Case report: Coexistence of Conn's adenoma and non-secreting pheochromocytoma in the same adrenal gland

The association between Conn's adenoma and pheochromocytoma is extremely rare; only a few cases have been described. A 52-year-old female with a history of hypertension and diabetes mellitus was referred for primary hyperaldosteronism and a right adrenal mass. Laboratory tests showed normal serum potassium, a high aldosterone-renin ratio, normal levels of plasma free metanephrine, and confirmed ACTH-dependent Cushing's syndrome. An adrenal scan indicated a heterogeneous right adrenal nodule with microcalcifications, measuring 38.5 × 29.5 mm, with a spontaneous density of 25 HU, an absolute washout of 20%, and a relative washout of 16%. A pituitary MRI showed a constitutionally enlarged pituitary compartment without visualization of a microadenoma. Additional investigations revealed a papillary thyroid carcinoma with normocalcemic primary hyperparathyroidism. Initially, a total thyroidectomy with lymph node dissection was performed, which was complicated by postoperative hypocalcemia. Subsequently, a right adrenalectomy was performed. Histology confirmed the presence of both Conn's adenoma and pheochromocytoma with positive immunohistochemistry for chromogranin A. Although immunohistochemistry for ACTH was not available, a positive dexamethasone suppression test after adrenalectomy suggests that ACTH was secreted by the resected mass. This is the first reported case of coexisting Conn's adenoma and non-secreting pheochromocytoma. The simultaneous presence of these two neuroendocrine tumors may be coincidental or may indicate a genetic predisposition to endocrine dysplasia, or an interaction between the adjacent medullary and cortical tissues. The possibility of a common etiology for these two endocrine tumors is also considered.

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Journal
Annales d Endocrinologie
Published
2024-09-30
DOI
https://doi.org/10.1016/j.ando.2024.08.597
Primary Topic
Adrenal and Paraganglionic Tumors
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article
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article

Case report: Coexistence of Conn's adenoma and non-secreting pheochromocytoma in the same adrenal gland

L. Merabet, S. Labter, M.M. Benghani, L. Lakehal et al.
Annales d Endocrinologie
Adrenal and Paraganglionic Tumors
article

Case report: Coexistence of Conn's adenoma and non-secreting pheochromocytoma in the same adrenal gland

L. Merabet, S. Labter, M.M. Benghani, L. Lakehal, N. Goumri, H. Sekkat, A. Sadouki, M. Derradji, Mohammed El Amine Amani
article en

Abstract

The association between Conn's adenoma and pheochromocytoma is extremely rare; only a few cases have been described. A 52-year-old female with a history of hypertension and diabetes mellitus was referred for primary hyperaldosteronism and a right adrenal mass. Laboratory tests showed normal serum potassium, a high aldosterone-renin ratio, normal levels of plasma free metanephrine, and confirmed ACTH-dependent Cushing's syndrome. An adrenal scan indicated a heterogeneous right adrenal nodule with microcalcifications, measuring 38.5 × 29.5 mm, with a spontaneous density of 25 HU, an absolute washout of 20%, and a relative washout of 16%. A pituitary MRI showed a constitutionally enlarged pituitary compartment without visualization of a microadenoma. Additional investigations revealed a papillary thyroid carcinoma with normocalcemic primary hyperparathyroidism. Initially, a total thyroidectomy with lymph node dissection was performed, which was complicated by postoperative hypocalcemia. Subsequently, a right adrenalectomy was performed. Histology confirmed the presence of both Conn's adenoma and pheochromocytoma with positive immunohistochemistry for chromogranin A. Although immunohistochemistry for ACTH was not available, a positive dexamethasone suppression test after adrenalectomy suggests that ACTH was secreted by the resected mass. This is the first reported case of coexisting Conn's adenoma and non-secreting pheochromocytoma. The simultaneous presence of these two neuroendocrine tumors may be coincidental or may indicate a genetic predisposition to endocrine dysplasia, or an interaction between the adjacent medullary and cortical tissues. The possibility of a common etiology for these two endocrine tumors is also considered.

Annales d EndocrinologieVol. 85(5)
Université Oran 1 Ahmed Ben Bella (DZ), Emir Abdelkader University of Islamic Sciences (DZ), University Hospital of Oran (DZ)
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Adrenal and Paraganglionic Tumors
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