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Familial pheochromocytoma.

G L Irvin, L M Fishman, J A Sher

    Surgery
    |December 1, 1983
    PubMed
    Summary

    Familial pheochromocytoma in a father and three daughters was treated. Subtotal adrenalectomy for bilateral tumors allowed normal adrenal function and no recurrence in two sisters.

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    Area of Science:

    • Endocrinology
    • Surgical Oncology
    • Genetics

    Background:

    • Pheochromocytoma is a rare tumor of the adrenal medulla.
    • Familial forms of pheochromocytoma can present with early-onset malignant hypertension.
    • This study examines a unique family with multiple affected members.

    Observation:

    • A 15-year study identified 24 pheochromocytoma patients, including a father and three daughters with familial disease.
    • The prominent feature was early-onset malignant hypertension.
    • All tumors were bilateral, adrenal-confined except for hepatic extension in the father.

    Findings:

    • No evidence of multiple endocrine adenomatosis was found in this family.
    • Subtotal adrenalectomy in two sisters resulted in normotension, normal urine catecholamines, and no medication for 3-7 years post-operation.
    • The father with residual liver tumor remains hypertensive; the youngest sister with total adrenalectomy requires steroid replacement.

    Implications:

    • Bilateral pheochromocytomas may be managed with subtotal adrenalectomy, preserving adrenal function in select cases without multiple endocrine adenomatosis.
    • This approach may avoid lifelong steroid replacement and reduce recurrence risk.
    • Further research into optimal surgical management for familial pheochromocytoma is warranted.

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