Residual arterial hypertension after excision of an extra-adrenal pheochromocytoma

Residual arterial hypertension after excision of a pheochromocytoma of the Zuckerkandl organ, has brought the problem of its etiology. The short term approach consisted in performing, after specific biochemical dosages, a scintigram with IMBG, a scan and a magnetic resonance imaging, to look for a second pheochromocytoma. A negative workup enabled to conclude to an essential arterial hypertension. Nevertheless, this does not exclude the possibility of a long term malignancy, requiring clinical monitoring and IMBG scintigraphy.     

An extra-adrenal abdominal pheochromocytoma causing ectopic ACTH syndrome

We report a 55-year-old woman with ectopic adrenocorticotropin (ACTH) secretion caused by extra-adrenal pheochromocytoma. The patient presented with a 6-month history of hypertension and diabetes mellitus. Her serum and urinary cortisol levels were extremely high and dexamethasone failed to suppress the cortisol secretion. Her plasma ACTH levels were also elevated (>300 pg/mL) and irresponsive to corticotropin-releasing hormone (CRH) or metyrapone administration. Gel filtration analysis of the patient's plasma detected the existence of large molecular weight ACTH being eluted with a major peak of authentic 1-39 ACTH. Abdominal computed tomographic scan and magnetic resonance imaging revealed a 5-cm paraganglioma located underneath the left kidney, in which (123)I-MIBG tracer specifically accumulated. Bilateral adrenal glands were diffusely enlarged. After surgical removal of the paraganglioma, the patient's clinical symptoms improved and biochemistry normalized including plasma ACTH, urinary free cortisol, and urinary catecholamines. Subsequent histologic evaluation of the transected paranglioma tissue revealed ACTH, synaptin, and chromogranin-A histologically immunostaining. Culture of primary cells collected from the resected paraganglioma demonstrated of in vitro production of ACTH, noradrenaline, and adrenaline. This is the first report of ectopic ACTH syndrome induced by an extra-adrenal abdominal paraganglioma.     

Familial extra-adrenal pheochromocytoma. A new syndrome

Pheochromocytomas in the same anatomic site, the right renal hilum, occurred in a family over three successive generations. For two patients in the latter two generations, scintigraphy with iodine 131-tagged metaiodobenzylguanidine (MIBG) showed tumors only in the region of the right renal hilum, thus indicating that they were primary lesions. At surgery, except for lymph node metastases noted microscopically in one patient, tumors were found only near the right renal hilum. The adrenal glands seemed normal on inspection, palpation, and computed tomography. In another family, a mother and son had primary pheochromocytomas arising from the urinary bladder. We suggest that primary extra-adrenal pheochromocytoma is a syndrome in which specific genetic abnormalities determine sites of tumor development.     

A case of metastatic extra-adrenal pheochromocytoma 12 years after surgery.

At the age of 53, a 65-year-old man had been diagnosed with extra-adrenal pheochromocytoma in the retroperitoneum and underwent total tumorectomy. Afterward, he had his serum catecholamine periodically measured in an outpatient clinic. In February 1999, 12 years after surgery, he complained of lower left abdominal pain. Computed tomography and magnetic resonance imaging revealed an osteolytic lesion in thoracic vertebrae 11Th (Th 11). Although his basal serum and urine catecholamines were at normal levels, glucagon injection increased blood pressure and plasma catecholamine levels. 131I-metaiodobenzylguanidine (MIBG) scintigraphy was specifically taken up to Th 11. By bone biopsy, the osteolytic lesion in Th 11 was finally diagnosed with metastasis of pheochromocytoma. For post-operative pheochromocytoma, long-term follow-up involving biochemical tests, including serum catecholamines, and MIBG is needed.     

Capnocytophaga ochracea causing severe sepsis and purpura fulminans in an immunocompetent patient

Capnocytophaga ochracea (C. ochracea), a known human microflora, has been reported to cause sepsis in immunocompromised patients and less severe infections such as intrauterine infections, endocarditis, peritonitis and septic arthritis in the immunocompetent patient. We present the first described case of C. ochracea causing severe sepsis and purpura fulminans in an immunocompetent host.     

Resistant hypertension in an obese patient with obvious obstructive sleep apnea and occult pheochromocytoma

We report the case of a 34-year-old male patient who presented with generalized weakness, poorly controlled hypertension, nocturnal hypertension spikes, and morning headaches. The history of resistant hypertension, obesity, enlarged neck size, and loud irregular snoring strongly suggested obstructive sleep apnea (OSA). To exclude other possible causes of resistant hypertension, the patient underwent an abdominal ultrasound examination, which revealed a lesion in the left adrenal gland area. A pheochromocytoma was successfully removed via laparoscopic adrenalectomy, and both his hypertension and OSA responded dramatically. This case highlights the importance of excluding all causes of resistant hypertension regardless of the initial diagnosis.     

Midgut volvulus in an elderly patient.

In adults, congenital anomalies of intestinal rotation are usually incidental findings. Any symptoms present may be the result of intermittent volvulus of the small bowel. We report classic fluoroscopic, computed tomographic, and angiographic findings in what is believed to be the oldest reported patient with this entity.     

Small ileal neurofibroma causing intussusception in a non-neurofibromatosis patient

Neurofibromas in the small intestine are usually accompanied by von Recklinghausen's disease (neurofibromatosis), and usually originate in the intramuscular plexus of Auerbach. We present here a solitary neurofibroma, which caused an ileocolic intussusception, originating in the submucosal plexus of Meissner in a non-neurofibromatosis patient. To our knowledge, there is no previous report of a neurofibroma originating in the plexus of Meissner. This condition was clearly confirmed by macroscopic and microscopic evaluation.     

Small ventricular septal defect associated with severe pulmonary hypertension.

A small ventricular septal defect in an 18 year old man was shown by serial cardiac catheterisation to be associated with a progressive increase in pulmonary arterial pressure. Surgical closure of the defect had no effect on the increased pulmonary arterial pressure. Whether pulmonary hypertension occurred secondary to the haemodynamics of the ventricular septal defect or as an independent event is uncertain.     

Non-operative management of dens fracture in an elderly patient with severe complications]

An 82-year-old woman was diagnosed with hypertension at the age of 50 and had been treated with antihypertensive agents. Proteinuria was detected at age 60 and her renal function subsequently deteriorated. Hemodialysis was started on February 14, 2000. She fell out of the bed and hit her neck in the early morning on February 15. Neck X-ray tomography revealed Anderson type III dens fracture. Since she had several complicating conditions including angina pectoris and arrhythmia, she was treated by external fixation using a halo-vest. The neck pain improved gradually. During her clinical course, she experienced delirium due to immobilization. However, bone union was complete 5 months later. Physicians should be aware of the possibility that even minor injury can cause dens fracture in elderly patients. Conservative management of type III dense fracture should be considered for elderly patients with a compromised condition.     

Treatment of renovascular hypertension using stent implantation in an elderly patient with NIDDM

A 70-year-old man with NIDDM was diagnosed as having renovascular hypertension (RVH), based on a stenosis of the ostial portion of the left renal artery with markedly elevated plasma renin activity (PRA) in both the left renal vein and the peripheral blood, and positive captopril tests. After percutaneous transluminal renal angioplasty (PTRA), his blood pressure (BP) and PRA normalized. However, since restenosis occurred three months later, stent therapy was applied, and consequently BP and PRA normalized immediately after this procedure. During the one-year follow-up, side effects have not been noted. We propose that stent therapy may be feasible for ostial renal artery stenosis in elderly diabetic patients.     

Evidence for an unidentified ACTH-induced steroid hormone causing hypertension.

The hypothesis that hyperaldosteronism is not the sole cause of hypertension in dexamethasone-suppressible hyperaldosteronsim was tested in an 18-year-old male. After six years of little or no treatment, the hypertension and mild hyperaldosteronism were promptly decreased by a small dose of dexamethasone. During dexamethasone treatment, when aldosterone secretion was suppressed to less than normal and he was normotensive, steroids were given by constant infusion in an attempt to reproduce the hypertension of the dexamethasone-free state. Neither five days of aldosterone or 18-hydroxydesoxycorticosterone (18-OH-DOC) at 1 mg/day, nor desoxycorticosterone (DOC) at 30 mg/day caused hypertension. However, sodium retention and potassium loss was observed during aldosterone and DOC infusion. Hypertension was produced within five days during infusion with ACTH or oral metyrapone. The hypertensive effect of the latter was abolished by addition of aminoglutethimide treatment. These studies suggest that a steroid other than aldosterone, 18-OH-DOC, or DOC may be the cause of the ACTH-induced hypertension in this patient. The aminoglutethimide data suggest that the ACTH effect on blood pressure is due to a steroid, and the metyrapone studies suggest that the steroid may be an 11-desoxysteroid. Urine and blood collected under ACTH stimulation and metyrapone treatment may be a rich source from which we may characterize this hormone.