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OJHAS Vol. 10, Issue 4:
(Oct-Dec 2011) |
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| Granulocytic
Sarcoma as the First Sign of Acute Leukemia
in Childhood |
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Aalia R
Sufi,
Government Medical
College, Jammu and Kashmir,
Tejit Singh, Prof. & Former Head, Department of Ophthalmology, Government Medical
College, Srinagar, Jammu and Kashmir. |
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Address for Correspondence |
Government Medical
College,
Jammu and Kashmir, India.
E-mail:
aaliarasool_s@yahoo.com |
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Sufi AR, Singh T. Granulocytic
Sarcoma as the First Sign of Acute Leukemia
in Childhood. Online J Health Allied Scs.
2011;10(4):9 |
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Submitted: July 17, 2011; Suggested revision: Oct 20, 2011; Revised: Oct 24, 2011; Accepted: Jan 4, 2012; Published: Jan 15, 2012 |
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| Abstract: |
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Acute myeloid leukemia (AML) may rarely involve the orbit as
a solid tumor termed granulocytic sarcoma. This report describes the case of a child who presented with rapidly progressive
unilateral proptosis and was diagnosed as rhabdomyosarcoma. However subsequent examination of the peripheral blood film
revealed AML. Thus proptosis may present as the initial manifestation of AML.
Key Words:
Granulocytic sarcoma; Acute myeloid leukemia; Proptosis.
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The
orbit, especially in children can be involved in a number of
diseases either ophthalmic or non-ophthalmic. The common lesions
include orbital cellulitis, dermoid cysts, rhabdomyosarcoma and hemangioma.1
However certain rare causes also exist, like granulocytic sarcoma. Thus the differential diagnosis should be kept in mind and
a thorough clinical examination and appropriate investigations
should be carried out to facilitate early treatment.
Eight years old child presented with proptosis of right eye
with ocular pain and redness over a span of 15
days.
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Vision
in right eye was 20/200 (later decreased to 20/400). Proptosis of
6 mm was noted with downward and lateral displacement of globe [Fig 1]. Extra ocular movements were restricted superiorly and inferiorly. Conjuctiva was congested and chemosed, cornea showed exposure
keratitis, minimal hypopyon, pupil reacted sluggishly to light,
intraocular pressure was 22 mmHg, fundus examination revealed normal
disc with dilated tortuous vessels. Left eye was normal.
Figure 1: Photograph showing unilateral proptosis. |
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A
provisional diagnosis of rhabdomyosarcoma was made due to the
acute onset of proptosis and young age, rhabdomyosarcoma being
the commoner primary orbital malignancy in children. CT Scan
revealed 3 x 3 cm well defined mass in superior aspect of right
orbit with contrast enhancement, superior rectus was not seen
separately. No bone destruction, no intracranial extension was seen [Fig 2]. Radiologists suggested hemangioma.
Figure 2: CT scan
showing a lesion in the right orbit.
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Subsequently
peripheral blood film (PBF) was ordered which showed lymphopenia
and thrombocytopenia, hypochromic macrocytes with anisocytosis, polymorphs 30%. lymphocytes 25%, >blasts 45% [Fig 3].
On
the basis of the PBF report, patient was diagnosed as
a case of Acute Myeloid Leukemia. Further tests were done,
bone marrow examination revealed: blasts 30%, 18
- 20 microns in size with high nucleocytoplasmic ratio, open
chromatin and 2-3 nucleoli, Auer rod present, myeloperoxide
positive, lymphopenia and thrombocytopenia. On account of severe thrombocytopenia, we did not proceed with orbital biopsy.
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Figure 3:
High power (100X) view of peripheral blood film slide showing myeloblast. |
Figure 4:
Photograph showing regression of proptosis after chemotherapy |
Patient
received Inj. Arabinoside C 65mg for 7 days and Daunorubucin
35 mg per day for 3 days. Patient showed drastic regression
of proptosis, but developed corneal abscess [Fig 4]. The patient returned home and was lost to further follow-up.
Granulocytic sarcoma or chloroma
is a tumor composed of granulocytic precursor cells occurring
in an extramedullary location.2 The
term chloroma " green tumor " is derived from the greenish
gross coloration of this lesion, attributable to the myeloperoxidase
in the cells of granulocytic lineage.3 The
theory for the formation of these lesions is that normal hematopoietic
progenitor cells selectively bind to bone marrow stroma and
then begin to proliferate and differentiate, while in vitro they
bind to skin fibroblasts . Binding of these cells to the fibroblasts
localized in non – hematopoietic tissues may result in the
formation of extramedullary myeloid metaplasia.4
The predilection for bone or subperiosteal involvement in the
axial skeleton is thought to be related to the active hematopoiesis
in these locations.5 Orbital cases can be explained
by their origin from adjacent bone, or less commonly, the lacrimal
gland or extraocular muscles.
Myeloid sarcomas are most common in certain subtypes of AML, in particular M5a (monoblastic),
M5b (monocytic), M4 (myelomonocytic), and M2 (myeloblastic with
maturation).6
The
differential diagnosis of acute childhood proptosis includes orbital
cellulitis, inflammatory pseudotumor, ruptured dermoid cyst, rhabdomyosarcoma,
hemangioma, chocolate cyst of lymphangioma, neuroblastoma, granulocytic
sarcoma. Of all the orbital lesions only 5% are malignant and granulocytic
sarcoma accounts for only 1 of 250 malignant cases with the incidence
being slightly higher in Africa and Asia.7
In
a study of 86 Indian patients with AML, 8 (9.3%)
were found to have orbital deposits in one or both eyes.8 Majority
of the cases present as bilateral proptosis. However our
case showed the rare unilateral presentation.
In many cases proptosis precedes the systemic manifestations of
AML. Most cases in patients without a previous diagnosis progress
to AML within one year and even earlier in the case
of an initial orbital disease. However, some patients do not
develop hematologic evidence of the disease as long as 30 months
following the presence of an orbital tumor.9
In our case too, proptosis preceded the systemic manifestation
of AML. In the absence of systemic features it becomes difficult
to diagnose granulocytic sarcoma. In such cases PBF, a simple
and inexpensive investigation proves effective in diagnosis and
acts as an easy tool in saving time and money spent on CT
and MRI and thus initiating early treatment.
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Sethi A, Ghose S, Gujral S, Jain P, Kumar R. Childhood proptosis: The invaluable,
though often overlooked peripheral blood smear. Indian J Ophthalmol
2001;49(2):121-123.
- Alkatan H, Chaudhry
I. Myeloid sarcoma of the orbit. Annals of Saudi Medicine 2008;28(6):461-465.
- Shields JA, Stopyra
GA, Marr BP, Shields CL. Bilateral Orbital Myeloid Sarcoma as Initial
Sign of Acute Myeloid Leukemia: Case Report and Review of the Literature.
Arch Ophthalmol 2003;121:138-142.
- Bekassy AN, Hermans
J, Gorrin NC, Gratwohl A . Granulocytic sarcoma after allogenic BMT:
a retrospective European multicenter survey. Bone Marrow Transplant
1996;17:801-808.
- Davis JL, Parke DW 2nd, Font RL. Granulocytic sarcoma of the orbit.
A clinicopathologic study. Ophthamol 1985;92(12):1758-1762.
- Byrd JC, Edenfield
J, Shields DJ, et al. Extramedullary myeloid cell tumors in acute non-lymphocytic
leukemia: a clinical review. J Clin Oncol 1995; 13: 1800-1816.
- Zimmerman LE, Font
RL. Ophthalmologic manifestations of granulocytic sarcoma (myeloid sarcoma
or chloroma): the third Pan American Association of Ophthalmology and
American Journal of Ophthalmology Lecture. Am J Ophthalmol 1975;80:975-990.
- Ghose S, Kumar
R,Chaudhuri S, Jain Y,Gujral S, Singh H, et al. Importance of bone marrow
examination in childhood proptosis In: Pasricha JK. (ed). Indian Ophthalmology
Year Book 1997, Proc 55th All India Ophthalmol Soc Conf. New Delhi. 1997. pp 414-416.
- da Fonseca Junior
NL, Paves L, Nakanami DM, Seivas MT, Manso PG. Sarcoma granulocitico
em orbita: relato de caso. Arq Bras Ofthalmol 2005;68(4):557-560.
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