Clinical course and prognostic factors in patients with malignant pheochromocytoma and paraganglioma: A single institution experience

Abstract

Patients with malignant PCC/PG have diverse clinical courses. Poor survival was independently associated with older age and synchronous metastasis.

Bone metastasis of malignant pheochromocytoma four years after adrenalectomy

“…PCC and paraganglioma are benign or malignant neuroendocrine neoplasms from neural crests, with diverse clinical courses [4][5][6][7]. Worthy of note, metastases of the malignant tumors involve lymph nodes, liver, lungs and bones, where chromaffin tissue is normally absent [7].…”

“…Worthy of note, metastases of the malignant tumors involve lymph nodes, liver, lungs and bones, where chromaffin tissue is normally absent [7]. PCC is located in adrenal glands, whereas paragangliomas develop in paraganglia of the head, neck, thorax, abdomen and pelvis [4][5][6][7]. Differences in clinical behaviour and in prognosis, have suggested that these tumors should be considered as separate clinical conditions [4,5].…”

“…PCC is located in adrenal glands, whereas paragangliomas develop in paraganglia of the head, neck, thorax, abdomen and pelvis [4][5][6][7]. Differences in clinical behaviour and in prognosis, have suggested that these tumors should be considered as separate clinical conditions [4,5]. Most frequently, the cases are sporadic, but in up to 25% they are inherited as familial paragangliomas, and associated with multiple endocrine neoplasia type 2A and 2B, von Hippel-Lindau disease, and von Recklinghausen disease, Carney's triad, and CarneyStratakis syndrome [6,7].…”

“…The rate of malignant or functional adrenal tumors is low, and for PCC is nearly 2% [1]. Approximately 10% of PCC is malignant, with 5-year survival rate is up to 75.4%, and early diagnosis yield better outcome [2,4]. Malignancy rate of adrenal PCC is up to 25%, and the main sites of metastasis are bones, lungs, liver, and lymph nodes [2].…”

Metastatic pheochromocytoma to liver without elevation of metanephrines and catecholamines

“…It is estimated that up to 5% of patients presenting with adrenal incidentalomas are found to have pheochromocytomas. [1], [13]. Of this, only 10% are malignant pheochromocytoma characterized by the presence of metastasis which spread to organs where no chromaffin cells are present.…”