PMC8127780_01
Patient's appearance upon visitation to Pediatric Nephrology Clinic, Cipto Mangunkusumo Hospital. He was undernourished (BMI 16.5 kg/m2) with normal stature. No skin lesions were noted.
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His BMI was 16.5 kg/m2 (undernourished with normal stature), and no skin lesions were noted (Figure 1).
Magnetic resonance imaging (MRI) demonstrating a circular, well-defined mass with heterogeneous high signal intensity on right suprarenal. Enhancement was seen post-contrast. (a) Pre-contrast view. (b) Post-contrast view. 131I-meta-iodobenzylguanidine (MIBG) results. (c) Anterior and posterior (24 hours and 48 hours) planar whole body images of 131I-MIBG scintigraphy showed pathological uptake in the right adrenal tumor (as seen on MRI) compatible with pheochromocytoma. No other pathological uptake considered as metastases were seen. (d) 131I-MIBG SPECT/CT image showed focal pathological uptake in the right adrenal mass as seen on planar images.
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Simultaneously, the patient underwent abdominal magnetic resonance imaging (MRI) (Figure 2a and b) and 131I-meta-iodobenzylguanidine (MIBG) scintigraphy (Figure 2c and d) were ordered. 131I-MIBG scintigraphy demonstrated the uptake of pathological tracer in the right suprarenal region corresponding to an adrenal tumor without metastatic lesions.
Macroscopic appearance of the resected tumor and pathology results. (a) A 5.5 cm tumor was shown after resection. (b) On cross section it appeared glistening tan-white with part of tan-brown surface. Nodular area due to vascular channels cut in various planes is shown by black arrow. An adrenal remnant is attached (white arrow). (c) Pheochromocytoma is circumscribed but unencapsulated. The tumor obliterates the adrenal cortex and expands to the capsule of the adrenal (white arrow). Periadrenal brown adipose tissue is noted (black arrow) (hematoxylin-eosin (HE), original magnification x40). (d) Classic architecture with prominent alveolar pattern or cell nest (Zellballen) is shown with black arrow. Nests consist of polygonal tumor cells separated by peripheral capillaries (HE, original magnification x100). (e) The tumor has solid and diffuse pattern consisted of cells with prominent nucleoli with typical numerous basophilic granules in the cytoplasms (HE, original magnification x400). (f) Positive chromogranin A in cytoplasm of tumor cells (immunohistochemistry stain, original magnification x400). (g) Positive CD 34 stain in vascular accentuates the alveolar pattern and zellballen formation (immunohistochemistry stain, original magnification x100).
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Surgery was performed using the posterior retroperitoneoscopic right adrenalectomy approach for removing the tumor (Figure 3a and b). The results of pathological examination suggesting pheochromocytoma are presented in Figure 3c to g.