PMC9789786_01
PMC9789786_02
intra-operative picture of retroperitoneal paraganglioma close to the abdominal aorta, encasing the proximal part of the left ureter and causing proximal hydroureteronephrosis
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F1
Abdominal ultrasound revealed left hydrocalicosis and intravenous urography showed prompt excretion of contrast on the right but delayed nephrogram on the left with clubbing and hydronephrosis (Figure 1). A retroperitoneal mass was missed at ultrasonography, and a working diagnosis of left congenital ureteropelvic junction obstruction with hypertension was made.
cut section of paraganglioma revealing an irregular greyish brown lobulated soft tissue tumor measuring 8 x 6.5 x 4cm
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F2
Therapeutic intervention: during surgery for left pyeloplasty via the flank approach, an incidental finding of a left retroperitoneal mass (paraganglioma) close to the abdominal aorta, encasing the proximal left ureter was discovered and converted to a midline transperitoneal approach which made complete excision of the mass easier (Figure 2, Figure 3).
hematoxylin and eosin stain with x 40 magnification showing the paraganglioma depicting the typical cuboidal nests of cells called zellballen, separated by vascularized fibrous stroma showing regular nuclei and few patches of pleomorphism with focal areas of degeneration
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F3
Therapeutic intervention: during surgery for left pyeloplasty via the flank approach, an incidental finding of a left retroperitoneal mass (paraganglioma) close to the abdominal aorta, encasing the proximal left ureter was discovered and converted to a midline transperitoneal approach which made complete excision of the mass easier (Figure 2, Figure 3). Diagnostic assessment: surgical pathology showed an irregular greyish brown lobulated soft tissue tumor (paraganglioma) measuring 8x6.5x4cm (Figure 3).
positivity to chromogranin on immunohistochemistry staining confirms the diagnosis of paraganglioma
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F4
The cells showed regular nuclei and few pleomorphic cells with focal areas of degenerative changes (Figure 4).
tumour showing positivity for neuron-specific enolase (chief cells) consistent with paraganglioma
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F5
Immunohistochemistry staining of the tumor was negative for cytokeratin (CK7) but positive on the urothelium, and it also showed high Chromogranin positivity (Figure 5).
tumour showing positivity for S-100 protein (sustentacular cells) consistent with paraganglioma
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F6
Additionally, the tumor exhibited positivity for neuron-specific enolase NSE (chief cells) and S-100 protein (sustentacular cells) all consistent with the diagnosis of paragangliomas (Figure 6).