cal Center, No. 325, Section 2, Cheng-Kung Road, Neihu
114, Taipei, Taiwan ([email protected]).
Financial Disclosure: None reported.
1. Janatpour KA, Choo PH, Lloyd WC III. Primary orbital peripheral T-cell lymphoma: histologic, immunophenotypic, and genotypic features. Arch
Ophthalmol. 2007;125(9):1289-1292.
2. Woog JJ, Kim Y-D, Yeatts RP, et al. Natural killer/T-cell lymphoma with ocular and adnexal involvement. Ophthalmology. 2006;113(1):140-147.
3. Coupland SE, Foss HD, Assaf C, et al. T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases. Ophthalmology. 1999;
106(11):2109-2120.
Orbital Chondromyxoid Fibroma
P
rimary tumors of orbital bone are rare, constituting up to 2% of all orbital masses.1 Chondromyxoid fibroma (CMF) is one of the least common
tumors of bone, composing less than 1% of bone tumors and less than 2% of benign bone tumors.2,3 Apart
from brief case reports,4-6 orbital CMF has not been clearly
documented in the ophthalmic literature. To our knowledge, we report for the first time the clinicopathological
features and management options of an orbital CMF arising from the frontal bone.
Report of a Case. A 37-year-old woman had slowly progressive swelling of the left upper eyelid temporally associated with occasional headache and shooting pain for
3 years. On examination, visual acuity was 20/20 OU. The
left eye showed 4 mm of proptosis with downward displacement, mild blepharoptosis, and choroidal folds at
the posterior pole (Figure 1A). Computed tomography disclosed a superotemporal, noninfiltrative orbital
mass with erosion of the adjacent frontal bone (Figure 1B
and C). Differential diagnosis included lacrimal gland tumors, atypical dermoid cysts, and benign fibro-osseous
lesions such as osteoma, fibrous dysplasia, or ossifying
fibromyxoid tumor of soft parts.
The patient underwent transcutaneous extraperiosteal orbitotomy. Intraoperatively, the periosteum was separated by blunt dissection from the bone in the peripheral portions of the mass both superiorly and inferiorly.
The mass proved to be located mainly in the extraperiosteal space, pushing the inferior and medial periosteal
border into the orbital soft tissue. The tumor centrally
showed a close connection to the bony wall. En bloc resection including the tumor, surrounding periosteum, and
adjacent bony wall was performed.
Macroscopically, the mass measured 20 ⫻ 15 ⫻ 7
mm. Histopathological examination revealed a CMF
(Figure 2A-C). The soft-tissue mass was surrounded
inferomedially by periosteum. The periosteum showed
calcification superiorly and laterally corresponding to the
radio-dense margins on the computed tomographic scan.
The lateral borders of the mass consisted of bone. The cellular elements displaying a low proliferation rate (MIB1⬍1%) were positive for S-100B protein in the central chondroid area and positive for vimentin and smooth muscle
actin in the peripheral fibroblastic area but negative for desmin, CD68, CD34, and CD31. Ultrastructural findings
(Figure 2D and E) supported the diagnosis of CMF.
A
B
C
D
Figure 1. Clinical features of an orbital chondromyxoid fibroma arising from the frontal bone in a 37-year-old woman. A, Slowly progressive proptosis, downward
displacement, and mild blepharoptosis of the patient’s left eye, of 3 years’ duration. B, Axial computed tomographic scan showing a circumscribed, round to
ovoid, superotemporal orbital mass with radio-dense margins and a lucent central component isodense to muscle tissue indenting the globe inferiorly. C, Axial
computed tomographic scan with bone window settings clearly delineating the incompletely formed sclerotic rim surrounding the round to ovoid mass with focal
erosion (arrow) and thinning of the adjacent frontal bone. D, Marked improvement of proptosis, downward displacement, and blepharoptosis without evidence of
recurrence or metastasis 2 years after transcutaneous extraperiosteal orbitotomy with en bloc resection including the tumor, surrounding periosteum, and
adjacent bony wall.
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A
B,D
C,E
B
C
D
E
Postoperatively, recovery was fast and unremarkable. Two years after surgery, the patient showed marked
improvement of proptosis, downward displacement, and
blepharoptosis without evidence of recurrence or metastasis (Figure 1D).
Comment. Chondromyxoid fibroma manifests most
frequently in the second and third decades of life, more
often in males than in females.2,3 The long bones are the
most common site, followed by the flat bones and the
bones of the hands and feet.3 Craniofacial involvement
is relatively rare.3 Histopathological differential diagnosis includes chondrosarcoma, enchondroma, or
chordoma.2
Chondromyxoid fibroma with orbital involvement has
been reported only in brief case reports.4-6 Hashimoto et
al4 and Cruz et al5 described a CMF of the ethmoid sinus
destroying the medial orbital wall. Wolf et al6 reported
an intracranial CMF of the frontal-sphenoid junction with
secondary orbital involvement. In our patient, there
was—to our knowledge for the first time—marked downward displacement and indentation of the globe with choroidal folds caused by progressive orbital tumor growth.
Because the adjacent periosteum was intact, we elected
to take an extraperiosteal approach, separating tumor and
periosteum from bone as much as possible. In the area of
close adhesion to the adjacent frontal bone, an en bloc
resection including the tumor, surrounding perios-
Figure 2. Histopathological and ultrastructural findings of an orbital
chondromyxoid fibroma arising from the frontal bone. A, Histopathological
section showing an overview of the orbital mass surrounded in part by
periosteum (arrows) and bony elements (arrowheads) (Masson trichrome,
original magnification ⫻12.5). The letters indicate the locations of figure parts
B, C, D, and E. B, Centrally located hypocellular area with spindle-shaped or
stellate cells in a myxoid matrix (hematoxylin-eosin, original magnification
⫻200). C, Peripherally located hypercellular area with many rather uniform
spindle-shaped cells and focal calcification (hematoxylin-eosin, original
magnification ⫻100). D, Transmission electron microscopy of the central
hypocellular area showing stellate chondroblastlike cells with irregularly
shaped, euchromatin-rich nuclei with inconspicuous nucleoli, prominent
dilated rough endoplasmic reticulum, bundles of intermediate filaments, and
isolated lipid droplets (bar = 5 µm). E, Transmission electron microscopy of the
peripheral hypercellular area showing rather spindle-shaped fibroblastic cells
with oval nuclei and moderate amounts of rough endoplasmic reticulum
(bar = 4 µm).
teum, and adjacent bony wall was performed. Complete
en bloc resection is important regarding both histopathological diagnosis and the prevention of tumor recurrence. Simple curettage may favor underdiagnosis
and explain in part why the entity has been rarely
documented.
In conclusion, CMF should be considered as a rare benign lesion in the differential diagnosis of primary orbital
bone tumors.
Ludwig M. Heindl, MD
Kerstin U. Amann, MD
Arndt Hartmann, MD
Friedrich E. Kruse, MD
Leonard M. Holbach, MD
Correspondence: Dr Heindl, Department of Ophthalmology and University Eye Hospital, University ErlangenNürnberg, Schwabachanlage 6, 91054 Erlangen, Germany ([email protected]).
Financial Disclosure: None reported.
Previous Presentation: This study was presented at the
47th Annual Meeting of the European Ophthalmic Pathology Society; June 2008; Besanc¸on, France.
Additional Contributions: Mathias Werner, MD, provided expert evaluation.
1. Selva D, White VA, O’Connell JX, Rootman J. Primary bone tumors of the
orbit. Surv Ophthalmol. 2004;49(3):328-342.
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2. Zillmer DA, Dorfman HD. Chondromyxoid fibroma of bone: thirty-six cases
with clinicopathologic correlation. Hum Pathol. 1989;20(10):952-964.
3. Wu CT, Inwards CY, O’Laughlin S, Rock MG, Beabout JW, Unni KK. Chondromyxoid fibroma of bone: a clinicopathologic review of 278 cases. Hum Pathol.
1998;29(5):438-446.
4. Hashimoto M, Izumi J, Sakuma I, Iwama T, Watarai J. Chondromyxoid fibroma of the ethmoid sinus. Neuroradiology. 1998;40(9):577-579.
5. Cruz AA, Mesquita IM, Becker AN, Chahud F. Orbital invasion by chondromyxoid fibroma of the ethmoid sinus. Ophthal Plast Reconstr Surg. 2007;23(5):
427-428.
6. Wolf DA, Chaljub G, Maggio W, Gelman BB. Intracranial chondromyxoid
fibroma: report of a case and review of the literature. Arch Pathol Lab Med.
1997;121(6):626-630.
Durable Response to Chemotherapy
for Recurrent Squamous Cell Carcinoma
of the Cheek With Perineural Spread
S
quamous cell carcinoma (SCC) in the periocular
region can invade the orbit and intracranial cavity.1 One route of such spread, known as perineural spread (PNS), involves the contiguous spread of
the tumor along the potential space between a nerve and
its sheath.2-6 Perineural spread is associated with a high
rate of recurrence, metastasis, and poor prognosis. The
usual treatment for SCC with PNS is surgical resection
followed by wide-field radiation therapy (RT).4 We herein
report a case of recurrent SCC with PNS treated with intravenous chemotherapy as a single modality with complete and sustained resolution of clinical and radiographic signs of PNS.
Report of a Case. A 70-year-old man had undergone excision of an invasive SCC (⬍1 cm wide) of the right cheek
Figure 1. Histologic section of the lesion on the cheek shows squamous cell
carcinoma (hematoxylin-eosin, original magnification ⫻10).
with positive excision margins 2 years before the referral. He developed right facial paralysis and lower eyelid
ectropion a few weeks after excision. He underwent surgical repair of paralytic ectropion presumed to be due to
idiopathic Bell’s palsy. The ectropion recurred after 8
months. Magnetic resonance imaging revealed an infraorbital right cheek mass that extended into the masticator space. The patient was referred to the M. D. Anderson Cancer Center for further management. The histologic
sections of the original lesion on the cheek were reviewed at our institution and the diagnosis of SCC was
confirmed (Figure 1). No perineural invasion was noted
on the representative section of the original outside
specimen.
Extraocular motility examination suggested a right
abduction deficit and right esotropia (Figure 2A).
Ocular adnexal examination results were significant for
right eyebrow ptosis and hypesthesia of the right cheek
and cornea. The patient had 11 mm of lagophthalmos,
right lower eyelid paralytic ectropion, and an associated
corneal ulcer. The patient denied significant pain or
paresthesias.
A diagnosis of recurrent SCC of the cheek with extensive PNS and resultant multiple cranial neuropathies
was made. Repeated magnetic resonance imaging again
revealed a right premaxillary soft tissue tumor with extensive PNS along the right infraorbital nerve with extension to the right pterygopalatine fossa, the right cavernous sinus, and the right Meckel cave (Figure 3A
and C).
Given the extensive skull base spread, it was felt that
the lesion was not surgically resectable. Because the patient had no significant pain, RT was deferred. Intravenous chemotherapy consisting of 736 mg of carboplatin
and 380 mg of paclitaxel was administered every 3 weeks
for 4 cycles. After 2 cycles of chemotherapy, the patient
experienced significant resolution of clinical (Figure 2B)
and radiographic (Figure 3B and D) signs of PNS. Three
years after completion of chemotherapy, the patient remains without clinical or radiographic evidence of disease recurrence.
Comment. We report here an impressive durable response to systemic cytotoxic chemotherapy delivered as
single-modality treatment for advanced recurrent SCC
with PNS. Chemotherapy for head and neck SCC is usually delivered as induction neoadjuvant chemotherapy
before surgery or RT or concurrently with RT. We were
unable to find any other example in the literature of the
use of systemic chemotherapy as single-modality treatment for SCC with PNS.
Perineural spread occurs in 2.5% to 14% of head and
neck SCCs4,5 and 1% to 8% of periocular SCCs.1 Of
those cases, only 30% to 40% are symptomatic.2 Patients who develop symptoms may have facial paresthesia, facial paralysis, exposure keratopathy, facial pain,
diplopia, and hearing loss. Perineural spread is often
missed until advanced stages; thus, the prognosis for
patients with PNS is often poor. McNab et al3 reported
that of 21 patients with PNS of SCC in the periorbital
region, 13 (62%) had died of disease by the 3-year
follow-up.
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