Malignant pheochromocytoma in the anterior mediastinum with sternal invasion: a case report

Abstract

Primarily arising from neural crest-derived tumors including the adrenal medulla and sympathetic paraganglia, pheochromocytomas consist of chromaffin cells that may produce, store, metabolize and secrete catecholamines (1-3). These tumors commonly originate from approximately 80-85% of cases from intra-adrenal chromaffin tissues and from approximately 15-20% of cases from extra-adrenal chromaffin tissues (4). As the rare disease, more than 40% of pheochromocytomas are associated with germline mutations in susceptibility genes (5). The malignant ones account for approximately 10% of all pheochromocytomas (6,7). Patients who suffer from pheochromocytomas mainly complain of hypertension, headache, sweating, palpitation, apprehension, tremor, pallor or flushing of the face, nausea and vomiting, chest pain, abdominal pain, or limb paresthesias (8,9). These symptoms are regarded as the result of the increased secretion of epinephrine and norepinephrine.Ectopic pheochromocytomas always originate from the extra-adrenal paraganglion system, which can be either benign Abstract: Pheochromocytomas are catecholamine-producing neuroendocrine tumors that usually occur in the adrenal medulla or sympathetic paraganglia. Anterior mediastinum involvement with pheochromocytoma is rare and may not present with typical symptoms. Its clinical manifestation may be unclear and a high index of suspicion is required for accurate diagnosis. We report a rare case of pheochromocytoma of the anterior mediastinum in a 51-year-old female. A painful hard mass on the sternum was the only clinical manifestation. Imageological examination indicated that there might be a malignant mass on the anterior mediastinum and thoracic wall. The patient accepted surgical curettage and thoracic wall reconstruction. Based on pathological results and WHO definition, the final diagnosis was malignant pheochromocytoma. After six months follow-up, the patient had no recurrence or any symptom. Malignant pheochromocytoma in the anterior mediastinum invading the sternum is rare. A local painful mass may be the only clinical manifestation without special laboratory results. Surgery remains as the first choice for these patients. For this rare case, 3D reconstruction by special software may be a good method to realize individualized treatment. The final decision of the diagnosis should be based on pathological results, past medical history and WHO definition.Long-term follow-up is necessary, while other suspicious lesions should also be given sufficient attention. Zuckerkandl and paraganglia (10). In addition to the classical symptoms of adrenal pheochromocytomas, the level of urine catecholamine and its derivatives can also be regarded as diagnostic criteria. However, not all extra-adrenal pheochromocytomas secrete these. In fact, some patients who suffer from pheochromocytomas are asymptomatic. To date, the resection of pheochromocytomas remains as the most effective method.Pheochromocytoma in the anterior mediastinum is very rare, especially those that that invade t...

Spontaneous Retroperitoneal Bleeding as a Complication of Unusual Renal Paraganglioma

“…Paragangliomas/pheochromocytoma are rare neuroendocrine tumors arising from chromaffin cell tumors located at any extra-adrenal site along the sympathetic or parasympathetic nervous system and account for 15 to 20% of chromaffin cell tumors [ 1 , 8 ]. The incidence of extra-adrenal paragangliomas is estimated to be 2–8 per million individuals and has been significantly increasing over the past two decades [ 17 ].…”

“…e majority of paragangliomas are nonhypersecretory and are found in the head and neck as compared to the abdominal ones [6]. e kidney, bladder, and mediastinum are among the common intra-abdominal sites [7][8][9]. Based on the ability of catecholamine secretion, paragangliomas can be classi ed as functional or nonfunctional [6,10].…”

Robotic approach to a subcarinal functional paraganglioma

“…Approximately 10% of these tumors arise from extra-adrenal tissues and are termed paragangliomas. Approximately 10-15% of pheochromocytomas will exhibit malignant behavior, defined as the presence of paraganglioma in non-chromaffin tissues [2]. Mediastinal paragangliomas are uncommon and usually non-functional, with an approximate incidence of 2% of all reported cases [3].…”