Clinical experience over 48 years with pheochromocytoma - PubMed (original) (raw)

. 1999 Jun;229(6):755-64; discussion 764-6.

doi: 10.1097/00000658-199906000-00001.

J A O'Neill Jr, G W Holcomb 3rd, W M Morgan 3rd, W W Neblett 3rd, J A Oates, N Brown, J Nadeau, B Smith, D L Page, N N Abumrad, H W Scott Jr

Affiliations

Clinical experience over 48 years with pheochromocytoma

R E Goldstein et al. Ann Surg. 1999 Jun.

Abstract

Objective: To analyze the presentation, localization, surgical management, pathology, and long-term outcome of a large series of patients with pheochromocytomas.

Summary background data: There are several areas of controversy pertaining to pheochromocytomas. Although many studies report a higher rate of malignancy for extraadrenal pheochromocytomas than for adrenal pheochromocytomas, the number of patients with the former tumor are small and statistical analysis is lacking. There has also been recent debate as to whether microscopic features of the tumor may be predictive of future behavior.

Methods: From 1950 to 1998, the authors observed 108 pheochromocytomas in 104 patients. The outcome of these patients has been followed prospectively. The medical records of these patients were reviewed for data on the presentation, localization, surgical management, pathology, and outcome. Patient survival was analyzed using Kaplan-Meier survival distributions.

Results: This study included 66 female patients and 38 male patients. The average age at surgery was 42.3 years. Sporadic cases accounted for 84% of the patients; the other 16% had multiple endocrine neoplasia type 2, von Recklinghausen's disease, von Hippel-Lindau disease, or Carney's syndrome. Of 64 adrenal tumors, 55 were initially considered benign, 6 had microscopic malignant features, and 3 had malignant disease. Mean patient follow-up was 12.6 years. To date, in five additional patients (none with microscopic disease) malignant disease developed (13% overall rate of malignancy). Recurrence occurred as late as 15 years after resection. Of 26 extraadrenal pheochromocytomas, 14 were initially considered benign, 8 had microscopic malignant features, and 4 had malignant disease. Thus, 46% of patients had either malignant disease or tumors with malignant features. Mean patient follow-up was 11.5 years. In one patient with benign disease and in one patient with malignant features, malignant disease developed (23% overall rate of malignancy). The difference in the rate of malignancy was not statistically significant between adrenal and extraadrenal pheochromocytomas. Patients with adrenal and extraadrenal pheochromocytomas also had similar rates of survival (p = NS).

Conclusions: The data suggest that patients with extraadrenal pheochromocytomas have the same risk of malignancy and the same overall survival as patients with adrenal pheochromocytomas. Lifelong follow-up of these patients is mandatory.

PubMed Disclaimer

Figures

None

Figure 1. Angiogram demonstrating the typical radiographic blush of an extraadrenal pheochromocytoma to the patient’s right of the aorta. This is also the angiogram that did not detect a second extraadrenal pheochromocytoma located on the patient’s left side; it was successfully resected at a second operation.

None

Figure 2. A representative noncontrast CT scan of the abdomen demonstrating a 5-cm left adrenal pheochromocytoma.

None

Figure 3. Overall actual survival. This figure depicts the Kaplan–Meier survival curve for all patients with pheochromocytoma dying from all causes. The patients were divided into categories based on whether they underwent bilateral adrenalectomies, unilateral adrenalectomies, or resection of extraadrenal pheochromocytomas. The survival times of the individual groups were compared using the log-rank test; p = 0.82, indicating that there was no statistical difference in the survival time between groups.

None

Figure 4. Cause-specific survival. This figure depicts the Kaplan–Meier survival curve for all patients with pheochromocytoma dying specifically from pheochromocytoma. The patients were divided into categories based on whether they underwent bilateral adrenalectomies, unilateral adrenalectomies, or resection of extraadrenal pheochromocytomas. The survival times of the individual groups were compared using the log-rank test; p = 0.31, indicating that there was no statistical difference in the survival time between groups.

Similar articles

Cited by

References

    1. Wilbourn RB. Early surgical history of phaeochromocytoma. Br J Surg 1987; 74: 594–596. - PubMed
    1. O’Riordain DS, Young WF Jr, Grant CS, et al. Clinical spectrum and outcome of functional extraadrenal paraganglioma. World J Surg 1996; 20: 916–922. - PubMed
    1. Pommier RF, Vetto JT, Billingsly K, et al. Comparison of adrenal and extraadrenal pheochromocytomas. Surgery 1993; 114: 1160–1166. - PubMed
    1. Proye CAG, Vix H, Jansson S, et al. “The” pheochromocytoma: a benign, intra-adrenal, hypertensive, sporadic unilateral tumor. Does it exist? World J Surg 1994; 18: 467–472. - PubMed
    1. Scott HW Jr, Reynolds V, Green N, et al. Clinical experience with malignant pheochromocytomas. Surg Gynecol Obstet 1982; 154: 801–818. - PubMed

MeSH terms

LinkOut - more resources