PMC8284332_01
The ocular appearance shows swelling of the lateral superior part and the lower eyelid of the left eye.
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On ophthalmic examination, a mass on the lateral superior orbital rim of the left eye, with congestion and edema of the superotemporal conjunctiva and swelling of the lower eyelid, was detected (Figure 1).
The radiological findings of the 2-year-old girl with an orbital mass. (a) Orbital axial CT showing the soft tissue mass on the lateral orbital rim of the left eye; (b) orbital coronal CT in the bone window showing the soft tissue mass on the lateral superior orbital rim of the left eye, with bone destruction in the lateral wall of the left orbit.
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Orbital CT showed a soft tissue mass on the lateral superior orbital rim of the left eye with bone destruction in the lateral wall of the left orbit (Figure 2).
The gross picture shows two masses measuring approximately 2.5 cm x 2 cm x 1.5 cm and 2.5 cm x 1.5 cm x 1 cm in size.
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On a general observation, the two tumor masses measured approximately 2.5 cm x 2 cm x 1.5 cm and 2.5 cm x 1.5 cm x 1 cm (Figure 3).
Histopathological examination of the tumor. (a) Hematoxylin-eosin (H&E) staining results of the tumor showed neuroblasts at different degrees of differentiation arranged in nests and mature gangliocytes with fibrous connective tissue septa (H&E, original magnification, 200x); (b) the number of CD56-positive tumor cells was 175.7 +- 6.5 per 3 high power field (HPF) (CD56, original magnification, 200x); (c) immunohistochemical staining results were positive for NSE and the number of these tumor cells was 163.3 +- 5.6 per 3 HPF (NSE, original magnification, 200x); (d) the number of Syn-positive tumor cells was 172.1 +- 25.2 per 3 HPF (Syn, original magnification, 200x); (e) the number of NeuN-positive cells was 122.7 +- 11.6 per 3 HPF (NeuN, original magnification, 200x); (f) the proportion of Ki-67-positive cells was 2-5% per HPF (Ki-67, original magnification, 200x). Scale bar = 200 mum. ImageJ 1.50b with cell counter plugin (https://imagej.nih.gov/ij/) was used for cell counting following the online guide.
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Immunohistochemical staining results were positive for CD56, neuron-specific enolase (NSE), and synaptophysin (Syn), partly positive for neuron-specific nuclear protein (NeuN) and Ki-67 (2-5%), and negative for leukocyte common antigen (LCA), confirming a diagnosis of GNB (Figure 4).
Abdominal CT findings. (a) A plain scan showing a left adrenal tumor of approximately 1.5 cm x 2.2 cm x 2.7 cm size (black arrow); (b) a plain scan showing a calcified nodule on the left side of the abdominal aorta (black arrow); (c) an enhanced scan revealing left adrenal tumor with increased calcification inside (black arrow); (d) an enhanced scan showing the mesenteric lymph nodes (black arrow).
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During one month follow-up period after surgery, abdominal CT indicated a 1.5 cm x 2.2 cm x 2.7 cm mass with calcification in the left adrenal gland, a calcified nodule on the left side of the abdominal aorta, and several mesenteric lymph nodes (Figure 5).