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

Singh, T

Sufi, AR

English

CogPrints Cognitive Sciences Eprint Archive

Granulocytic Sarcoma as the First Sign of Acute Leukemia in Childhood

Cognitive Sciences ePrint Archive

 Case Report:
Granulocytic Sarcoma as the First Sign of Acute Leukemia in Childhood
Aalia R Sufi, Government Medical College, Jammu and Kashmir,
Tejit Singh, Prof. & Former Head, Department of Ophthalmology, Government Medical College, Srinagar, Jammu and Kashmir.
Address for Correspondence:
Dr. Sufi Aalia R,
Government Medical College,
Jammu and Kashmir, India. 
E-mail: aaliarasool_s@yahoo.com
Citation: Sufi AR, Singh T. Granulocytic Sarcoma as the First Sign of Acute Leukemia in Childhood.  Online J Health Allied Scs.  
2011;10(4):9
URL: http://www.ojhas.org/issue40/2011-4-9.htm
Open Access Archives: http://cogprints.org/view/subjects/OJHAS.html and http://openmed.nic.in/view/subjects/ojhas.html
Submitted: July 17, 2011; Suggested revision: Oct 20, 2011; Revised: Oct 24, 2011; Accepted: Jan 4, 2012; Published: Jan 15, 2012
Abstract: Acute myeloid leukemia (AML) may rarely involve 
the orbit as a solid tumor termed granulocytic sarcoma. This re-
port describes the case of a child who presented with rapidly 
progressive  unilateral  proptosis  and  was  diagnosed  as  rhab-
domyosarcoma. However subsequent examination of the peri-
pheral blood film revealed AML. Thus proptosis may present 
as the initial manifestation of AML. 
Key Words: Granulocytic sarcoma; Acute myeloid leukemia; 
Proptosis.
Introduction:
The orbit, especially in children can be involved in a number of 
diseases either ophthalmic or non-ophthalmic. The common le-
sions  include  orbital  cellulitis,  dermoid  cysts,  rhabdomyosar-
coma 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.
Case Report:
Eight years old child presented with proptosis of right eye with  
ocular pain and   redness over a span of 15 days.
Vision  in  right  eye  was  20/200  (later  decreased  to  20/400). 
Proptosis of 6 mm was noted with downward and lateral dis-
placement of globe [Fig 1]. Extra ocular movements were re-
stricted superiorly and inferiorly. Conjuctiva was congested and 
chemosed, cornea showed exposure keratitis, minimal hypopy-
on, pupil reacted sluggishly to light, intraocular pressure was 22 
mmHg, fundus examination revealed normal disc with dilated 
tortuous vessels. Left eye was normal.
A provisional diagnosis of rhabdomyosarcoma was made due 
to the acute onset of proptosis and young age, rhabdomyosar-
coma being the commoner primary orbital malignancy in chil-
dren. 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  heman-
gioma.
Subsequently peripheral blood film (PBF) was ordered which 
showed  lymphopenia  and  thrombocytopenia,  hypochromic 
macrocytes  with anisocytosis,  polymorphs  30%. lymphocytes 
25%, >blasts 45% [Fig 3].
Figure 1: Photograph showing unilateral proptosis.
Figure 2:  CT scan showing a lesion in the right orbit.
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,  lymph-
1
This work is licensed under a
Creative Commons Attribution-
No Derivative Works 2.5 India License
Online Journal of Health and Allied Sciences
Peer Reviewed, Open Access, Free Online Journal
Published Quarterly : Mangalore, South India : ISSN 0972-5997
Volume 10, Issue 4; Oct-Dec 2011
openia and thrombocytopenia. On account of severe thrombo-
cytopenia, we did not proceed with orbital biopsy.
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 Daun-
orubucin 35 mg per day for 3 days. Patient showed drastic re-
gression  of proptosis,  but developed corneal abscess [Fig 4]. 
The patient returned home and was lost to further follow-up.
Discussion:
Granulocytic  sarcoma  or  chloroma  is  a  tumor  composed  of 
granulocytic precursor cells occurring in an extramedullary loc-
ation.2  The term chloroma " green tumor " is derived from the 
greenish gross coloration of this lesion, attributable to the myel-
operoxidase in the cells of granulocytic lineage.3 The theory for 
the formation of these lesions is that normal hematopoietic pro-
genitor 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 local-
ized 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 loca-
tions.5 Orbital cases can be explained by their origin from adja-
cent bone, or less commonly, the lacrimal gland or extraocular 
muscles.  Myeloid sarcomas are most common in certain sub-
types of AML, in particular M5a (monoblastic), M5b (mono-
cytic), M4 (myelomonocytic), and M2 (myeloblastic with mat-
uration).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 sar-
coma accounts for only 1 of 250 malignant cases with the incid-
ence 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 de-
velop 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 inex-
pensive 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.
References:
1. Sethi A, Ghose S, Gujral S, Jain P, Kumar R. Childhood 
proptosis: The invaluable, though often overlooked peri-
pheral blood smear. Indian J Ophthalmol 2001;49(2):121-
123.
2. Alkatan H, Chaudhry I. Myeloid sarcoma of the orbit. An-
nals of Saudi Medicine 2008;28(6):461-465.  
3. 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.
4. Bekassy  AN,  Hermans  J,  Gorrin  NC,  Gratwohl  A  . 
Granulocytic sarcoma after allogenic BMT: a retrospect-
ive  European  multicenter  survey.  Bone  Marrow Trans-
plant 1996;17:801-808.
5. Davis JL, Parke DW 2nd, Font RL. Granulocytic sarcoma 
of  the  orbit.  A  clinicopathologic  study.  Ophthamol 
1985;92(12):1758-1762.
6. 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.
7. 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.
8. 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 Ophthal-
mology Year Book 1997, Proc 55th All India Ophthalmol 
Soc Conf. New Delhi. 1997. pp 414-416.
9. 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.
2


Aalia R Sufi

Tejit Singh

Directory of Open Access Journals

http://doaj.org/search?source=%7B%22query%22%3A%7B%22bool%22%3A%7B%22must%22%3A%5B%7B%22term%22%3A%7B%22id%22%3A%22334fee13fb1445fc9c57953db2a85c42%22%7D%7D%5D%7D%7D%7D

Granulocytic Sarcoma as the First Sign of Acute Leukemia in Childhood

Abstract

Similar works

Full text

Available Versions

CogPrints Cognitive Sciences Eprint Archive

Cognitive Sciences ePrint Archive

Directory of Open Access Journals