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Bilateral pheochromocytomas.

V Kalff, B Shapiro, R Lloyd

    Journal of Endocrinological Investigation
    |August 1, 1984
    PubMed
    Summary
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    Isolated bilateral pheochromocytomas, occurring without genetic syndromes, present in younger patients with more aggressive disease. These rare cases highlight distinct clinical and pathological features compared to syndromic forms.

    Area of Science:

    • Endocrinology
    • Oncology
    • Genetics

    Background:

    • Bilateral pheochromocytomas are typically associated with genetic syndromes like multiple endocrine neoplasia (MEN) types 2a and 2b, von Hippel-Lindau (VHL) disease, and neurofibromatosis (NF).
    • These syndromes often present with characteristic physical stigmata and a positive family history, guiding diagnosis.

    Observation:

    • This study details four patients with isolated bilateral pheochromocytomas, lacking syndromic features or family history.
    • These patients were compared to fourteen individuals with syndromic bilateral pheochromocytomas.

    Findings:

    • Patients with isolated bilateral pheochromocytomas were significantly younger (10-14 years) and exhibited more rapidly progressing disease.
    • Three of the four isolated cases showed adrenal medullas entirely replaced by tumor, unlike the usual hyperplasia seen in syndromic cases.

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  • Malignant transformation occurred in both a non-syndromic and a VHL-syndrome patient.

    • Implications:

    • Isolated bilateral pheochromocytomas represent a distinct clinical entity with potentially more aggressive behavior and unique pathological findings.
    • Early identification and monitoring are crucial for these younger patients due to the risk of rapid progression and malignancy.
    • Further research into the genetic and molecular underpinnings of isolated pheochromocytomas is warranted to understand their pathogenesis and guide therapeutic strategies.