the cases present
as midline mass and rarely appear in the lateral region7
Ninety percent of cervical teratomas are diagnosed in the
pediatric population; however, a minority can be seen in
. In our case, the mass was diagnosed since fetal
life and it presented as a lateral neck mass. The majority of
cervical teratomas presenting in infancy and early childhood
are mature in nature and exhibit benign clinical behavior;
whereas those presenting after the first decade of life exhibit
. In a study of 65 cases of childhood
teratomas under 7 years of age, cervical teratomas made up
only 4 cases with three mature and one immature case10
Most of the congenital cervical teratomas can be diagnosed
by antenatal ultrasound11
; however, cystic hygroma is
the most common mimicker4,
as in our case.
Approximately one third of the reported cervical teratomas
complicate maternal hydramnios12. However, there was
no maternal hydramnios in this case although the mother
had history of gestational diabetes.
Pathologically, teratomas have heterogeneous histological
characteristics. Mature teratomas may contain all manners
of adult tissues with varying degrees of organoid development,
whereas immature lesions may produce embryonic
or extra embryonic fetal tissue4. Up to 90% of childhood
teratomas contain derivatives from all three embryonic
germ layers. 20–40% contain some immature tissues.
In childhood, 75–85% of teratomas of the head and neck
region usually contain neuroectodermal elements (both
mature and immature)6. Mature neuroectodermal tissue
such as cerebellum, ependymal tubules, pituitary and
retina can be seen in mature teratoma and it does not result
misclassification as an immature teratoma13. Ependymal
rosettes should not be mistaken for the primitive rosettes
seen in an immature teratoma. The cells lining ependymal
rosettes have ample cytoplasm, the nuclei form only one or
two layers, and mitotic figures are not present14. In this
case, foci of ependymal cells forming tubules and vague rosettes
were also seen.
Grading and classification of both gonadal and extragonadal
teratomas are important for the management and
sometimes challenging for the anatomic pathologist. Teratomas
are generally classified into four grades according
to the Gonzalez-Crussi grading system4. Grade (0): all
component tissues appear well differentiated, grade (1): occasional
microscopic foci contain incompletely differentiated
tissues but not exceeding 10% of the sampled tissue,
grade (2): Immature tissue composed of 10-50% of sampled
tissue, and grade (3): more than 50% of sampled tissue are
composed of undifferentiated tissue. Both grade (0) and
(1) are considered as mature teratoma and both grade (2)
and (3) are considered immature teratoma (4). Our case
contained foci of incompletely differentiated immature cartilage
which accounted less than 10% of all sampled tissue;
we therefore decided the case was mature teratoma, grade
(1). All teratomas that contain areas of germ cell tumour,
non-germ cell malignant tumour and immature teratoma
with metastasis are considered malignant teratoma (4).
Most of the gynaecological literatures for ovarian teratoma
put grade (1) teratoma under immature teratoma15.
In this case, the striking feature was presence of large multiple
foci of mature neuroglial tissue consisting of astrocytes,
oligodendrocytes, neuron, microglia, ependymal cells and
even multiple foci of psammomatous calcification. Most
of the foci of glial tissue were hypercellular like a glial tumour.
However, they did not exhibit any pleomorphism or
mitosis. Foci of mature neuroglial tissue were also seen in
section of cervical lymph node adjacent to the tumour that was diagnosed as nodal gliomatosis. Nodal gliomatosis is
not indicative of malignant biological behavior and appears
to have the same prognostic significance as gliomatosis
peritonei in mature ovarian teratoma16. In this case, it
is possible that previous sclerotherapy caused extraction of
neuroglial tissue from cystic teratoma and implantation in
the nearby cervical lymph node resulting in nodal gliomatosis.
In conclusion, most of the congenital head and neck
teratomas are benign and mature in nature with a good
prognosis. Teratoma should be in the list of differential
diagnosis for congenital lesions presenting as a neck mass.
An ample amount of neuroectodermal tissue including
ependymal rosettes should not be mistaken as immature
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