A clinicopathological study of pheochromocytoma and paraganglioma with review of literature

Abstract

Background and Aim: Pheochromocytoma and paraganglioma (PPGL) are rare chromaffin cell tumors with variable clinical presentation which are derived from neural crest cells. Pheochromocytomas arising from the adrenal glands are sympathetic, and the paragangliomas arising from extra-adrenal sites are either sympathetic or parasympathetic in origin. These tumors usually come to clinical attention due to the catecholamine secretion or maybe detected as incidentalomas while imaging for unrelated symptoms. Our objective is to study the histopathological and histochemical features of PPGL with clinical correlation. Materials and Methods: PPGLs arising from both sympathetic and parasympathetic nervous systems were retrospectively selected from the records for 5 years, and clinical details were obtained. Routine hematoxylin and eosin (H and E) stain and argentaffin and argyrophilic reactions were employed. Immunohistochemistry for chromogranin was done for cases with doubtful histomorphology in H and E sections. Results: Our study material consisted of 10 cases out of which five cases were adrenal pheochromocytomas; 4, carotid body tumors; and 1, paraganglioma of the organ of Zuckerkandl. Three pheochromocytomas out of five and the paraganglioma of organ of Zuckerkandl were functional and clinically presented with palpitation and hypertension. All the carotid body tumors and two of the pheochromocytomas were nonfunctional. The 24 h urinary levels of vanillylmandelic acid was increased in two cases, and urinary fractionated metanephrine levels was increased in one case of pheochromocytoma. Urinary normetanephrine alone increased in paraganglioma of organ of Zuckerkandl. All pheochromocytomas except one were positive for argentaffin reaction, and all paragangliomas at other sites were positive for argyrophilic reaction. Chromogranin immunohistochemistry stain was done for organ of Zuckerkandl lesion which came out to be positive. Conclusion: Proper clinical evaluation, preoperative preparation, and appropriate early surgical treatment can cure the lesion. Preoperative diagnosis, if not done and if there is a delay in the treatment, will lead to complications such as intraoperative hypertensive attack, persistent hypertension even after removal of the tumor, and progression to malignancy.