Elsevier

Surgery

Volume 136, Issue 6, December 2004, Pages 1129-1137
Surgery

American Association of Endocrine Surgeon
Should pheochromocytoma size influence surgical approach? A comparison of 90 malignant and 60 benign pheochromocytomas

https://doi.org/10.1016/j.surg.2004.05.058Get rights and content

Background

Diagnostic tests cannot reliably distinguish malignant from benign pheochromocytomas. Laparoscopic adrenalectomy for pheochromocytomas >6 cm is controversial because of a perceived increased risk of malignancy that is based on anecdotal reports and small series. The aim of this study was to determine if pheochromocytoma size should affect the choice of surgical approach.

Methods

Malignant pheochromocytomas in the Surveillance Epidemiology and End Results (SEER) database (1988-2000) were compared to benign pheochromocytomas in our institutional database (1993-2003). The sensitivity, specificity and likelihood ratio for tumor size to predict malignancy were calculated for both groups.

Results

Ninety malignant and 60 benign pheochromocytomas were compared. Overall, malignant pheochromocytomas were larger than benign pheochromocytomas (7.6 ± 4.2 cm vs 5.3 ± 2.3 cm). However, tumor size was not significantly different between malignant pheochromocytomas without local invasion or metastases and benign pheochromocytomas (6.1 ± 3.1 cm vs 5.3 ± 2.3 cm). In pheochromocytomas with local disease only, maximum likelihood ratio to predict malignancy was at a tumor size of greater than 8 cm (2.84).

Conclusions

Although risk of malignancy increases with size for all pheochromocytomas, size does not reliably predict malignancy in pheochromocytomas with local disease only. Regardless of tumor size, laparoscopic adrenalectomy for pheochromocytoma should be converted to open adrenalectomy for difficult dissection, invasion, adhesions, or surgeon inexperience.

Section snippets

Methods

The SEER database is a comprehensive source of information regarding cancer incidence and survival in the United States. The information in the SEER database is accrued from several regional cancer registries and includes demographics, tumor characteristics, treatment, and survival for more than 3 million patients between 1973 and 2000. We reviewed the SEER database13 for patients who were treated for malignant pheochromocytoma. Size data for tumors in the SEER database have only been collected

Results

We identified 90 malignant pheochromocytomas in the SEER database treated between 1988 and 2000. From the UCSF database, we identified 56 patients with 60 benign pheochromocytomas who underwent adrenalectomy between 1993 and 2003. Follow-up time was 50 ± 42 months for the SEER group. Mean time from surgery was 62 ± 31 months for the UCSF group; all of the UCSF patients are seen by their surgeon within 1 month of operation and are then followed closely by our referring primary care physicians

Discussion

When signs of regional involvement or distant disease are absent, there is currently no reliable preoperative diagnostic test that can differentiate between malignant and benign pheochromocytomas. Because pheochromocytomas are rare, and malignant pheochromocytomas represent only 2.4% to 26% of these already uncommon tumors,4., 5., 6., 7., 8. few centers have accumulated enough patients to produce accurate and generalizable information regarding malignant pheochromocytomas. We therefore used the

Addendum

Before publication of this article we were informed by a referring physician that 1 patient in the benign pheochromocytoma database had developed local recurrence at the resection site as well as pulmonary metastases. This individual is currently undergoing 131-I MIBG treatment under an investigational protocol. The primary tumor was a 9-cm, right adrenal pheochromocytoma, and the operation was converted from laparoscopic to open adrenalectomy because of tumor adhesions to the liver. This new

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Cited by (110)

  • Outcomes of malignant pheochromocytoma based on operative approach: A National Cancer Database analysis

    2021, Surgery (United States)
    Citation Excerpt :

    Pheochromocytoma is diagnosed with a combination of typical symptomatology, biochemical testing, and imaging.1,4 Reports have levied large tumor size as a marker of malignant nature, although this is not widely accepted.13,21 Other than visualizing the presence of local invasion or distant metastatic disease on preoperative imaging, designating a pheochromocytoma as malignant before adrenalectomy remains difficult.

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Presented at the 25th Annual Meeting of the American Association of Endocrine Surgeons, Charlottesville, Virginia, April 4-6, 2004.

Supported in part by the Robert Wood Johnson/Harold Amos Faculty Development Program Fellowship, the Hellman Family Grant, the Albert Clark Family Foundation, the Sanford and Helen Diller Foundation, Friends of Endocrine Surgery, and the Mt. Zion Health Systems.

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