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Composite pheochromocytoma-ganglioneuroma of the adrenal gland: An uncommon entity with distinctive clinicopathologic features

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Abstract

Background

Adrenal composite pheochromocytoma-ganglioneuroma is uncommon. The aim of this study is to investigate the characteristics of patients with this tumor.

Methods

Histologic features of 46 pheochromocytomas diagnosed over a 28-yr period (1971–1988) were reviewed. The clinical records, pathologic features, and ploidy patterns of patients with composite pheochromocytoma-ganglioneuroma were studied. Cases reported in the literature were also reviewed and compared with typical pheochromocytomas.

Results

Four patients (two men; two women) with composite pheochromocytoma-ganglioneuromas were found. The tumors comprised 8.7% of pheochromocytomas. The two male patients with composite tumors were younger than the two female patients (age=32,52 vs 73,75, respectively). The radiologic, gross, and microscopic appearances of the tumors were heterogeneous. One patient had malignant tumor with distant metastases. Flow cytometric analysis showed that the pheochromocytoma component was aneuploid and the ganglioneuroma portion was diploid. A review of the literature showed 31 adrenal composite pheochromocytoma-ganglioneuromas. Composite tumors were seen in older patients and were bigger than typical pheochromocytomas.

Conclusion

Composite pheochromocytoma-ganglioneuromas had distinctive clincopathologic features. Life-long clinical and biochemical follow-up of these patients is essential in view of the nondiploid DNA pattern.

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Authors and Affiliations

  1. Department of Pathology, University of Hong Kong, Hong Kong

    King-Yin Lam F.R.C.P.A.

  2. Department of Surgery, University of Hong Kong, Hong Kong

    Chung-Yao Lo F.R.C.S.Ed.

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  1. King-Yin Lam F.R.C.P.A.
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Correspondence to King-Yin Lam F.R.C.P.A..

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Lam, KY., Lo, CY. Composite pheochromocytoma-ganglioneuroma of the adrenal gland: An uncommon entity with distinctive clinicopathologic features. Endocr Pathol 10, 343–352 (1999). https://doi.org/10.1007/BF02739777

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