Carney Complex Complicated with Primary Pigmented Nodular Adrenocortical Disease without Cushing's Syndrome Recurrence for Five Years after Unilateral Adrenalectomy.

Abstract

We herein report a case of Carney complex (CNC) complicated with primary pigmented nodular adrenocortical disease (PPNAD) after unilateral adrenalectomy. A 44-year-old woman was admitted to our hospital for PPNAD surgery. She had previously undergone surgery for cardiac myxoma and had a PRKAR1A mutation with no family history of CNC. She had Cushing's signs, but her metabolic abnormalities were mild. Adrenal insufficiency due to poor medication adherence was a concern, so she underwent unilateral adrenalectomy. Cushing's signs improved postoperatively and without recurrence for five years. Treatment plans for PPNAD should be determined based on the patient's condition, medication adherence, and wishes.

• Authors: Yuya Tsurutani, Kanako Kiriyama, Mai Kondo, Masanori Hasebe, Akira Sata, Yuzo Mizuno, Chiho Sugisawa, Jun Saito, Tetsuo Nishikawa
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 61 Issue 2 Pg. 205-211 ( 2022) ISSN: 1349-7235 [Electronic] Japan
• PMID: 35034934 (Publication Type: Case Reports, Journal Article)

Chemical References

• Cyclic AMP-Dependent Protein Kinase RIalpha Subunit

Topics

• Adrenal Cortex Diseases (genetics, surgery)
• Adrenalectomy
• Adult
• Carney Complex (genetics, surgery)
• Cushing Syndrome (etiology, surgery)
• Cyclic AMP-Dependent Protein Kinase RIalpha Subunit (genetics)
• Female
• Humans