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Primary pigmented nodular adrenocortical dysplasia

G Köhler¹, K Anding, N Böhm

¹Department of Pathology, University Freiburg, Germany.

Pathology, Research and Practice

|May 20, 1998

Summary

This study reports a case of Cushing's syndrome caused by primary pigmented nodular adrenocortical dysplasia (PPNAD). The patient's history of melanoma suggests a possible link to Carney complex.

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Area of Science:

Endocrinology
Oncology
Genetics

Background:

Cushing's syndrome is a hormonal disorder caused by prolonged exposure to high cortisol levels.
Adrenocortical neoplasms can cause Cushing's syndrome, but imaging may not always reveal the cause.
Primary pigmented nodular adrenocortical dysplasia (PPNAD) is a rare cause of Cushing's syndrome.

Observation:

A 29-year-old female presented with Cushing's syndrome, but MRI showed normal adrenal glands.
Diagnostic adrenalectomy revealed nodules consistent with PPNAD.
The patient had a prior history of malignant melanoma.

Findings:

Microscopic examination confirmed PPNAD, characterized by enlarged cortical cells with eosinophilic cytoplasm and nuclear pleomorphism.
The patient underwent a second adrenalectomy for curative treatment.
The findings suggest PPNAD may be linked to Carney complex, especially given the patient's melanoma history.

Implications:

This case highlights the diagnostic challenges in PPNAD and Cushing's syndrome.
It underscores the potential association between PPNAD, Carney complex, and melanoma.
Further research into the genetic basis of PPNAD, possibly involving chromosome 16, is warranted.

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