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Year : 2020  |  Volume : 58  |  Issue : 4  |  Page : 293-295

Bilateral proptosis as initial manifestation of granulocytic sarcoma in a child

1 Department of Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Medical Research Foundation, Chennai, Tamil Nadu, India
2 Department of Pediatric Oncology, Apollo Hospitals, Chennai, Tamil Nadu, India

Date of Submission31-Jul-2020
Date of Acceptance21-Sep-2020
Date of Web Publication16-Dec-2020

Correspondence Address:
Dr. Bipasha Mukherjee
Orbit, Oculoplasty, Reconstructive & Aesthetic Services, Medical Research Foundation Sankara Nethralaya 18, College Road, Chennai - 600 006, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_100_20

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Proptosis in the pediatric age-group can augur an ominous underlying pathology. These patients should be investigated promptly and thoroughly to rule out life-threatening disorders such as leukemia. This can mean the difference between life and death. Granulocytic sarcoma, earlier known as chloroma or extramedullary myeloblastoma is usually seen in patients with acute myeloid leukemia (AML). It can either precede or emerge in conjunction with the systemic disease. One of the extramedullary locations is the orbits, more so in children. Here, we present a case of bilateral proptosis in a child due to extramedullary deposits of AML. Despite the best possible treatment measures, she succumbed to her disease process 5 months from the presentation.

Keywords: Granulocytic sarcoma, leukemia, pediatric, proptosis

How to cite this article:
Mukherjee B, Shrirao N, Ramesh A. Bilateral proptosis as initial manifestation of granulocytic sarcoma in a child. TNOA J Ophthalmic Sci Res 2020;58:293-5

How to cite this URL:
Mukherjee B, Shrirao N, Ramesh A. Bilateral proptosis as initial manifestation of granulocytic sarcoma in a child. TNOA J Ophthalmic Sci Res [serial online] 2020 [cited 2023 Jan 28];58:293-5. Available from: https://www.tnoajosr.com/text.asp?2020/58/4/293/303664

Granulocytic sarcoma (GS), earlier known as chloroma or extramedullary myeloblastoma, is usually seen in patients with acute myeloid leukemia (AML). It can either precede or emerge in conjunction with the systemic disease. One of the extramedullary locations is the orbits, more so in children.[1],[2]

A 6-year-old female child presented to our emergency department with complaints of progressive prominence of both eyes since the past 2 months associated with pain and diminution of vision for the last 15 days [Figure 1]a. The parents gave a history of weight loss, high-grade fever with rash and associated with joint pain for the last 6 months. There was no history of trauma, surgery, dental/sinus infection. On examination, she could perceive light in her right eye and able to count fingers close to face in the left. The right eye showed relative afferent pupillary defect and left eye pupil was sluggishly reacting; findings that indicated optic nerve dysfunction in both the eyes. Ocular motility was limited in all gazes. Measurement with Hertel's exophthalmometer revealed a reading of 36 mm in the right eye and 28 mm in the left eye with base 90. Both eyes had severe conjunctival chemosis and the right eye showed damage to the cornea due to exposure resulting from incomplete eye closure. The retinal examination was unremarkable. On systemic examination, enlarged submandibular, cervical, and supraclavicular lymph nodes were seen on both sides. The child was listless and irritable.
Figure 1: (a) Child at presentation showing bilateral severe proptosis (b) T2.weighted Magnetic resonance imaging images of the patient revealing homogenously hypointense masses in bilateral superior orbits (c) soft, friable, whitish, pink mass seen during incisional biopsy (d) H and E, ×40 showing auer rods (arrow) (e) child showing resolution of proptosis after chemotherapy

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A peripheral blood smear (PBS) revealed neutrophilia. Magnetic resonance imaging (MRI) of the orbits was requested which revealed diffuse T1 hypointense and T2 isointense lesions bilaterally in the extraconal space [Figure 1]b. Similar lesions were also seen in the infratemporal fossa, maxilla, and ramus of the mandible [Figure 1]b. Suspecting leukemic deposits, an urgent incisional biopsy was performed. Soft, friable, whitish, pink mass seen during incisional biopsy was seen [Figure 1]c. Histopathological examination showed a dense collection of malignant round cells with scattered lymphocytes and tingible body macrophages with a starry sky pattern [Figure 1]d. The definitive diagnosis of AML was established by bone marrow aspiration biopsy which revealed the presence of multiple blast cells showing Auer rods. Computed tomography (CT) chest and abdomen were normal and cerebrospinal fluid cytology showed the absence of central nervous system spread. She was started on the induction phase of chemotherapy with daunorubicin and cytarabine and consolidation phase with cytarabine alone. Her ocular and orbital condition improved dramatically after 4 cycles of chemotherapy. Her vision in both eyes improved to counting fingers at 3 m and exophthalmometry showed a reading of 18 mm in both the eyes [Figure 1]e. However, despite all measures, the child succumbed to the disease within 5 months of the presentation.

In children, AML accounts for 15% of all leukemias.[3] GS is a solid tumor comprising of primitive precursors of the granulocytic series of white blood cells such as myeloblasts, promyelocytes, and myelocytes which has been reported in 2.5%–9.1% of patients with AML.1 It is seen most commonly in children and young adults.[2] Although most commonly seen in AML, it can also be found in patients with, chronic myelogenous leukemia, myelodysplastic syndromes, and rarely acute lymphoblastic leukemia.[3] The incidence of orbital GS in AML is reportedly between 9.3 and 36%.[4]

Unilateral painful proptosis and lid swelling with visual loss, ocular motility limitation, and diplopia are the common presenting features of orbital GS. Only a few cases of bilateral GS have been reported.[5],[6],[7],[8] Imaging studies reveal intraconal or extraconal, homogeneous, well-defined orbital masses that mold to the bone and contiguous structures. The density of lesions is variable on CT, whereas on MRI they are hypo or iso-intense on T1-weighted and iso or hyper-intense on T2-weighted images, and homogeneously enhance on contrast.[9] Due to an overlap in clinical and radiological features, GS can be confused with orbital cellulitis, rhabdomyosarcoma, neuroblastoma, or lymphoma. PBS, done empirically in these patients, can be life-saving, as 72%–88% of the patients' GS develops before the systemic disease.[1],[10] Since the smear failed to reveal immature cells in our patient, an orbital biopsy was done, which was equivocal. Bone marrow aspiration revealed blast cells with Auer rods and clinched the diagnosis.

Chemotherapy, commonly with cytarabine and daunorubicin is the treatment modality of choice. Radiotherapy has also been tried in cases where chemotherapy has failed.[1] Median survival is reported to be 6–14 months for patients with GS with AML.[11]

We conclude that acute proptosis in a child can be challenging for the general ophthalmologist. They should consider leukemia in the differential of similar clinical presentation, as prompt referral to an oncologist, and early institution of therapy can prolong survival.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Aggarwal E, Mulay K, Honavar SG. Orbital extra-medullary granulocytic sarcoma: Clinicopathologic correlation with immunohistochemical features. Surv Ophthalmol 2014;59:232-5.  Back to cited text no. 1
Stockl FA, Dolmetsch AM, Saornil MA, Font RL, Burnier MN Jr. Orbital granulocytic sarcoma. Br J Ophthalmol 1997;81:1084-8.  Back to cited text no. 2
Thakur B, Varma K, Misra V, Chauhan S. Granulocytic sarcoma presenting as an orbital mass: Report of two cases. J Clin Diagn Res 2013;7:1704-6.  Back to cited text no. 3
Sethi A, Ghose S, Gujral S, Jain P, Kumar R. Childhood proptosis: The invaluable but overlooked peripheral blood smear. Indian J Ophthalmol 2001;49:121-3.  Back to cited text no. 4
[PUBMED]  [Full text]  
Chaudhry IA, Alaraj AM, Alkatan HM. Unilateral eyelid swelling, proptosis and diplopia as initial manifestation of acute myeloid leukemia. Saudi J Ophthalmol 2012;26:241-4.  Back to cited text no. 5
Takhenchangbam DS, Laishram RS, Thoudem TS, Sunita A, Imchen LT. Proptosis and facial palsy as an unusual clinical presentation of acute myeloid leukemia. Iran J Cancer Prev 2013;6:52-4.  Back to cited text no. 6
Michelson JB, Shields JA, Leonard BC, Caputo AR, Bergman GE: Periorbital chloroma and proptosis in a two-year old with acute myelogenous leukemia. J Pediatr Ophthalmol 1975;12:255-8.  Back to cited text no. 7
Shields JA, Stopyra GA, Marr BP, Shields CL, Pan W, Eagle RC Jr., et al. Bilateral orbital myeloid sarcoma as initial sign of acute myeloid leukemia: Case report and review of the literature. Arch Ophthalmol 2003;121:138-42.  Back to cited text no. 8
Noh BW, Park SW, Chun JE, Kim JH, Kim HJ, Lim MK. Granulocytic sarcoma in the head and neck: CT and MR imaging findings. Clin Exp Otorhinolaryngol 2009;2:66-71.  Back to cited text no. 9
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;80:975-90.  Back to cited text no. 10
Yamauchi K, Yasuda M. Comparison in treatments of nonleukemic granulocytic sarcoma: Report of two cases and a review of 72 cases in the literature. Cancer 2002;94:1739-46.  Back to cited text no. 11


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