Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was refer...Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. After surgical resection of the tumor, diarrhea subsided and both electrolytes and affected hormone levels normalized. Immunohistochemical examination confirmed a diagnosis of pheochromocytoma, which contained VIP-positive ganglion-like cells. We herein present the clinical and histogenetic implications of this rare clinical entity, with literature review.展开更多
Splenic metastasis is generally not a common clinical event. However, metastasis to the spleen from adrenal pheochromocytoma is extremely rare and has not been reported in literature. This report presents a case of a ...Splenic metastasis is generally not a common clinical event. However, metastasis to the spleen from adrenal pheochromocytoma is extremely rare and has not been reported in literature. This report presents a case of a 58 year-old male patient who developed spleen-only metastases in July 2007. The patient had a previous history of left epinephroectomy for adrenal pheochromocytoma in January 2003. Abdominal computed tomography demonstrated multiple enhancing lesions suggestive of metastases; thus splenectomy was performed. Pathological examinations confirmed the diagnosis of splenic metastases from pheochromocytoma. The patient was alive without recurrence 48 months after splenectomy. qttis study is the first report on splenic metastasis from previous adrenal pheochromocytoma, and long-term survival was achieved by splenectomy. A history of malignancy indicates a high index of suspicion for splenic metastasis, and long-term survival can be achieved by splenectomy for spleen-only metastasis.展开更多
In bilateral pbeochromocytoma, localization of the dominant adrenal is challenging but highly important since the removal of dominant side can markedly improve cardiovascular outcomes. To demonstrate the usefulness of...In bilateral pbeochromocytoma, localization of the dominant adrenal is challenging but highly important since the removal of dominant side can markedly improve cardiovascular outcomes. To demonstrate the usefulness of glucagon-stimulated BAVS (bilateral adrenal venous sampling) in determining the dominant adrenal to be removed, the authors reviewed records of patients who underwent BAVS with glucagon stimulation from 1997-2010. Nineteen out of 44 patients were diagnosed with bilateral pheochromocytoma. Mean age at diagnosis was 33 ± 14 years. Duration of hypertension was 5 ± 6 years with highest systolic BP (blood pressure) of 186 ±30 mmHg and diastolic BP of 113 ±18 mmHg. Headache (68%) is the most common symptom followed by paroxysmal hypertension (58%). Majority were taking 〉 3 anti-hypertensive drugs. On glucagon-stimulated BAVS, 63% had right adrenal dominance. Three patients, who were hypertensive for 1, 6 and 12 years, underwent removal of the dominant adrenal. On follow-up (mean period = 36 months), there was marked improvement in BP control [pre-op vs. post-op: (systolic) 160-240 mmHg vs. 120-150 mmHg; (diastolic) 90-110 mmHg vs. 70-90 mmHg] and reduction in number of anti-hypertensive medications (from 3-5 to 2 classes of drugs). BAVS with glucagon stimulation is a valuable tool in the identification of the dominant adrenal to be removed in patients with bilateral pheochromocytoma to alleviate chronic hypertension.展开更多
文摘Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. After surgical resection of the tumor, diarrhea subsided and both electrolytes and affected hormone levels normalized. Immunohistochemical examination confirmed a diagnosis of pheochromocytoma, which contained VIP-positive ganglion-like cells. We herein present the clinical and histogenetic implications of this rare clinical entity, with literature review.
文摘Splenic metastasis is generally not a common clinical event. However, metastasis to the spleen from adrenal pheochromocytoma is extremely rare and has not been reported in literature. This report presents a case of a 58 year-old male patient who developed spleen-only metastases in July 2007. The patient had a previous history of left epinephroectomy for adrenal pheochromocytoma in January 2003. Abdominal computed tomography demonstrated multiple enhancing lesions suggestive of metastases; thus splenectomy was performed. Pathological examinations confirmed the diagnosis of splenic metastases from pheochromocytoma. The patient was alive without recurrence 48 months after splenectomy. qttis study is the first report on splenic metastasis from previous adrenal pheochromocytoma, and long-term survival was achieved by splenectomy. A history of malignancy indicates a high index of suspicion for splenic metastasis, and long-term survival can be achieved by splenectomy for spleen-only metastasis.
文摘In bilateral pbeochromocytoma, localization of the dominant adrenal is challenging but highly important since the removal of dominant side can markedly improve cardiovascular outcomes. To demonstrate the usefulness of glucagon-stimulated BAVS (bilateral adrenal venous sampling) in determining the dominant adrenal to be removed, the authors reviewed records of patients who underwent BAVS with glucagon stimulation from 1997-2010. Nineteen out of 44 patients were diagnosed with bilateral pheochromocytoma. Mean age at diagnosis was 33 ± 14 years. Duration of hypertension was 5 ± 6 years with highest systolic BP (blood pressure) of 186 ±30 mmHg and diastolic BP of 113 ±18 mmHg. Headache (68%) is the most common symptom followed by paroxysmal hypertension (58%). Majority were taking 〉 3 anti-hypertensive drugs. On glucagon-stimulated BAVS, 63% had right adrenal dominance. Three patients, who were hypertensive for 1, 6 and 12 years, underwent removal of the dominant adrenal. On follow-up (mean period = 36 months), there was marked improvement in BP control [pre-op vs. post-op: (systolic) 160-240 mmHg vs. 120-150 mmHg; (diastolic) 90-110 mmHg vs. 70-90 mmHg] and reduction in number of anti-hypertensive medications (from 3-5 to 2 classes of drugs). BAVS with glucagon stimulation is a valuable tool in the identification of the dominant adrenal to be removed in patients with bilateral pheochromocytoma to alleviate chronic hypertension.