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Diagnostic and operative problems in multiple pheochromocytomas.

E S Heikkinen, H K Akerblom

    Journal of Pediatric Surgery
    |April 1, 1977
    PubMed
    Summary
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    This study presents two pediatric cases of multiple pheochromocytomas, highlighting diagnostic challenges and successful preoperative management with chlorpromazine. One patient exhibited a rare pattern of multiple endocrine adenomatosis syndrome.

    Area of Science:

    • Pediatric Endocrinology
    • Oncology
    • Genetics

    Background:

    • Pheochromocytomas are rare neuroendocrine tumors, often associated with genetic syndromes.
    • Multiple endocrine neoplasia (MEN) syndromes can present with complex tumor patterns.

    Observation:

    • Two 14-year-old patients with multiple pheochromocytomas and positive family histories were studied.
    • Preoperative aortography visualized intra-adrenal tumors but missed extra-adrenal ones in one case.
    • One patient presented with bilateral adrenal pheochromocytomas, islet cell adenomas, and cholecystolithiasis.

    Findings:

    • Chlorpromazine effectively managed preoperative adrenergic blockade in both patients.
    • Postoperative catecholamine levels indicated residual pheochromocytoma in the first case.

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  • The second patient, after surgery, developed hyperparathyroidism and signs suggestive of medullary thyroid carcinoma, indicating a unique multiple endocrine adenomatosis (MEA) pattern.

    • Implications:

    • Early diagnosis and comprehensive genetic screening are crucial for pediatric pheochromocytoma management.
    • Adrenergic blockade is vital for safe surgical preparation.
    • This case underscores the diverse and complex presentations of MEA syndromes, necessitating long-term surveillance.