|Year : 2014 | Volume
| Issue : 1 | Page : 35-37
Acute myeloid leukemia presenting as bilateral proptosis and right temporal swelling
Sajid Ansari1, Raj Kumar Rauniyar1, Kanchan Dhungel1, Panna Lal Sah1, Kaleem Ahmad1, Mukesh Kumar Gupta1, Meenu Agrawal2
1 Department of Radiodiagnosis and Imaging, B.P. Koirala Institute of Health Sciences, Dharan, Nepal
2 Department of Pathology, B.P. Koirala Institute of Health Sciences, Dharan, Nepal
|Date of Web Publication||1-Mar-2014|
Departments of Radiodiagnosis and Imaging, B.P. Koirala Institute of Health Sciences, Dharan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Ansari S, Rauniyar RK, Dhungel K, Sah PL, Ahmad K, Gupta MK, Agrawal M. Acute myeloid leukemia presenting as bilateral proptosis and right temporal swelling. Oman J Ophthalmol 2014;7:35-7
|How to cite this URL:|
Ansari S, Rauniyar RK, Dhungel K, Sah PL, Ahmad K, Gupta MK, Agrawal M. Acute myeloid leukemia presenting as bilateral proptosis and right temporal swelling. Oman J Ophthalmol [serial online] 2014 [cited 2020 Jun 1];7:35-7. Available from: http://www.ojoonline.org/text.asp?2014/7/1/35/127927
| Introduction|| |
In acute myeloid leukemia (AML), there is proliferation of malignant clones of immature myeloid cells, which replaces the bone marrow and invades other tissues of the body.  This tumor can manifest prior to concomitantly or even during remission of systemic leukemia. 
A 6-year-old boy presented with low-grade fever for one month, with sudden onset progressively increasing bilateral proptosis and right temporal swelling. On examination, he was cachexic and had significant loss of weight and appetite. There was no organomegaly or lymphadenopathy. He had bilateral proptosis with normal visual acuity (6/6). Extraocular mobility was restricted laterally and inferiorly in both eyes. Pupillary reaction was normal in both the eyes. There was an ill-defined, nontender, firm swelling in the right temporal region. His neurological examination was normal. His hemoglobin was 7.3gm%, total leukocyte count was 17,600/cumm (64% neutrophils and 36% lymphocytes), and platelet count was 51000/mcL. Rest of the cells were immature and deformed.
Computed tomography (CT) of the orbit in axial section was done with coronal reconstruction. CT scan axial section revealed bilateral extraconal masses along the lateral orbital walls abutting the globes causing bilateral proptosis with extension of mass in right temporal fossa [Figure 1]. The mass in left orbit was abutting the lateral rectus muscle with loss of fat plane at places, likely to be involved by the mass. Preseptal swelling was also noted bilaterally. Coronal reconstructed CT images also show bilateral extraconal masses along with soft tissue density masses in bilateral maxillary and ethmoid sinuses [Figure 2]; however, there was no evidence of bony destruction.
|Figure 1: Axial computed tomography image showing bilateral extraconal masses along lateral orbital walls abutting the globes (large arrows) causing bilateral proptosis with extension of mass in right temporal fossa (small arrow)|
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|Figure 2: Coronal computed tomography image showing bilateral orbital masses, right temporal swelling and masses in bilateral maxillary and ethmoid sinuses|
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Gray scale ultrasonography (USG) of the eye revealed homogeneously hypoechoic masses with mild internal vascularity along the lateral wall of both orbits and in right temporal fossa region [Figure 3] and [Figure 4].
|Figure 3: Ultrasonography image showing left eye and homogeneously hypoechoic mass (arrow) in left extraconal space|
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|Figure 4: Ultrasonography image showing right eye and homogeneously hypoechoic mass (arrow) in right temporal fossa region|
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Fine needle aspiration cytology (FNAC) from the left orbital mass and right temporal swelling showed clusters of atypical cells with a high nuclear-cytoplasmic ratio. Bone marrow aspirate showed hypercellular marrow smears with blast cells proliferations (approximately 20-24%) and decreased erythroid cells and megakaryocytes [Figure 5]a and b. The findings were suggestive of AML. The patient was advised for chemotherapy/radiotherapy and referred to higher center.
|Figure 5: ( a) Giemsa stain and (b) Papanicolaou stain. cytological examination showing blast cells and atypical cells with high N/C ratio|
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The accumulation of leukemic cells infiltrating the extramedullary sites are known as granulocytic sarcomas; first described as "green tumor" by Allen Burns in 1811. Due to presence of myeloperoxidase enzyme in these leukemic cells, it appears green when exposed to ultraviolet light; however, this phenomenon may be absent in about one-third of the tumors.  Children and young adults are most frequently affected by AML; mean age is approximately 7 years.  AML is found in about 15% of all leukemia's in children.  Granulocytic sarcoma is a rare manifestation of AML and it is seen in approximately 3% of cases of AML.  They can involve skull, orbits, paranasal sinuses, sacrum, spine, sternum, and ribs. But granulocytic sarcoma presenting as an orbital mass in AML is very rare. 
The common differential diagnoses in this age group are dermoid, hemangioma, orbital cellulitis, orbital hematoma, orbital extension of retinoblastoma, etc., Less common diseases are rhabdomyosarcoma, metastatic neuroblastoma, leukemia, or lymphoma. In our patient there was bilateral orbital masses causing proptosis with extension of mass in right temporal region and paranasal sinuses without any bony destruction.
The presence of unilateral and bilateral proptosis has been reported with AML. , Proptosis is found predominantly due to leukemic infiltrates, retrobulbar hemorrhage, orbital muscle infiltration, or venous blockage. Ptosis, lacrimal gland involvement, conjuctival masses, iridic and diffuse uveal involvement are some other presentations of this disease.  Decreased visual acuity and restricted ocular movements have been reported in majority of the cases, contemporary to our patient in whom visual acuity was normal but with restricted ocular movements.
CT scan reveals soft tissue density masses, isodense or slightly hyperdense to muscles  with uniform homogeneous enhancement after administration of intravenous contrast medium, usually confined to the orbits but sometimes extends to the extradural temporal fossa and temporal and frontal regions. They may rarely extend to the paranasal sinuses as seen in our patient. On magnetic resonance imaging (MRI), masses appear hyperintense to muscle on T2-weighted images with homogenous enhancement on T1-weighted contrast enhanced images. Peripheral smear is an invaluable tool in diagnosing AML and it shows immature blast cells with a high total leukocyte count and relative neutropenia.
These tumors respond very well to chemotherapy, radiotherapy or both. , However, bone marrow transplantation is the best long-term treatment option for providing remission-free survival for most of the patients.  Prognosis is related to the course of the underlying hematologic malignancy.
Similar case has been reported by Gupta et al. in a 2-year-old child with bilateral proptosis and palpable mass in superomedial aspect of both orbits, diagnosed on aspiration cytology. Imaging studies were not done in their case. But in our case, aspiration cytology along with imaging studies (CT and USG) were done, which aids to assess the extent of the mass, involvement of the surrounding structures, and similar soft tissue masses in the paranasal sinuses.
In conclusion, child presenting with rapidly growing orbital mass or bilateral proptosis, AML should be kept in the differential diagnosis. For early diagnosis of AML, radiological imaging, peripheral blood smear along with bone marrow aspirate should be performed in all cases.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]