| Pulmonary manifestations of pheochromocytoma are infrequent and are not well documented. A medline search in the English language revealed no cases of endobronchial involvement from a pheochromocytoma. We report a case of endobronchial metastases in a 37-year-old woman known to have a recurrent extra-adrenal pheochromocytoma. She presented with symptoms of wheezing and a nonproductive cough for 8 months and was being treated for asthma. A flexible bronchoscopy with endobronchial biopsy established the diagnosis. The patient underwent a Nd-YAG laser photoresection (LPR) to ablate the tumor, which was followed by placement of a Wallstent (Pfizer Medical technology Group; Rutherford, NJ). She remains well 18 months later, having required multiple palliative LPRs. To our knowledge, this is the first reported case of endobronchial pheochromocytoma. The pulmonary manifestations of this rare disease and their management are reviewed. ( info) |
2/1379. heart failure induced by pheochromocytoma: laparoscopic treatment and intraoperative changes of several new cardiovascular hormones. Since 1992, adrenalectomy for pheochromocytoma has been recognized as a safe and efficient technique when performed by a laparoscopic approach. Most of the cases of pheochromocytomas treated as such and published in the literature were not associated with malignant hypertension and acute heart failure. We report the case of a 23-year-old woman who presented with this clinical picture and show that laparoscopic adrenalectomy may be as safe and efficient as conventional adrenalectomy when performed in this situation. The intraoperative changes in the secretion of catecholamines, endothelin-1, angiotensin ii, N- and C-terminus of atrial natriuretic factor prohormone were also analyzed. Noradrenaline release during tumor dissection was associated with a stimulation of atrial natriuretic factor. ( info) |
3/1379. CNS pseudovasculitis in a patient with pheochromocytoma. The authors report a patient with angiographic findings resembling CNS vasculitis (CNS pseudovasculitis) who was found to have a pheochromocytoma. The angiographic changes resolved after surgical resection of the pheochromocytoma. pheochromocytoma should be included in the differential diagnosis of angiographic findings suggestive of CNS vasculitis. ( info) |
| Cardiac pheochromocytoma is an exceedingly rare and unusual clinical entity. Only 37 previous surgically treated adult patients were found in review of the surgical literature. We report the case of a 13-year-old boy who had a cardiac pheochromocytoma that was localized by the 111-indium diethylenetriamine pentaacetic acid octreotide scintigraphy scan and confirmed by magnetic resonance imaging after computed tomographic and B1-iodine-metaiodobenzylguanidine scans had failed. At operation, a 6-cm pheochromocytoma of the left atrium was found and successfully resected with reconstruction of the left atrium using autologous pericardium. ( info) |
5/1379. Unsuspected extra-adrenal pheochromocytoma simulating ovarian tumor. We report on a case of an extraadrenal pheochromocytoma simulating an ovarian tumor. Before intervention, the patient exhibited no symptoms suggestive of pheochromocytoma. Nevertheless, during surgery she experienced marked blood pressure fluctuations, and an unsuspected extraadrenal pheochromocytoma was diagnosed. Thus, although rare, when preparing to remove a pelvic mass, the gynecologist should consider the possibility of an extraadrenal pheochromocytoma. ( info) |
| We report the first patient who had bilateral pheochromocytoma associated with multiple endocrine neoplasia type 2a syndrome (men 2a) and underwent unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy 2 years later. The postoperative course was uneventful both times, and the patient was cured of hypertension without any need for steroid replacement. Endoscopic partial adrenalectomy is a minimally invasive procedure for pheochromocytoma with mild symptoms. We believe that this procedure has considerable potential for treating bilateral pheochromocytoma, which is frequently observed in patients with men 2a. ( info) |
7/1379. Severe paroxysmal hypertension (pseudopheochromocytoma): understanding the cause and treatment. Severe, symptomatic paroxysmal hypertension always generates suspicion of a pheochromocytoma, a catecholamine-secreting tumor. However, most patients with this disorder do not have this tumor and their condition remains undiagnosed and ineffectively treated. This case series, summarizing the course of 21 such patients, suggests a cause and an effective treatment approach. All 21 patients insisted that the paroxysms were not related to stress or emotional distress, initially discouraging consideration of a link to emotions. Nevertheless, with careful psychosocial interviewing, the disorder could be attributed to emotions patients were not aware of, and, therefore, unable to report. Such emotions were related either to previous severe emotional trauma or to a general tendency to keep distressful emotions out of awareness. With treatment based on this understanding, further paroxysms were eliminated in 13 (62%) of 21 patients. Alpha- plus beta-blockade was used, combined, when necessary, with an antidepressant agent, with or without an anxiolytic agent. In 3 cases, the disorder was cured with psychotherapy alone. Because the presenting symptoms are physical rather than emotional, patients present to internists and primary care physicians rather than to psychotherapists. For this reason, more awareness of this disorder in the medical community is needed. ( info) |
| Cerebral ischemia and symptoms of stroke can occur as a rare manifestation in patients with pheochromocytoma. We describe a 45-year-old woman who was admitted because of a right-sided hemiparesis due to an ischemic lesion in the left hypothalamus. The clinical diagnosis of a pheochromocytoma was proven by highly elevated urinary catecholamines and confirmed histologically after operation. The successful removal of the tumor led to the almost complete recovery of the neurological deficiencies. It is of vital importance to know this atypical presentation of pheochromocytoma. The diagnosis of pheochromocytoma should be suspected in patients with focal cerebral symptoms, particularly in the presence of intermittent hypertension or other paroxysmal symptoms suggestive of pheochromocytoma. ( info) |
| A germline mutation either in exon 10 or 11 of the RET proto-oncogene is found in the majority of patients with multiple endocrine neoplasia type 2a (men 2A). A 41-year-old female patient was referred for further evaluation of incidentally discovered right adrenal tumor. She had bilateral adrenal pheochromocytomas and medullary thyroid carcinomas detected by endocrinological and radiological examination, and diagnosed as men 2A. Molecular genetic testing of the RET exons 10 and 11 exhibited the identical somatic missense mutation at codon 634 in both tumors but did not confirm germline mutations in the corresponding sites. Possible mechanisms for tumorigenesis in this patient are discussed. ( info) |
10/1379. Reversible catecholamine-induced cardiomyopathy in a heart transplant candidate without persistent or paroxysmal hypertension. BACKGROUND: Both dilated and hypertrophic cardiomyopathy have been reported in patients with pheochromocytoma, who were almost always hypertensive. The outcome frequently has been fatal, yet cardiac dysfunction can be reversible after medical or surgical therapy for the pheochromocytoma. methods: We report the case of a patient with dilated cardiomyopathy without persistent or paroxysmal hypertension, who was found to have a pheochromocytoma during initial medical evaluation. RESULTS: The identification and treatment of the pheochromocytoma led to significant improvement in cardiac function and cardiac transplantation was avoided. CONCLUSIONS: This case illustrates some unusual features in pheochromocytoma-induced cardiomyopathy: (1) absence of persistent or paroxysmal hypertension, (2) initial presentation with acute myocardial infarction and normal coronary arteries, and (3) recurrent episodes of nonsustained ventricular tachycardia. ( info) |