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Case Studies | Pathology | India | Volume 13 Issue 10, October 2024 | Popularity: 5.3 / 10
A Case Report on Primary Pigmented Nodular Adrenocortical Disease (PPNAD) Presenting as Cushing Syndrome in a 25 Yr Old Female
Dr. Praveena K P, Dr. Kalaranjini K. V. , Dr. Shashikiran M. S.
Abstract: Aim: To describe the case of primary pigmented nodular adrenocortical disease (PPNAD) presenting as cushing syndrome in a 25 year old female. Background: Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing's syndrome, which is adrenocorticotropic hormone-independent and involves both adrenal glands. The characteristic pathological features include multiple pigmented cortical nodules and atrophy of the internodular cortex. Case Vignette: A 25-year-old female presented with excessive weight gain with increased abdominal girth and abdominal striae. She is k/c/o ACTH independent cushing syndrome and hypertension. She underwent hemithyroidectomy 2 years back for papillary carcinoma thyroid. Treatment: Laparoscopic bilateral adrenalectomy under general anesthesia was done. The histopathology findings were consistent with PPNAD. Genetic studies for PRKAR1A, PDE11A gene was done and no mutation was detected. Conclusion: The early diagnosis and treatment allowing timely bilateral adrenalectomy will not only prevent severe complications of hypercortisolism, but may also preclude the development of adrenocortical carcinoma. The diagnosis of Carney complex can be made in our case as the patient has papillary carcinoma of thyroid and PPNAD.
Keywords: Cushing's syndrome, primary pigmented nodular adrenocortical disease
Edition: Volume 13 Issue 10, October 2024
Pages: 447 - 449
DOI: https://www.doi.org/10.21275/SR241004191751
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