Histopathological analysis of spontaneous large necrosis of adrenal pheochromocytoma manifested as acute attacks of alternating hypertension and hypotension: a case report

Abstract

BackgroundPheochromocytomas are rare catecholamine-producing neuroendocrine tumors. Hypertension secondary to pheochromocytoma is often paroxysmal, and patients occasionally present with sudden attacks of alternating hypertension and hypotension. Spontaneous, extensive necrosis within the tumor that is associated with catecholamine crisis is an infrequent complication of adrenal pheochromocytoma, but its pathogenesis remains unclear.Case presentationA 69-year-old Japanese man developed acute-onset episodic headaches, palpitations, and chest pains. During the episodes, both marked fluctuations in blood pressure (ranging from 40/25 to 300/160 mmHg) and high plasma levels of catecholamines were found simultaneously. Radiological findings indicated a 4-cm left adrenal pheochromocytoma. These episodic symptoms disappeared within 2 weeks with normalization of plasma catecholamine levels. Two months later, the patient underwent adrenalectomy. Microscopic examinations revealed pheocromocytoma with a large central area of coagulative necrosis. The necrotic material was immunohistochemically positive for chromogranin A. Granulation tissue was adjacent to the necrotic area, accompanied by numerous hemosiderin-laden macrophages and histiocytes with vascular proliferation. Viable tumor cells, detected along the periphery of the tumor, demonstrated pyknosis, and the Ki-67 labeling index was 2 % in the hot spot. No embolus or thrombus formation was found in the resected specimen harboring the whole tumor. The Pheochromocytoma of the Adrenal gland Scaled Score was 2 out of 20. The patient’s postoperative course was unremarkable for > 7 years.ConclusionsPresumed causal factors for the extensive necrosis of adrenal pheochromocytoma in previously reported cases include hemorrhage into the tumor, hypotension induced by a phentolamine administration, embolic infarction, high intracapsular pressure due to malignant growth of the tumor, and catecholamine-induced vasoconstriction. In the present case, histopathological and clinical findings suggest that under conditions of chronic ischemia due to catecholamine-induced vasoconstriction, an acute infarction occurred after sudden attacks of alternating hypertension and hypotension. Over the subsequent 2 weeks, repetitive massive release of catecholamines from the infarcts into circulation likely accelerated infarction progression by causing repeated attacks of alternating hypertension and hypotension and resulted in the large necrosis. This case highlights the need for physicians to consider acute spontaneous tumor infarction accompanying episodic catecholamine crisis as a rare but severe complication of pheochromocytoma.

Emergent transcatheter arterial embolization to control critical blood pressure fluctuation associated with hypercatecholaminemic crisis in a patient with an unruptured retroperitoneal paraganglioma

“…Various triggers, including a forward-bending posture, can induce this crisis and occasionally lead to critical fluctuation in BP, such as that manifested by our patient [ [12] , [13] , [14] , 24 , 25 ]. Ohara and colleagues reported acute attacks of critical fluctuation in BP in a patient with a pheochromocytoma with spontaneous large coagulative necrosis [11] . They surmised that catecholamine-induced vasoconstriction had produced acute infarcts in the tumor under chronic ischemia that progressed through repetitive massive releases of catecholamines into the circulation, resulting in extensive necrosis [11] .…”

“…Ohara and colleagues reported acute attacks of critical fluctuation in BP in a patient with a pheochromocytoma with spontaneous large coagulative necrosis [11] . They surmised that catecholamine-induced vasoconstriction had produced acute infarcts in the tumor under chronic ischemia that progressed through repetitive massive releases of catecholamines into the circulation, resulting in extensive necrosis [11] . Similarly in our patient, histological examination confirmed a large area of coagulative necrosis without hemorrhage within the paraganglioma.…”

“…Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumors that originate from chromaffin cells in the adrenal medulla (pheochromocytomas) and the extra-adrenal autonomic ganglia (paragangliomas) [1] , [2] , [3] , [4] , [5] , [6] . PPGL-associated hypercatecholaminemic crisis is a rare lethal condition resulting from uncontrolled catecholamine secretion, which may lead to severe hyper- and/or hypotension, multisystem organ failure, and, less frequently, critical fluctuation in blood pressure (BP) [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] . Generally, the first-line treatment for PPGLs is elective surgical tumor resection preceded by antihypertensive therapy using alpha-1 blockade [15] .…”

“…Surgery carries a high risk of intra- and perioperative morbidity and mortality, whereas patients experiencing hypercatecholaminemic crisis may require emergency surgery [16] , [17] , [18] , [19] , [20] . In patients with critical fluctuation in BP, particularly with episodes of severe hypotension, medical control using alpha-1 blockade may be contraindicated [11] , [12] , [13] , [14] . Emergent transcatheter arterial embolization (TAE) has been sometimes performed for spontaneous rupture of PPGLs, but we believe it has not been utilized in patients manifesting severe BP fluctuation associated with hypercatecholaminemic crisis in the absence of rupture [21] , [22] , [23] .…”

A case of pheochromocytoma with a marked decrease in catecholamine levels after rupture in which a good outcome was achieved by elective surgery

“…The cause of intratumoral hemorrhage and rupture was probably increased intratumoral pressure. A massive release of catecholamines from pheochromocytoma into the circulation probably accelerates the progression of tumor infarction, resulting in the extensive necrosis of adrenal pheochromocytoma [10]. Acute infarction and subsequent necrosis decrease the release of catecholamines from the tumor.…”