Sporadic Coexistence of Parathyroid Adenoma, Papillary Thyroid Carcinoma, Pheochromocytoma, and Cardiac Myxoma: A Multidisciplinary Approach to an Extremely Rare Tumor Constellation

TL;DR

A 74-year-old woman had four rare tumors coexisting without a known genetic syndrome, requiring coordinated surgery and long-term monitoring.

Contribution

Reports a rare sporadic case of multiple endocrine and cardiac tumors managed through multidisciplinary care.

Findings

A patient presented with parathyroid adenoma, thyroid carcinoma, pheochromocytoma, and cardiac myxoma without hereditary syndrome.

Surgical sequencing and interdisciplinary coordination ensured safe treatment and confirmed diagnoses with no malignancy.

The case suggests a possible low-penetrance or undiagnosed hereditary syndrome due to the unusual delayed onset.

Abstract

A 74-year-old woman was referred for evaluation of asymptomatic hypercalcemia detected during a routine health examination. Laboratory testing confirmed primary hyperparathyroidism, with elevated serum calcium and intact parathyroid hormone (PTH) levels. Further diagnostic workup revealed a constellation of neoplasms: a parathyroid adenoma, papillary thyroid carcinoma, a right adrenal pheochromocytoma, a non-functional left adrenal cortical adenoma, and an incidental left atrial myxoma. There was no family history suggestive of hereditary tumor syndromes, and the patient declined genetic testing despite medical recommendation. A multidisciplinary team, including specialists in endocrinology, urology, otolaryngology, cardiology, cardiovascular surgery, and anesthesiology, collaborated to formulate a treatment strategy that minimized perioperative risks. Given the risk of hypertensive crisis, the right adrenal pheochromocytoma was resected first following adequate alpha-adrenergic blockade. This was followed by parathyroidectomy and left thyroid lobectomy with central neck dissection. Finally, to prevent potential embolic complications, the left atrial myxoma was excised via a transseptal approach under cardiopulmonary bypass. Histopathological examination confirmed the diagnoses of all tumors, with no evidence of malignant transformation or residual disease. This case illustrates a rare constellation of endocrine and cardiac tumors. Although the presentation raised clinical suspicion for hereditary syndromes such as multiple endocrine neoplasia (MEN) or Carney complex, the patient's advanced age, absence of mucocutaneous features, and negative family history favored a sporadic etiology. Nonetheless, the unusually delayed onset raises the possibility of a low-penetrance or as-yet-undescribed hereditary syndrome. Strategic surgical sequencing and interdisciplinary coordination were critical to ensuring patient safety and favorable clinical outcomes. Lifelong follow-up remains essential to monitor for recurrence and to reassess genetic risks as genomic technologies continue to evolve.

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