Osteogenic sarcoma is a malignant bony tumor of mesenchymal cell ori gin. The association of osteogenic sarcoma with Paget's disease of the bone (osteitis deformans) is well-known. However, orbital involvement is rare; only two other cases have been reported in all of the literature. Untreated, the tumor is lethal, with death usually occurring from extension into the cranial fossa

Fry, CL

Hesse, RJ

Trevisani, MG

Willis, GW

Mary G. Trevisani

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Archives of Ophthalmology

A Rare Case of Orbital Osteogenic Sarcoma

University of Queensland eSpace

Figure 1. Goldmann visual field 1 month after initial evaluation. Figure 2. Fundus photograph of macular star formation at 1 month with
resolving disc edema.
Report of a Case. A 10-year-old girl
was examined for decreased vision in
the left eye. One month earlier, her
5-month-old household kitten had
scratched her face, and about 2 weeks
later, a febrile illness that lasted for
5 days developed in the child.
Her visual acuity was 20/20 OD
and she counted fingers at 2 ft in the
left eye. A 1.8 log unit relative affer¬
ent pupillary defect was present in
the left eye, and Goldmann perim-
etry demonstrated a central sco-
toma in the visual field of that eye
and a normal visual field in the right
eye. The results of the slit-lamp ex¬
amination were normal in both eyes.
Ophthalmoscopy showed a normal
right eye, with the left eye having
marked disc edema with peripapil-
lary retinal edema, a macular star,
and exudates. Bartonella henselae an¬
tibody titer was 35 enzyme immu-
noassay units (>20 units is consid¬
ered positive). Oral doxycycline
hyclate, 100 mg daily for fO days,
was initiated after consultation with
the patient's pediatrician.
One month later, visual acuity
had improved to 20/200 OS, with
a 0.6 log unit left afferent pupillary
defect. Goldmann perimetry showed
a central scotoma and marked
enlargement of the blind spot(Figure 1 ). Although there was less
edema of the left optic disc, the macu-
lar star was more pronounced(Figure 2). At 2 months, the optic
disc was pale, with only a few macu¬
lar exudates remaining.
Comment. Neuroretinitis is charac¬
terized by visual loss, disc edema,
macular exudates, central and ceco-
central scotomas, and relative affer¬
ent pupillary defects; recovery of vi¬
sion occurs in most, but not all, cases.
Reported causative agents have in¬
cluded viruses such as mumps, vari¬
cella, and herpes simplex; parasites
such as Toxocara cants and Toxo-
plasma gondii; and bacteria such as
Borrelia burgdorferi,Treponema pal-
lidum, Salmonella typhi, and  
henselae. The last agent is also impli¬
cated in cat-scratch disease. Al¬
though  henselae is thought to be a
common cause of neuroretinitis, di¬
agnosis has required either lymph
node or conjunctival biopsies (War-
thin-Starry silver impregnation stain
to identify the bacilli in tissue) or skin
antigen testing (intradermal injec¬
tion of treated pus from suppurative
lymph nodes of patients with cat-
scratch disease). The enzyme immu-
noassay test used in this case is a prac¬
tical way to determine the cause of
neuroretinitis. It is a relatively non-
invasive test, and it has high sensitiv¬
ity and specificity.2 Although guide¬
lines for treatment of neuroretinitis
associated with  henselae have not
been fully established, the identifica¬
tion of  henselae as the presumed
causative agent simplifies the diag¬
nostic investigation and offers sub¬
stantial prognostic information.
Roger W. Newsom, MD
Timothy J. Martin, MD
Ben Wasilauskas, MD
Winston-Salem, NC
Reprint requests to Wake Forest Uni¬
versity Eye Center, Medical Center
Boulevard, Winston-Salem, NC 27157(Dr Martin).
1. Regnery RL, Olson JG, Perkins BA, Bibb W. Se-
rological response to 'Rochalimaea henselae' an-
tigen in suspected cat-scratch disease. Lancet.
1992;339:1443-1444.
2. Zangwill KM, Hamilton DH, Perkins BA, et al.
Cat scratch disease in Connecticut: epidemiol-
ogy, risk factors, and evaluation of a new diag-
nostic test. N Engl J Med. 1993;329:8-13.
3. Golnik KC, Marotto ME, Fanous MM, et al. Oph-
thalmic manifestations of Rochalimaea species.
Am J Ophthalmol. 1994;118:145-151.
A Rare Case of Orbital
Osteogenic Sarcoma
Osteogenic sarcoma is a malignant
bony tumor of mesenchymal cell ori
gin. The association of osteogenic
sarcoma with Paget's disease of the
bone (osteitis deformans) is well\x=req-\
known. However, orbital involve-
ment is rare; only two other cases
have been reported in all of the lit-
erature. Untreated, the tumor is le-
thal, with death usually occurring
from extension into the cranial fossa.
Report of a Case. A 58-year-old man
was seen for a 1-month history of dip-
lopia, pain on eye movement, and left
upper eyelid ptosis. Ofsignificance in
his medical history was Paget's dis-
ease of the bone for 23 years and no
prior radiation therapy. A review of
systems was unremarkable.
The ophthalmic evaluation re-
vealed a best-corrected visual acuity
of 20/30 OD and 20/80 OS. The left
eyelid demonstrated 3 mm ofptosis.
Additionally, a 3-mm axial propto-
sis was present, and the globe was dis-
Downloaded From: http://archopht.jamanetwork.com/ by a UQ Library User  on 09/16/2015
Figure 1. Coronal view showing the extent of
the lesion (small arrows) and the areas of
calcification (large arrow).
placed 8 mm laterally and 4 mm in-
feriorly. A firm, slightly tender mass,
measuring 1.5X1.5 mm, was fixed to
the superior orbital rim. The pupils
were normal, with no relative affer¬
ent defect. Extraocular motility of the
left eye was limited in upgaze. Three
prism diopters of left hypotropia were
present. Other examination findings
were unremarkable.
A plain skull x-ray film revealed
bony erosion of the left orbital roofand
the frontal and ethmoidal sinuses, as
well as diffuse changes throughout the
calvarium, consistent with Paget's dis¬
ease. A contrast-enhanced computed
tomography scan of the brain and or¬
bits outlined an osteolytic lesion with
dystrophic calcification. The lesion in¬
volved the entire orbital roof, from the
orbital rim to the apex, and extended
into the left frontal and ethmoidal si¬
nuses and into the cribriform plate(Figure I ). The mass extended to the
dura of the frontal lobe without asso¬
ciated cerebral edema. Bony changes
throughout the calvarium were con¬
sistent with Paget's disease.
Significant results of a complete
diagnostic workup included a mark¬
edly elevated alkaline phosphatase of
1587 U/L (normal, 55 to 135 U/L), and
a normal chest x-ray film. An incisional
biopsy was performed.
Microscopic examination of the
sections, which were stained with
hematoxylin-eosin, revealed a tumor
composed ofneoplastic cells forming
osteoid, the histologie criterion for a
diagnosis ofosteosarcoma. The osteo-
blasts were pleomorphic and hyper-
chromatic and the amorphous islands
ofosteoid, formed by surrounding os-
teoblasts, did not polarize and stained
negatively with Alcian blue and mu-
Figure 2. Pleomorphic and hyperchromatic osteoblasts forming amorphous islands of osteoid that did
not polarize and stained negatively with Aldan blue and mucicarmine (hematoxylin-eosin,  400 oil
immersion).
cicarmine (Figure 2). Further elec¬
tron microscopic studies indicated the
tumor cells were producing masses of
tangledcollagen fibersconsistentwith
unmineralized osteoid. These findings
indicated osteogenic sarcoma in this
patient.
Results ofa subsequent systemic
evaluation, including a computed to-
mographic scan of the chest, were
normal. The patient was treated with
radiation and chemotherapy, but was
unavailable for follow-up 7 months
after the diagnosis was made.
Comment. Osteogenic sarcoma is typi¬
cally classified into two subtypes: pri¬
mary and secondary. The primary tu¬
mors arise de novo, typically in devel¬
oping bones, and account for 70% of
all cases. Secondary osteosarcomas de¬
velop in bones with preexisting frac¬
tures or disease such as Paget's disease,
in regions previously exposed to a car¬
cinogenic influence such as radiation
therapy, and in patients with heredi¬
tary retinoblastoma.
Only one case of orbital osteo¬
genic sarcoma concurrent with Pag¬
et's disease has been reported in the lit¬
erature. The patient described by Biodi '
in 1969 is strikingly similar to our pa¬
tient. Osteogenic sarcoma of the orbit
developed in this 69-year-old patient
after a 24-year history of Paget's dis¬
ease. He had painless exophthalmos
and a mass in the superior orbit and
died 6 months after surgical excision,
radiation therapy, andchemotherapy.
In his textbook of orbital diseases,
Henderson2 described a patient with
osteogenic sarcoma thatdeveloped 10
years after the onset ofPaget's disease.
Osteogenic sarcoma occurring
with osteitis deformans is a well-
recognized, yetuncommon disease that
is difficult to treat. The tumor causes
extensive local destruction and is
spread hematogenously. Aggressive
multimodal therapy, including surgi¬
cal resection followed by chemo¬
therapy and irradiation, is usually nec¬
essary.3 Despite aggressive interven¬
tion, the prognosis remains guarded,
particularlywhen Paget's disease is also
present.
Mary G. Trevisani, MD
Constance L. Fry, MD
Richard J. Hesse, MD
Gladden W. Willis, MD
New Orleans, La
We thank Alan Girch, MD, Vander-
bilt University Medical Center, Nash¬
ville, Tenn, for performing and in¬
terpreting the electron microscopy.
Reprint requests to Ochsner
Clinic, 1516 Jefferson Hwy, New Or¬
leans, LA 70121 (Dr Hesse).
1. Blodi FC. Pathology of orbital bones. Am J Oph-
thalmol. 1976;81:1-26.
2. Henderson JW. Fibro-osseous, osseous, and car-
tilaginous tumors of orbital bone. In: Hender-
son JW, ed. Orbital Tumors. New York, NY:
Raven Press; 1994:166.
3. Abramson DH, Ellsworth RM, Kitchin FD, Tung
G. Second nonocular tumors in retinoblastoma
survivors: are they radiation induced? Ophthal-
mology. 1984;91:1351-1355.
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A rare case of orbital osteogenic sarcoma

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A rare case of orbital osteogenic sarcoma

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