Congenital orbital teratomas are rarely malignant. In fact, a total
of 4 prior reports exist in the literature describing malignant orbital teratomas,
and only 2 of these describe the nature of the malignant cells. We report
a case of a congenital orbital teratoma containing foci of neuroblastomatous
elements confirmed by immunohistochemical techniques. Given that the tumor
was noninvasive and no systemic involvement was discovered, no further treatment
was undertaken. Two years following the operation, the patient remained free
of tumor in the orbit and at other sites. A review of the literature concludes
that similar cases should be handled by complete excision, and further therapy
should be based on the pathological findings of the individual case.
Teratomas are generally benign congenital growths, which by definition
consist of derivatives from each of the 3 germ-cell layers. They are most
commonly found in the gonads, but they may develop ectopically in cervical,
mediastinal, sacrococcygeal, intracranial, and orbital locations.1 Primitive neuroectodermal tissue has long been
known to be associated with teratomatous tissue in both intracranial2 and ovarian sites.3,4
One report found a neuroblastoma within a testicular teratoma.5
Primary congenital teratomas of the orbit are uncommon and rarely malignant.
Four prior reports describe malignant orbital teratomas.6- 9
One case was primarily an intracranial teratoma involving the optic nerve.6 Another report had no description of the nature
of the malignant cells.7 Garden and McManis8 described a case in which the malignant cell population
appeared 3 years after the excision of a benign orbital teratoma and consisted
of cells believed to be a malignant germ cell tumor. An orbital teratoma with
neuroblastic and rhabdomyoblastic elements has also been documented.9 We report a case of orbital teratoma with malignant
The patient was the product of an uncomplicated pregnancy and was born
at full term via normal abdominal delivery. She was initially seen at age
5 weeks for the evaluation of congenital and increasing proptosis of her left
eye. At that time, grossly she had 4 to 5 mm of proptosis of that eye, and
there was positive resistance to retropulsion. Pupils were 3 mm and sluggish
bilaterally, without a relative afferent defect. The anterior chamber was
quiet and of moderate depth. Biomicroscopy and fundoscopy were unremarkable.
Cycloplegic retinoscopy revealed a refraction of +3.50 +.50 × 80 OD
and +5.50 +1.50 × 90 OS. A magnetic resonance imaging scan showed a
diffuse mass that enhanced with contrast in the left orbit. An orbital ultrasound
revealed cystic cavities with fluid levels present within the orbit. Lymphangioma
was believed to be the most likely diagnosis, and the mass was observed.
By 6 months of age, the patient had progressive proptosis of her left
eye. The extraocular movements remained intact, but the left eye was unable
to fix. External examination of the left eye revealed a palpable mass in the
inferolateral orbit. No relative afferent pupillary defect was present. Cycloplegic
retinoscopy revealed increased hyperopia with a refraction of +4.00 +1.50
× 90 OD and +14.00 +3.00 × 85 OS. The right fundus was unremarkable,
while the left showed chronic papilledema characterized by a pale halo of
edema around the disc. An orbital computed tomography scan showed a septated
intraconal mass with calcifications that had increased in size from the previous
magnetic resonance imaging findings, and had extended into the lacrimal fossa
(Figure 1). The orbital rim was
expanded and thinned, but there was no osseous destruction. The orbital tumor
was excised by a left lateral orbitotomy.
Axial computed tomography scan
at age 6 months showing marked proptosis secondary to a partially septated
intraconal mass with calcifications. Note that the orbital rim is expanded
and thinned without osseous destruction.
The specimen consisted of 3 pieces of tissue, the largest measuring
30 × 19 × 9 mm. The specimen was bisected, and examination revealed
areas of a serosalike surface. In addition, there was one cystic area filled
with gelatinous material and hair. Another area was firm and gray, suggestive
Microscopic examination revealed a complex cystic structure lined partially
by pseudostratified ciliated columnar epithelium and partially by keratinized
stratified squamous epithelium. There were adnexal structures in the portion
of the wall lined by the squamous epithelium. The cyst was filled with keratinous
debris. Adjacent to the cyst was a well-circumscribed cartilaginous structure.
In addition, there was mature central nervous system tissue present. In one
focal area associated with the central nervous system tissue, there was a
focus (measuring 5 × 4 mm) of undifferentiated, small, round cells with
modest amounts of eosinophilic cytoplasm (Figure 2 and Figure 3).
The chromatin of the cells was moderately condensed, and there were occasional
small nucleoli. There was 1 mitosis in 20 high-power fields. There were no
Close proximity of mature nervous
tissue with immature neuroblastic tissue in the teratoma (hematoxylin-eosin,
original magnification ×200).
Area of neuroblastoma, demonstrating
undifferentiated small, round cells with high nuclear-cytoplasmic ratios,
moderately condensed chromatin, and occasional nucleoli (hematoxylin-eosin,
original magnification ×200).
Paraffin-embedded sections were stained using an immunoperoxidase technique.
The small, undifferentiated cells displayed negative immunoreactivity with
keratin, CD99 (product of MIC2 gene, positive in
glioblastoma, ependymoma, Ewing sarcoma, and acute lymphoblastic leukemia),
and vimentin. There was positive immunoreactivity with neuron-specific enolase
(Figure 4) and synaptophysin. The
pattern of reactivity was consistent with neuroblastoma, and would be unlikely
in any of the morphologic differentials in this case, which include leukemic
infiltrates, Ewing sarcoma, primitive neuroectodermal tumor, and Wilms tumor.
Neuron-specific enolase stain
of the immature cells within the teratoma. Positive (brown) staining is consistent
with a diagnosis of neuroblastoma (original magnification ×400).
The intriguing aspect of this case was the presence of immature cells
consistent with neuroblastoma in an otherwise typical orbital teratoma. The
presence of these cells raised interesting questions in the subsequent management
of the patient. Some authorities feel that the presence of immature cellular
components should not be taken as evidence of malignancy.4
However, the prognosis of central nervous system teratomas is worse for the
immature teratoma than for the mature teratoma, and dissemination of tumor
cells systemically and in cerebrospinal fluid has been documented in immature
Mayberger et al,4 in a report on a
benign cystic teratoma of the ovary with neuroblastomatous change, suggested
that if the teratoma is noninvasive, without metastasis, then removal alone
is adequate. This suggestion relies on the theory that the primitive neuroectodermal
tissue within the teratoma is derived from ectodermal germ cells and not from
mature neural tissue, which developed into undifferentiated tissue. However,
a separate study warned that the clinician must be aware that on occasion,
primary neuroectodermal tumors arise ectopically in the ovary, along with
teratomatous tissue.3 These latter tumors
are known to metastasize, with the risk increasing as the degree of differentiation
of the neuroectodermal tissue decreases. The same study suggested that tumors
with endodermal and mesodermal features along with primitive neuroectodermal
tissue may be classified and treated as benign teratomas, while those with
massive, confluent growth of neuroectodermal tissue and little of the other
2 germ layers may be primary malignant tumors.
Ulbright et al5 argue that treatment
should be individualized depending on the type of non–germ cell tumor
involved, speculating that some forms of non–germ cell malignancies
(specifically leiomyosarcoma, nephroblastoma, neuroblastoma, and giant cell
tumor), when found within teratomas, may not affect the prognosis.5 As a result, they argue that such tumors should
be called "non-germ cell malignancy in teratoma" and advocate treatment of
the growth as a benign teratoma with complete resection. The results of their
study further indicate that the role of chemotherapy is questionable in such
The management of this case was discussed at the Pediatric Tumor Board.
Based on the above considerations, this tumor was classified as an immature
teratoma containing neuroblastomatous elements. The tumor had been completely
excised, and no other treatment was undertaken. The child has been followed
up for 2 years and has no evidence of recurrent orbital tumor or neuroblastoma
at other sites.
In conclusion, orbital teratomas may rarely contain non–germ cell
malignancies. The initial clinical management of these cases should be complete
excision. Decisions regarding adjunctive chemotherapy or radiation therapy
should be based on the pathologic findings for the individual case.
Corresponding author and reprints: David J. Wilson, MD, 3375 SW Terwilliger
Blvd, Casey Eye Institute, Portland, OR 97201-4197 (e-mail: email@example.com).
Wilson DJ, Dailey RA, Wobig JL, Dimmig JW. Neuroblastoma Within a Congenital Orbital Teratoma. Arch Ophthalmol. 2002;120(2):213-215. doi: