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Related Experiment Videos

Experience with extra-adrenal pheochromocytoma.

D A Goldfarb1, A C Novick, E L Bravo

  • 1Department of Urology, Cleveland Clinic Foundation, Ohio 44106.

The Journal of Urology
|October 1, 1989
PubMed
Summary

Extra-adrenal pheochromocytomas are often benign, with a lower malignancy rate than previously reported. This study highlights their varied presentations and successful surgical outcomes.

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Area of Science:

  • Endocrinology
  • Surgical Oncology
  • Urology

Background:

  • Extra-adrenal pheochromocytomas are rare neuroendocrine tumors originating outside the adrenal glands.
  • These tumors can present with diverse symptoms, often mimicking other conditions due to their varied anatomical locations.
  • Previous literature suggested a higher incidence of malignancy in extra-adrenal compared to adrenal pheochromocytomas.

Purpose of the Study:

  • To analyze the clinical characteristics, diagnostic methods, and outcomes of patients with extra-adrenal pheochromocytomas.
  • To evaluate the incidence of malignancy and recurrence in this patient cohort.
  • To compare findings with previously reported data on extra-adrenal pheochromocytoma.

Main Methods:

  • Retrospective review of 20 patients diagnosed with extra-adrenal pheochromocytomas between 1955 and 1985.

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  • Analysis of patient demographics, presenting symptoms, tumor location, diagnostic imaging, and treatment outcomes.
  • Review of postoperative follow-up data, including recurrence and long-term complications.

    • Main Results:

    • 22 extra-adrenal pheochromocytomas were identified in 20 patients (13 male, 7 female), with peak incidence in the second decade.
    • Unusual presentations included mediastinal, neck, and bladder masses; elevated plasma epinephrine was noted in 5/6 patients.
    • The superior para-aortic region was the most common location; computerized tomography was the most accurate imaging modality.
    • Complete surgical excision was achieved in 19 patients; follow-up (median 120 months) revealed 3 recurrences, all successfully treated.
    • No metastatic disease was observed, and the incidence of malignancy was low (2/22 tumors).

    Conclusions:

    • Extra-adrenal pheochromocytomas can present with atypical symptoms related to tumor location.
    • Computerized tomography is a valuable tool for pre-operative localization.
    • This study suggests a lower incidence of malignancy and a generally benign course for extra-adrenal pheochromocytomas, contrasting with prior reports.