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Case Reports
. 2021 Jan-Mar;65(1):85-88.
doi: 10.22336/rjo.2021.17.

Acute unilateral proptosis in childhood: suspect myeloid sarcoma

Singh Manpreet  1 , Snehi Sagarika  1 , Rastogi Pulkit  2 , Jayakumar Kalaivani  1 , Kaur Manpreet  1 , Gupta Pankaj  1
Affiliations
Case Reports

Acute unilateral proptosis in childhood: suspect myeloid sarcoma

Singh Manpreet et al. Rom J Ophthalmol. 2021 Jan-Mar.

Abstract

As the first and only presenting feature of acute myeloid leukemia (AML), unilateral proptosis in children is uncommon. We report the cases of two girls who had no systemic clinical manifestations of AML. Orbital imaging showed space-occupying infiltrating lesions without surrounding bone erosion. Incisional biopsy and immunohistochemistry were diagnostic for myeloid sarcoma. Systemic workup and bone marrow examination showed features of AML. Systemic chemotherapy was administered to both children, who responded well to the treatment. Myeloid sarcoma should be kept in the differentials of the children presenting with isolated proptosis. Immunohistochemistry may provide an accurate diagnosis and early treatment may lead to a prompt recovery with a good prognosis.

Keywords: acute myeloid leukemia; acute proptosis; childhood proptosis; myeloid sarcoma; proptosis.

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Figures

Fig. 1
Fig. 1
a Primary gaze - OD shows inferior dystopia with conjunctival congestion; b. The lateral view shows supero-temporal sulcus fullness, proptosis, and conjunctival congestion c. MRI (axial, T2W, superior) shows a hyperintense soft-tissue lesion in supero-temporal orbit (lacrimal gland region) with extension into posterior orbit; d. MRI (coronal, posterior) soft-tissue lesion in the extraconal compartment with an infero-medial displacement of orbital contents i.e., extraocular muscles
Fig. 2
Fig. 2
a (H&E, 100x) Small round-blue tumor cells arranged in diffuse sheets, cells having fine round-oval nuclei with fine chromatin, prominent nucleoli, and moderate cytoplasm; b,c. IHC showing positivity for myeloperoxidase (MPO) and CD45; d. Bone marrow aspirate showing blast cells with premature configuration
Fig. 3
Fig. 3
a Multiplex PCR showing positive reaction at 395 base pair (red arrow); b. Wound healthy, suture intact with increased proptosis, chemosis and cornea protected with antibiotic ointment; c. Marked improvement is seen after the completion of chemotherapy
Fig. 4
Fig. 4
a Case 2- OS shows marked proptosis with superior globe displacement and conjunctival congestion; b. Significant improvement after 3 months of chemotherapy
See this image and copyright information in PMC

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