 |
|
Orbital Mass Secondary to Precursor T-Cell Acute Lymphoblastic Leukemia
A Rare Presentation
Bita Esmaeli, MD;
L. Jeffrey Medeiros, MD;
Jeffrey Myers, MD;
Richard Champlin, MD;
Sanjay Singh, MD;
Lawrence Ginsberg, MD
Arch Ophthalmol. 2001;119:443-446.
ABSTRACT
We describe a 40-year-old woman with a history of precursor T-cell acute
lymphoblastic leukemia who developed an orbital mass associated with diffuse
infiltration of the paranasal sinuses. The clinical and radiologic findings
suggested an orbital abscess. Examination of orbital and ethmoid sinus biopsy
specimens revealed relapse of precursor T-cell acute lymphoblastic leukemia.
Although orbital involvement by granulocytic sarcoma (also known as extramedullary
myeloid cell tumor and chloroma) with or without concurrent acute myeloid
leukemia is well described in the literature, similar presence of acute lymphoblastic
leukemia of either precursor T-cell or B-cell lineage is rare.
INTRODUCTION
Orbital disease secondary to acute lymphoblastic leukemia (ALL) is rare,
particularly in the adult population. Despite several reports that describe
the clinical and radiologic features of orbital extramedullary myeloid cell
tumor (also known as granulocytic sarcoma or chloroma) secondary to myeloid
leukemias,1-3 we
were able to find only 1 case report of orbital and choroidal disease secondary
to ALL in an infant.4 We report the case of
a 40-year-old woman with an orbital mass and diffuse infiltration of paranasal
sinuses secondary to precursor T-cell ALL. The radiologic findings in this
patient were not typical for the previously described features of orbital
chloroma,5-8
but were more consistent with an orbital abscess. The clinical, radiologic,
as well as histopathologic features of this neoplastic process are described
in the present article.
REPORT OF A CASE
A 40-year-old woman with a history of precursor T-cell ALL with mediastinal
involvement was admitted to the hospital with a fever. She had a computed
axial tomography scan of the head and neck, which showed opacification of
the maxillary sinuses and ethmoidal sinuses, as well as a subperiosteal orbital
"abscess." An ophthalmological consult was requested.
MEDICAL HISTORY
The patient was diagnosed with precursor T-cell ALL approximately 1
year prior to this hospitalization. She had experienced multiple relapses
of her leukemia in the preceding year, and had been treated with various chemotherapeutic
regimens. She was being considered for allogenic bone marrow transplantation.
Four weeks prior to this hospitalization, she underwent uneventful sinus surgery
for pansinusitis. The cultures from the ethmoidal sinuses at that time grew
coagulase-negative Staphylococcus. She was treated
with systemic antibiotics and responded well.
CLINICAL FEATURES
The patient was found to be alert, oriented, and in no distress. She
denied any ocular or periocular symptoms or headaches. Her visual acuity was
20/20 OU. The external examination revealed quiet globes. The ocular adnexal
examination was positive for mild edema and echymosis of the left upper eyelid.
The results of the extraocular motility examination were normal with no diplopia.
There was minimal to no proptosis, although Hertel exophthalmometry measurements
were not done during the bedside examination. Confrontation visual fields
were full. The pupils reacted normally to light and accommodation, and there
was no afferent pupillary defect. A penlight examination revealed a normal
cornea and anterior chamber in each eye, with no evidence of inflammatory
signs. Intraocular pressures with a TonoPen (Mentor, Norwell, Mass) measured
18 mm Hg in each eye. A dilated fundus examination revealed cup-disc ratios
of 0.2 bilaterally with sharp disc margins. The central and peripheral retina
appeared normal in each eye.
The patient's white blood cell count was found to be 63 x 109/L on admission to the hospital.
COMPUTED TOMOGRAPHIC SCAN FEATURES
A low-density, extraconal mass was found in the superior medial left
orbit with marginal enhancement (peripheral enhancement with central nonenhancement)
consistent with an orbital abscess. There was no bone destruction (Figure 1). There was considerable opacification
of the frontal, maxillary, and ethmoid sinuses (Figure 2).
|
|
|
|
Figure 1. Enhanced computed tomography images
reveal the left superior medial orbital mass. A, Coronal computed tomography
image shows the left extraconal, marginally enhancing mass (arrows). B, An
axial computed tomography image also demonstrates the superior orbital mass
(arrowheads).
|
|
|
|
|
|
|
Figure 2. Axial enhanced computed tomography
images demonstrate sinus disease. Mucosal thickening is seen in the sphenoid
sinuses, and complete opacification of the ethmoid air cells is present.
|
|
|
HOSPITAL COURSE
The patient was taken to the operating room where explorations of the
left orbit and endoscopic sinus surgery were undertaken simultaneously. A
superonasal orbitotomy (Lynch incision) was performed, and a collection of
brownish gray material was found in the subperiosteal space. This material
was friable and difficult to excise, but as much of this material as possible
was removed. Bacterial (anaerobic and aerobic) and fungal cultures were obtained.
The tissue was also sent for histopathologic diagnosis. The cultures from
both orbital and sinus specimens were negative for bacteria and fungus.
The patient's high white blood cell count was treated with plasmaphoresis.
She then received systemic chemotherapy as recommended by her hematologist.
She subsequently underwent allogenic bone marrow transplantation. At the time
of this report, she remains asymptomatic from an ocular standpoint, and remained
free of disease for 3 months following her bone marrow transplantation.
HISTOPATHOLOGIC FEATURES
Biopsy specimens from the left orbit, left ethmoid sinus, and right
ethmoid sinus were obtained for examination. Histologically, all biopsy specimens
were similar, and the orbital specimen is shown in Figure 3A and Figure 3B.
Within the orbital connective tissue and sinusoidal mucosa, aggregates of
lymphoblasts were present intersecting between collagen bundles (Figure 3A). At high power, the lymphoblasts
had irregular nuclear contours and finely granular, blastic nuclear chromatin
without prominent nucleoli (Figure 3B).
Mitotic figures were easily identified.
|
|
|
|
Figure 3. Precursor T-cell acute lymphoblastic
leukemia involving the orbit and ethmoid sinuses. A, Low-power view of lymphoblasts
intersecting between collagen bundles in the orbit. The pigment in the field
is hemosiderin secondary to hemorrhage (hematoxylin-eosin, original magnification
x200). B, High-power field of lymphoblasts with finely granular blastic
chromatin and irregular nuclear contours (hematoxylin-eosin, original magnification
x1000). C and D, The lymphoblasts are strongly positive for TdT (C)
and CD3 (D) and were negative for CD20 (not shown) (immunoperoxidase with
hematoxylin counterstain, original magnification x400).
|
|
|
Immunohistochemical studies were performed using fixed, paraffin-embedded
tissue sections of the left ethmoid sinus tissue. The lymphoblasts were positive
for terminal deoxynucleotidyl transferase (Figure 3C) and the T-cell antigen CD3 (Figure 3D) and were negative for the B-cell antigen CD20 (not shown).
These results supported the diagnosis of precursor T-cell ALL.
COMMENT
We report an unusual orbital and paranasal sinus presentation of ALL,
which appeared radiologically as an abscess. To our knowledge, there is only
one other report of a leukemic infiltration of the orbit secondary to ALL.4 That case was in a 6-month-old boy, and the orbital
involvement was diffuse and also associated with intraocular (choroidal) infiltration.
Radiologically, the orbital lesion in our patient was a well-circumscribed,
hypodense mass with peripheral enhancement. This is in contrast to granulocytic
sarcomas, which are usually homogeneously enhancing masses.5, 8
Given our patient's previous history of sinus infection and the radiologic
appearance consistent with an orbital abscess, the most likely diagnosis was
an infectious process in the sinuses and in the orbit. However, histopathologic
evaluation of the biopsy specimens confirmed the diagnosis of leukemic infiltration
secondary to precursor T-cell ALL.
Unlike the orbital mass described in this patient, the majority of head
and neck or orbital masses secondary to leukemia are of the myeloid lineage.
Extramedullary myeloid cell tumors (also known as granulocytic sarcoma, chloroma,
or myeloblastoma) are the most common form of leukemic infiltration involving
the orbit.9 Orbital chloromas may occur in
various forms, especially in children. Focal masses may arise intraconally
or extraconally and may be bilateral.10 Generally,
the diagnosis of leukemia is known, but as with leukemic masses elsewhere,
they may precede the onset of systemic disease.
Before modern chemotherapy, central nervous system involvement by leukemia
was common, affecting approximately 75% of patients with ALL and up to 50%
of those with acute myelogenous leukemia.9
Extramedullary disease secondary to ALL has been reduced with chemoprophylaxis
and more advanced therapy for this disease. Our patient had several relapses
of her ALL prior to this presentation and was in blast crisis while she developed
the leukemic infiltration of the orbit and paranasal sinuses. Localized leukemic
nodules in the orbit may respond to external beam radiation therapy, but more
commonly, since they are associated with systemic blast crisis, chemotherapy
or bone marrow transplantation may be necessary.
Leukemic infiltration should be considered on the differential diagnosis
of a marginally enhancing mass of the orbit, even in the presence of diffuse
paranasal sinus disease, which may look suspicious for an infectious process
radiologically.
AUTHOR INFORMATION
Accepted for publication September 8, 2000.
Corresponding author: Bita Esmaeli, MD, Ophthalmology Section, Department
of Plastic Surgery, 1515 Holcombe Blvd, Box 443, Houston, TX 77030 (e-mail: besmaeli{at}mdanderson.org).
From the Department of Plastic Surgery, Ophthalmology Section (Dr Esmaeli),
and the Departments of Hematopathology (Dr Medeiros), Head and Neck Surgery
(Dr Myers), Bone Marrow Transplantation (Dr Champlin), and Radiology (Drs
Singh and Ginsberg), M. D. Anderson Cancer Center, Houston, Tex.
REFERENCES
| |
1. Jordan DR, Noel LP, Carpenter BF. Chloroma. Arch Ophthalmol. 1991;109:734-735.
ISI
| PUBMED
2. Bulas RB, Laine FJ, Narla LD. Bilateral orbital granulocytic sarcoma (chloroma) preceding the blast
phase of acute myelogenous leukemia: CT findings. Pediatr Radiol. 1995;25:488-489.
FULL TEXT
|
ISI
| PUBMED
3. Uyesugi WY, Watabe J, Petermann G. Orbital and facial granulocytic sarcoma (chloroma): a case report. Pediatr Radiol. 2000;30:276-278.
FULL TEXT
|
ISI
| PUBMED
4. McManaway III JW, Neely JE. Choroidal and orbital leukemic infiltrate mimicking advanced retinoblastoma. J Pediatr Ophthalmol Strabismus. 1994;31:394-396.
ISI
| PUBMED
5. Ginsberg LE, Leeds NE. Neuroradiology of leukemia. AJR Am J Roentgenol. 1995;165:525-534.
FREE FULL TEXT
6. Banna M, Aur R, Akkas S. Orbital granulocytic sarcoma. AJNR Am J Neuroradiol. 1991;12:255-258.
ISI
| PUBMED
7. Barnett MJ, Zussman WV. Granulocytic sarcoma of the brain: a case report and review of the
literature. Radiology. 1986;160:223-225.
FREE FULL TEXT
8. Sowers JJ, Moody DM, Naidich TP, Ball MR, Laster DW, Leeds NE. Radiographic features of granulocytic sarcoma (chloroma). J Comput Assist Tomogr. 1979;3:226-233.
ISI
| PUBMED
9. Bleyer AW. Central nervous system leukemia. In: Henderson HS, Likster TA, eds. Leukemia.
5th ed. Philadelphia, Pa: WB Saunders; 1990:207-224.
10. Zimmerman LE, Font RL. Ophthalmologic manifestations of granulocytic sarcoma (myeloid sarcoma
or chloroma). Am J Ophthalmol. 1975;80:975-990.
ISI
| PUBMED
SECTION EDITOR: W. RICHARD GREEN, MD
|
