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Carney complex presenting with a unilateral adrenocortical nodule: a case report.

Talaei A, Aminorroaya A, Taheri D, Mahdavi KN - J Med Case Rep (2014)

Bottom Line: A 27-year-old Iranian woman was referred to our endocrinology clinic with amenorrhea and hirsutism, further confirming a diagnosis of adrenocorticotropic hormone-independent Cushing's syndrome.Pituitary magnetic resonance imaging also documented a microadenoma that was considered to be an incidentaloma based on normal pituitary function tests.Rarely, adrenal and pituitary imaging can be misleading.

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Affiliation: Thyroid Disorders Research Center, Arak University of Medical Science, Arak, Iran. talaei@arakmu.ac.ir.

ABSTRACT

Introduction: Carney complex is an autosomal dominant syndrome with multiple neoplasms in different sites, including myxomas, endocrine tumors and lentigines lesions. To the best of our knowledge, this is the first report of Carney complex presenting with a unilateral adrenal adenoma associated with a pituitary incidentaloma.

Case presentation: A 27-year-old Iranian woman was referred to our endocrinology clinic with amenorrhea and hirsutism, further confirming a diagnosis of adrenocorticotropic hormone-independent Cushing's syndrome. The cause was believed to be a right adrenocortical adenoma based on a computed tomography scan. Our patient underwent a right laparoscopic adrenalectomy and pathological examination revealed pigmented micronodular adrenal hyperplasia. Pituitary magnetic resonance imaging also documented a microadenoma that was considered to be an incidentaloma based on normal pituitary function tests. Recurrence of hypercortisolism led to a left laparoscopic adrenalectomy, providing further evidence for the diagnosis of primary pigmented nodular adrenocortical disease. Carney complex was established in light of her history of cardiac myxomas.

Conclusion: We present what we believe to be the first case of Carney complex presenting with a unilateral adrenocortical adenoma in association with a pituitary incidentaloma. Although primary pigmented nodular adrenocortical disease is rare as a component of Carney complex, it should be considered in the differential diagnosis of Cushing's syndrome. Rarely, adrenal and pituitary imaging can be misleading.

No MeSH data available.


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Pathology of the right adrenal adenoma (first operation; large cortical cells with granular eosinophilic cytoplasm containing small nodules of lipofuscin pigments; hematoxylin and eosin stain ×40).
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Figure 5: Pathology of the right adrenal adenoma (first operation; large cortical cells with granular eosinophilic cytoplasm containing small nodules of lipofuscin pigments; hematoxylin and eosin stain ×40).

Mentions: Brain magnetic resonance imaging showed that our patient had a microadenoma (measuring 8×6mm) (Figures 1 and2) and left parietal infarction (Figure 3). A 15×10mm adenoma of her right adrenal gland was identified in adrenal computed tomography (CT) (Figure 4), whereas her left adrenal gland appeared normal. The hormonal and radiological findings led to a diagnosis of ACTH-independent CS caused by an adrenocortical adenoma of her right adrenal gland. A right laparoscopic adrenalectomy was therefore performed. Pathological examination revealed cortical cell hyperplasia containing small nodules composed of lipofuscin pigment (Figure 5). Postoperative follow-up did not show any improvement in her hypertension or other symptoms. Her plasma cortisol level (12μg/dL) was not suppressed after repeated low dose dexamethasone suppression tests, so a left laparoscopic adrenalectomy was performed and pathological examination revealed the same cortical cell hyperplasia as found in her right adrenal gland (Figure 6). Bilateral adrenal hyperplasia with small pigmented cortical nodules established the diagnosis of PPNAD.


Carney complex presenting with a unilateral adrenocortical nodule: a case report.

Talaei A, Aminorroaya A, Taheri D, Mahdavi KN - J Med Case Rep (2014)

Pathology of the right adrenal adenoma (first operation; large cortical cells with granular eosinophilic cytoplasm containing small nodules of lipofuscin pigments; hematoxylin and eosin stain ×40).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3927851&req=5

Figure 5: Pathology of the right adrenal adenoma (first operation; large cortical cells with granular eosinophilic cytoplasm containing small nodules of lipofuscin pigments; hematoxylin and eosin stain ×40).
Mentions: Brain magnetic resonance imaging showed that our patient had a microadenoma (measuring 8×6mm) (Figures 1 and2) and left parietal infarction (Figure 3). A 15×10mm adenoma of her right adrenal gland was identified in adrenal computed tomography (CT) (Figure 4), whereas her left adrenal gland appeared normal. The hormonal and radiological findings led to a diagnosis of ACTH-independent CS caused by an adrenocortical adenoma of her right adrenal gland. A right laparoscopic adrenalectomy was therefore performed. Pathological examination revealed cortical cell hyperplasia containing small nodules composed of lipofuscin pigment (Figure 5). Postoperative follow-up did not show any improvement in her hypertension or other symptoms. Her plasma cortisol level (12μg/dL) was not suppressed after repeated low dose dexamethasone suppression tests, so a left laparoscopic adrenalectomy was performed and pathological examination revealed the same cortical cell hyperplasia as found in her right adrenal gland (Figure 6). Bilateral adrenal hyperplasia with small pigmented cortical nodules established the diagnosis of PPNAD.

Bottom Line: A 27-year-old Iranian woman was referred to our endocrinology clinic with amenorrhea and hirsutism, further confirming a diagnosis of adrenocorticotropic hormone-independent Cushing's syndrome.Pituitary magnetic resonance imaging also documented a microadenoma that was considered to be an incidentaloma based on normal pituitary function tests.Rarely, adrenal and pituitary imaging can be misleading.

View Article: PubMed Central - HTML - PubMed

Affiliation: Thyroid Disorders Research Center, Arak University of Medical Science, Arak, Iran. talaei@arakmu.ac.ir.

ABSTRACT

Introduction: Carney complex is an autosomal dominant syndrome with multiple neoplasms in different sites, including myxomas, endocrine tumors and lentigines lesions. To the best of our knowledge, this is the first report of Carney complex presenting with a unilateral adrenal adenoma associated with a pituitary incidentaloma.

Case presentation: A 27-year-old Iranian woman was referred to our endocrinology clinic with amenorrhea and hirsutism, further confirming a diagnosis of adrenocorticotropic hormone-independent Cushing's syndrome. The cause was believed to be a right adrenocortical adenoma based on a computed tomography scan. Our patient underwent a right laparoscopic adrenalectomy and pathological examination revealed pigmented micronodular adrenal hyperplasia. Pituitary magnetic resonance imaging also documented a microadenoma that was considered to be an incidentaloma based on normal pituitary function tests. Recurrence of hypercortisolism led to a left laparoscopic adrenalectomy, providing further evidence for the diagnosis of primary pigmented nodular adrenocortical disease. Carney complex was established in light of her history of cardiac myxomas.

Conclusion: We present what we believe to be the first case of Carney complex presenting with a unilateral adrenocortical adenoma in association with a pituitary incidentaloma. Although primary pigmented nodular adrenocortical disease is rare as a component of Carney complex, it should be considered in the differential diagnosis of Cushing's syndrome. Rarely, adrenal and pituitary imaging can be misleading.

No MeSH data available.


Related in: MedlinePlus