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Oncologic aspects of pheochromocytoma: the importance of follow-up
Insights
Pheochromocytoma diagnosis and management over 33 years revealed a 13% incidence of malignant pheochromoblastoma. Lifetime follow-up is crucial for patients with pheochromocytoma due to high rates of secondary cancers.
Area of Science:
- Endocrinology
- Oncology
- Surgical Pathology
Background:
- Pheochromocytoma is a rare tumor of the adrenal medulla.
- Hypertension is a common presenting symptom.
- Delayed diagnosis can lead to poor outcomes.
Purpose of the Study:
- To evaluate the long-term outcomes of patients with pheochromocytoma.
- To determine the incidence of malignant pheochromocytoma and secondary malignancies.
- To emphasize the importance of lifelong patient follow-up.
Main Methods:
- Retrospective review of 69 patients diagnosed with pheochromocytoma over 33 years.
- Analysis of clinical diagnosis, surgical outcomes, and histopathological findings.
- Long-term follow-up of patients to assess for secondary malignancies.
Main Results:
- 13% of patients had malignant pheochromoblastoma.
- 23% of patients with pheochromocytoma developed additional malignant tumors.
- 15% of patients with benign pheochromocytoma developed other benign neoplasms.
- Overall incidence of malignant disease in this cohort was 36%.
Conclusions:
- Pheochromocytoma diagnosis has improved over time, but remains challenging.
- Patients with pheochromocytoma have a high risk of developing secondary malignancies.
- Lifelong surveillance by the operating surgeon is essential for optimal patient management.
Abstract:
During a 33-year period at Vanderbilt University Medical Center and its long-standing affiliated hospitals, Veterans Administration Medical Center and Metropolitan Nashville General Hospital, 69 patients with pheochromocytoma were seen. Despite the fact that hypertension was present in all patients at initial admission, in 11 patients seen during the 1950s the clinical diagnosis of pheochromocytoma was not made and the existence of this tumor was discovered at autopsy examination. In 58 patients the clinical diagnosis of pheochromocytoma was made and surgical excision of pheochromocytoma (or biopsy only in two cases) was carried out. One patient died during the initial postoperative period (1.7%). All of the operative survivors were followed to date or the death, with the exception of three who were lost to follow-up at 18 months and 7 and 11 years, respectively. The incidence of malignant disease in this carefully followed group of patients with pheochromocytoma was impressive. Nine of 69 patients (13%) proved to have malignant pheochromoblastoma, all but one of whom have died. Additional malignant tumors have occurred in two of these nine patients (22%) with proved malignant pheochromoblastoma and in 14 of 60 patients (23%) with benign pheochromocytoma. The overall incidence of additional malignant tumors in the collective group of patients with the histopathologically confirmed diagnosis of pheochromocytoma was thus 23% (16/69 patients). It is also interesting that a variety of benign neoplasms occurred in the 60 patients with benign pheochromocytomas (9/60; 15%). If the nine patients with malignant pheochromoblastoma are added, the overall incidence of malignant disease in these 69 patients with pheochromocytoma is 25/69 or 36%. Follow-up of patients with pheochromocytoma is mandatory on a lifetime basis and preferably by the operating surgeon.