PMC9538310_01
Appearance of the patient, adrenal CT, postoperative gross, HE and histochemical staining. (A) Appearance and lower abdomen purple streaks; (B, C) Lip mucosa and multiple skin spot-like pigmentation; (D) Posterior neck skin myxoma; (E) Adrenal CT: bilateral adrenal nodules, the left side diameter is 1.9 cm, the enhanced CT value is approximately 40 HU; (F, G) Postoperative gross after left adrenalectomy: The diseased tissue is grayish-yellow-gray-brown, 4.5 2.5 2 cm, multinodular, some cells have pigmentation, and the surrounding cortex is squeezed and thinned; (H) After hematoxylin-eosin staining, brown are seen particles  400; (I-L) Immunohistochemistry of SYN ( 200), Inhibin-alpha ( 200), CYP11B1and CYP11B2 ( 400). CT, Computed tomography; HE, hematoxylin-eosin.
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A physical examination at the time of admission revealed bruising at the elbow skin, purple streaks on the lower abdomen ( Figure 1A ), spotty pigmentation of the lip mucosa and multiple skin areas ( Figures 1B, C ), skin myxoma of the posterior neck ( Figure 1D ), and no thyroid enlargement. Enhanced scanning showed uniform enhancement ( Figure 1E ). The pathological diagnosis was primary pigmented nodular adrenocortical disease (PPNAD) after left adrenalectomy ( Figures 1F-L ), postoperative gross, hematoxylin-eosin (HE), and histochemical staining).
Sanger sequencing showed the patient's C.439 A>G mutation in exon 4 of the PRKAR1A gene.
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A>G) of the PRKAR1A gene ( Figure 2 ). A diagnosis of CNC was finally made.