Phaeochromocytoma: a ten-year survey

Area of Science:

• Endocrinology
• Oncology
• Nephrology

Background:

• Phaeochromocytoma is a rare neuroendocrine tumor originating from chromaffin cells.
• It can cause significant morbidity and mortality due to catecholamine excess.
• Understanding clinical presentation, diagnosis, and outcomes is crucial for patient management.

Purpose of the Study:

• To retrospectively analyze the clinical characteristics, diagnostic methods, and surgical outcomes of phaeochromocytoma patients.
• To evaluate the role of biochemical tests and imaging in pre-operative diagnosis and localization.
• To assess long-term outcomes and recurrence rates in a cohort of surgically treated patients.

Main Methods:

• Retrospective review of 18 surgically-proven phaeochromocytoma cases from January 1986 to December 1995.
• Analysis of clinical data, including symptoms, hypertension, tumor location (adrenal vs. extra-adrenal), and familial associations.
• Evaluation of pre-operative diagnostic methods: urine vanillylmandelic acid (VMA), plasma catecholamines, CT scan, and MR imaging.

Main Results:

• 18 patients (3 male, 15 female, age 12-81) diagnosed with phaeochromocytoma.
• 16 patients were hypertensive; classical triad symptoms present in only 6.
• 16 adrenal, 2 extra-adrenal tumors; 4 familial cases (MEN-IIA, NF-I, VHL).
• Elevated urine VMA/catecholamines and plasma catecholamines confirmed diagnosis.
• CT/MR imaging successfully localized tumors pre-operatively.
• 15 patients cured surgically; 2 asymptomatic with recurrence; 1 died post-operatively.

Conclusions:

• Phaeochromocytoma diagnosis relies on a combination of clinical suspicion, biochemical testing, and advanced imaging.
• Surgical intervention offers a high chance of cure, but recurrence necessitates long-term follow-up.
• Familial syndromes should be considered in the diagnostic workup.