Bilateral proptosis and bitemporal swelling: A rare manifestation of acute myeloid leukemia

Abstract

Background: In Acute Myeloid Leukemia (AML), malignant clones of immature myeloid cells (primarily blasts) proliferate, replace bone marrow, circulate in blood and invade other tissues. The unique presentation of bilateral proptosis and bilateral temporal swelling in AML is being reported.

Case report: A 6-year-old girl presented with low-grade fever, progressively increasing bitemporal swelling and bilateral proptosis. Contrast Enhanced Computed Tomographic (CECT) images revealed enhancing infiltrates occupying the lateral orbital wall, causing proptosis. The infiltrate extended toward the bilateral temporal fossae beneath the temporalis muscle and extradurally beneath the frontal and temporal bones. A high total leucocytic count with immature and deformed cells and, Fine Needle Aspiration Cytology (FNAC) from the temporal swelling, the bone marrow aspirate and biopsy showing leukemic blast cells confirmed the diagnosis of AML. Chemotherapy brought about remission of the disease.

Conclusions: To the best of the authors' knowledge, simultaneous presence of both bilateral proptosis and bitemporal swellings have not been previously reported in AML. A peripheral blood smear with bone marrow aspirate and biopsy help in the early detection of AML. Institution of early intervention in this potentially fatal disease is often associated with gratifying survival rates.

Keywords: Acute myeloid leukemia; bitemporal swelling; chemotherapy; proptosis.

Figure 1

A 6-year-old female child showing bilateral proptosis and bitemporal swelling

Figure 2

Axial CECT showing enhancing infiltrates occupying the lateral orbital wall and causing proptosis. The infiltrate extended toward the bilateral temporal fossae beneath the temporalis muscle. There were extradural infiltrates extending bilaterally extradurally beneath the temporal bones

Figure 3

Axial CECT showing extradural infiltrates extending bilaterally beneath the frontal and temporal bones. On both sides, small lobules were extending into the cortex of the frontal lobes and causing perifocal edema

Figure 4

Coronal CT showed the left maxilla also infiltrated by the lesion

Figure 5

Bone marrow biopsy showing hypercellular marrow with sheets of blast cells (H & E, 40×)

Figure 6

FNAC from temporal swelling with hemorrhagic background showing blast cells (May Grunwald Geimsa stain, 20×)

Figure 7

FNAC from temporal swelling showing clumped blast cells with an occasional signal blast cell (May Grunwald Geimsa stain, 20×)

Figure 8

Peripheral blood smear showing blast cells (Giemsa stain, 40×)