异位嗜铬细胞瘤的诊断与治疗

目的 总结异位嗜铬细胞瘤的临床特点,增进对本病的认识,提高诊断和治疗水平.方法 回顾性分析天津医科大学总医院1990年至2010年36例经病理证实为异位嗜铬细胞瘤的临床资料.就诊原因:高血压28例,排尿终末血压升高5例,查体发现1例,腹痛就诊2例.36例均检测24 h尿香草基扁桃酸(VMA),VMA异常升高阳性率为88.9%(32/36).经腹部B超、CT、MRI及131碘-间位碘代苄胍(131 I-MIBG)等检查进行定位.36例均行手术治疗,4例行腹腔镜手术.结果 本组异位嗜铬细胞瘤单发34例,多发2例.手术切除肿瘤直径为3.4～18.2 cm.术后病理证实均为嗜铬细胞瘤,其中恶性嗜铬细胞瘤9例.结论 VMA是异位嗜铬细胞瘤定性诊断的主要依据,131 I-MIBG定位、定性准确可靠.充分的术前准备是手术成败的关键.手术切除肿瘤是最佳治疗方法.腹腔镜手术治疗异位嗜铬细胞瘤也成为一种术式选择.恶性异位嗜铬细胞瘤术后可给予131 I-MIBG辅助治疗.     

131I-MIBG肾上腺髓质扫描对嗜铬细胞瘤的诊断价值

目的：评价^131I—MIBG肾上腺髓质扫描在嗜铬细胞瘤的诊断价值。方法：16例患者术前均经B超检查及^131I—MIBG肾上腺髓质扫描，10例术前行CT检查。结果：14例患者经^131I—MIBG肾上腺髓质扫描直接提示病灶部位核素浓聚，其中包括6例血压正常或临界正常患者。B超检查提示嗜铬细胞瘤不能排除诊断5例，CT检查提示嗜铬细胞瘤可能4例。本组^131I-MIBG肾上腺髓质扫描敏感性87．5％，特异性100％。结论：^131I-MIBG肾上腺髓质扫描对嗜铬细胞瘤的诊断价值在于其可直接反映可疑肿瘤的嗜铬性质，具有高效、可靠的优点，应作为嗜铬细胞瘤的首选功能性诊断手段。     

"Silent" pheochromocytomas

The results of surgical treatment of 126 patients with "hormonally inactive" and catecholamine secreting tumors of the adrenals were studied. Among them 2 cases (1.6%) of "dumb" pheochromocytoma were diagnosed. The clinical observations have shown the difficulties in the diagnosis of "dumb" pheochromocytoma before operation, risk of performing adrenalectomy and necessity to correct hemodynamic disorders during anesthesia in connection with latent catecholamine activity. The laboratory and instrumental means of examination of patients with suspected "hormonally inactive" tumor of the medullary substance of the adrenal are proposed. The variants of prevention and arrest of hemodynamic disorders during ablation of the "dumb" pheochromocytoma were considered. When the "dumb" pheochromocytoma had the diameter less than 5 cm and the adequate preparation was conducted the authors propose a laparoscopic access for adrenalectomy on the right, and retroperitoneoscopic access on the left. The detection of the catecholamine secreting tumor of more than 5 cm diameter, when problems with the clipping of the central vein of the adrenal take place, open accesses should be preferred--mainly thoracophrenotomy in the X intercostal space. If it was not possible to prove "dumb" pheochromocytoma before operation and it was started with endovideosurgical intervention during which it was not possible to first clip the central vein of the adrenal and the risk of hemodynamic disorders was high, the early transition to open operative intervention is thought to be expedient.     

Elective Resection of Pheochromocytoma with Concomitant Abdominal Aortic Aneurysm Repair: Report of a Case

We report a rare case of coexistent pheochromocytoma and abdominal aortic aneurysm (AAA). The patient was a 72-year-old man who presented to the Emergency Department with severe dyspnea and hypertension. Physical examination revealed a pulsatile mass in his abdomen. A computed tomography scan, done after pharmacological treatment of his symptoms, showed a thoraco-AAA and a left adrenal mass. His urinary vanylmandelic acid and metanephrine levels were found to be elevated, strongly indicative of a diagnosis of pheochromocytoma. We gave the patient phenoxybenzamine, an α-blocker, preoperatively, then resected the adrenal mass and repaired the AAA. The patient had an uneventful recovery. The diagnosis of pheochromocytoma was confirmed histologically. To our knowledge, this is the first reported case of elective resection of both these lesions.     

Metachronous pheochromocytoma metastasis to the upper dorsal spine—6-year survival

BACKGROUND CONTEXT: Malignant pheochromocytoma is a rare neoplasm of chromaffin tissue. Very few cases of malignant adrenal pheochromocytoma metastatic to vertebrae exist. PURPOSE: To determine the prognosis of a patient with an excised adrenal pheochromocytoma and a single metachronous metastasis to the upper dorsal spine. STUDY DESIGN: Case report METHODS: The authors report a patient who underwent total excision of an adrenal pheochromocytoma of the left adrenal gland in 2000 who developed a single metastasis to the second dorsal vertebra in 2002 with no evidence of abdominal recurrence. RESULTS: Four-year survival is documented after the spinal metastasis was first detected after two attempts at excision and radiotherapy. CONCLUSIONS: Patients with adrenal pheochromocytomas must be screened periodically with whole body imaging despite normal abdominal imaging as there is a definite risk of metachronous metastasis. Aggressive therapy may result in improving survival significantly in a subset of patients with isolated spinal metastases.     

嗜铬细胞瘤手术切口的选择（附１０３例报告）

目的：提高嗜铬细胞瘤手术治疗水平,方法：回顾性分析１０３例嗜铬细胞瘤患者手术切口的选择方法及选择原则。结果：４９例行１１肋间切口,１８例行１０肋间切口,１５例行腹剖正中切口,９例行胸腹联合切口,5例行腹部肋下切口,３例行下腹正中切口,３例后外侧切口,１例行腹腔镜手术,结论：（１）大部分肾上腺嗜铬细胞瘤宜用１１肋间切口１０肋间切口,(2) 大嗜铬细胞瘤或与大血管关系紧密的嗜铬细胞宜用腹剖正中切口,甚至胸腹联合切口,（３）多发性嗜铬细胞瘤肾上腺外嗜铬细胞瘤以腹部正中切口较为适用。     

A case of pheochromocytoma with von Recklinghausen's and review of 67 Japanese cases

A 19-year-old woman with von Recklinghausen's disease was referred to our hospital because of right adrenal pheochromocytoma. The tumor was detected incidentally with the abdominal ultrasonography when she complained epigastralgia to the home doctor who treated her hypertension. Plasma and urinary catecholamines level were elevated. The tumor was removed by laparoscopy assisted adrenalectomy without pneumoperitoneum. The resected specimen was 35 x 60 x 75 mm in size and weighed 70 g. Pathological diagnosis was adrenomedullary pheochromocytoma. Postoperative course was uneventful. She has been well with no signs of recurrence after 7.5 years. We reviewed 67 Japanese patients previously reported as von Recklinghusen's disease with pheochromocytoma. Of the 60 patients whose details were described, 16.7% had metastases and pathological malignancy from pheochromocytoma.     

Spontaneous adrenal pheochromocytoma rupture complicated by intraperitoneal hemorrhage and shock

MEN2A is a hereditary syndrome characterized by medullary thyroid carcinoma, hyperparathyroidism, and pheochromocytoma. Classically patients with a pheochromocytoma initially present with the triad of paroxysmal headaches, palpitations, and diaphoresis accompanied by marked hypertension. However, although reported as a rare presentation, spontaneous hemorrhage within a pheochromocytoma can present as an abdominal catastrophe. Unrecognized, this transformation can rapidly result in death. We report the only documented case of a thirty eight year old gentleman with MEN2A who presented to a community hospital with hemorrhagic shock and peritonitis secondary to an unrecognized hemorrhagic pheochromocytoma. The clinical course is notable for an inability to localize the source of hemorrhage during an initial damage control laparotomy that stabilized the patient sufficiently to allow emergent transfer to our facility, re-exploration for continued hemorrhage and abdominal compartment syndrome, and ultimately angiographic embolization of the left adrenal artery for control of the bleeding. Following recovery from his critical illness and appropriate medical management for pheochromocytoma, he returned for interval bilateral adrenal gland resection, from which his recovery was unremarkable. Our review of the literature highlights the high mortality associated with the undertaking of an operative intervention in the face of an unrecognized functional pheochromocytoma. This reinforces the need for maintaining a high index of suspicion for pheochromocytoma in similar cases. Our case also demonstrates the need for a mutimodal treatment approach that will often be required in these cases.     

Adrenal incidentaloma, borderline elevations of urine or plasma metanephrine levels, and the "subclinical" pheochromocytoma

OBJECTIVE: To assess the risk of pheochromocytoma in patients with borderline-elevated urine or plasma metanephrine levels. DESIGN: Retrospective review. SETTING: University tertiary care center. PATIENTS: Forty-two consecutive patients with adrenal incidentalomas (defined as adrenal tumors identified during routine imaging for another condition) who were treated at the UCSF (University of California, San Francisco) Medical Center between January 1, 1995, and July 31, 2005. Patients with genetic syndromes were excluded. INTERVENTION: Laparoscopic adrenalectomy for adrenal incidentaloma based on size criteria and preoperative hormonal test results. MAIN OUTCOMES MEASURES: Urine or plasma metanephrine and catecholamine levels, tumor size, and presence of pheochromocytoma. RESULTS: Of 42 patients, 14 (33%) had a pheochromocytoma (11 of whom had clear-cut elevations in urine or plasma metanephrine levels defined as greater than 2 times the upper limit of normal) and 28 did not. Ten of the 42 patients (24%) had borderline elevations in urine or plasma metanephrine levels (defined as 1-2 times the upper limit of normal), 3 of whom had a pheochromocytoma (30%). Of patients with borderline elevations, mean +/- SD tumor size was 5.4 +/- 3.1 and 4.8 +/- 1.9 cm for patients with and without pheochromocytoma, respectively (P = .37). In these 10 patients, no clinical factors (age, sex, hypertension, presence of symptoms, number of antihypertensive medications, preoperative hemodynamics, or size of tumor on computed tomographic scan) allowed differentiation between those with and without pheochromocytoma. CONCLUSIONS: Thirty percent of patients with adrenal incidentaloma and borderline-elevated urine or plasma metanephrine levels had a pheochromocytoma. Clinical factors cannot distinguish between those with and without pheochromocytoma. In this group of patients, we advocate either routine alpha-blockade preoperatively or further diagnostic tests to better characterize the tumor.     

Normotensive Pheochromocytoma: Institutional Experience

We report an audit of nine cases of subclinical or normotensive pheochromocytoma managed in our department. This is the first report from India of such a series. During the period 1990–2003 a total of 45 patients of pheochromocytoma were diagnosed and managed in the Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences. Among them, nine patients were diagnosed as having subclinical or normotensive pheochromocytoma. Inclusion criteria for normotensive pheochromocytoma were: no previous history of hypertension clinically and, in the previous treatment documents, any episode of symptoms suggesting high blood pressure. The demographic profile, clinical presentation, biochemical investigations, imaging results, surgical notes, and follow-up record were reviewed retrospectively. All patients had a primary complaint of flank/abdominal pain and were normotensive. Seven had elevated urinary metanephrine levels, and one patient had normal values. One patient did not undergo the urinary metanephrine assay. Imaging of the abdomen showed seven adrenal and two extra-adrenal masses (eight had computed tomography scans of the abdomen, and one underwent ultrasonography). After selective α-adrenergic blockade (prazosin), surgery was performed. Six patients required infusion of sodium nitroprusside intraoperatively. The final histopathology was pheochromocytoma in all patients. Metanephrine levels were normal during the follow-up. Normotensive pheochromocytomas are a distinct entity, and all adrenal incidentalomas should be investigated for catecholamine hypersecretion. We support the use of preoperative α-adrenergic blockade, and these patients should be treated along the same lines as hypertensive pheochromocytoma.     

嗜铬细胞瘤手术径路分析

目的 探讨高嗜铬细胞瘤手术治疗方法。方法 分析嗜铬细胞瘤８９例的手术方法所取径路。结果 ３９例行第１肋间切口,１５例行第１０肋间切口,１４例行腹部正中切口,９例行胸腹联合切口,５例行腹部肋下斜切口,３例行下腹正中切口,３例行后外侧切口。１例行腹腔镜手术。结果 （１）大部分定位明确肾上腺嗜铬细胞瘤,拟用１１肋间切口或１０肋间切口。（２）巨大嗜铬细胞瘤或大血管关系紧密的嗜铬细胞瘤,拟用腹部正中切口,必     

Extra-adrenal pheochromocytoma causing renal artery stenosis and implications of magnetic resonance imaging as diagnostic tool

Extra-adrenal pheochromocytoma causing renal artery stenosis is very uncommon. We describe a patient who had hypertension from this entity. Surgical excision of the mass corrected the renal artery stenosis without the need for bypass grafting, and the patient has remained normotensive. Magnetic resonance imaging (MRI) showed an extremely bright lesion on T2 weighted image, which is virtually pathognomonic. MRI is the most sensitive modality for the detection of an extra-adrenal or unsuspected multiple pheochromocytoma. This should obviate the need for routine abdominal exploration in the evaluation and treatment of pheochromocytoma. Therefore we strongly recommend magnetic resonance imaging in the management of adrenal masses in general and pheochromocytomas in particular.     

A case of composite pheochromocytoma-ganglioneuroblastoma in the adrenal gland with primary hyperparathyroidism

We report a case of composite pheochromocytoma-ganglioneuroblastoma in the adrenal gland with primary hyperparathyrodisim. A 55-year-old woman consulted our hospital for an examination of a right adrenal tumor, incidentally found by screening abdominal ultrasound sonography. On the clinical diagnosis of pheochromocytoma in the right adrenal gland from the findings of enhanced abdominal computed tomography, endocrinal examinations and 123I-metaiodobenzyl-guanidine scintigram, right adrenalectomy was performed transperitoneally. Histopathological diagnosis was an adrenal composite pheochromocytoma-ganglioneuroblastoma. This combination of compound adrenal tumor is extremely rare, and to date this case may be the seventh reported in Japan. Moreover, since her serum calcium level and intact parathyroid hormone level were high, so we considered the existence of multiple endocrine neoplasia type 2A. 99mTc-methoxyisobutylisonitrile scintigram and ultrasound of the neck revealed hyperparathyroidism, but medullary thyroid carcinoma was not detected. One year later, she was readmitted for parathyroid tumor excision, and histopathological finding was parathyroid adenoma. We concluded that she had both adrenal composite pheochromocytoma and hyperparathyroidism incidentally.     

Adrenal oncocytic pheochromocytoma

We report a case of adrenal oncoctyic pheochromocytoma in a 37-year-old woman. The patient presented with a 2-year history of an increase in abdominal girth. Computed tomographic studies revealed a large left adrenal mass, which was subsequently excised. Grossly, the tumor measured 17 x 14 x 8.5 cm, weighed 1,150 g, and had a solid, brown cut surface. Histologically, it consisted of large polygonal tumor cells containing eosinophilic granular cytoplasm and arranged in nesting, alveolar, and trabecular patterns. Electron microscopy revealed closely packed mitochondria and dense-core membrane-bound granules in almost all tumor cells. The latter were immunohistochemically positive for chromogranin, synaptophysin, neuron-specific enolase, neurofilament, serotonin, bombesin, ACTH, vimentin, desmin, S-100 protein, and cytokeratins, including AE1/3, CAM 5.2, cytokeratin 7, and cytokeratin 20. To the best of our knowledge, this is the first reported case of adrenal oncocytic pheochromocytoma confirmed by ultrastructural study. The immunoreactivity of this tumor adds several unusual features to the wide immunohistochemical spectrum of pheochromocytoma.     

Familial pheochromocytoma in a daughter (extra-adrenal) and her mother

The nineteenth kindred of familial pheochromocytoma in Japan is reported. A 13-year-old girl had an extra-adrenal pheochromocytoma and her 40-year-old mother had a right adrenal pheochromocytoma. This is the first case of an extra-adrenal pheochromocytoma in familial pheochromocytoma. A statistical analysis was performed on the 46 cases in 19 kindreds reported so far in Japan.     

[11C]Metahydroxyephedrine and [18F]Fluorodeoxyglucose Positron Emission Tomography Improve Clinical Decision Making in Suspected Pheochromocytoma

Background Pheochromocytomas are rare tumors of chromaffin cells for which the optimal management is surgical resection. Precise diagnosis and localization may be elusive. We evaluated whether positron emission tomography (PET) scanning with the combination of [¹⁸F]fluorodeoxyglucose (FDG) and the norepinephrine analogue [¹¹C]metahydroxyephedrine (mHED) would allow more exact diagnosis and localization. Methods Fourteen patients with suspected pheochromocytoma were evaluated by anatomical imaging (computed tomography or magnetic resonance imaging) and [¹³¹I]metaiodobenzylguanidine (MIBG) planar imaging. PET imaging was performed by using mHED with dynamic adrenal imaging, followed by a torso survey and FDG with a torso survey. Images were evaluated qualitatively by an experienced observer. Results Eight patients had pathology-confirmed pheochromocytoma. Of the other six, two patients had normal adrenal tissue at adrenalectomy, and the other four had subsequent clinical courses inconsistent with a diagnosis of pheochromocytoma. In four of eight patients with pheochromocytoma, MIBG failed to detect one or more sites of pathology-confirmed disease. The mHED-PET detected all sites of confirmed disease, whereas FDG-PET detected all sites of adrenal and abdominal disease, but not bone metastases, in one patient. MIBG and FDG-PET results were all negative in the six patients without pheochromocytoma. One patient with adrenal medullary hyperplasia had a positive mHED-PET scan. PET scanning aided the decision not to operate in three of six patients. The resolution of PET functional imaging was superior to that of MIBG. Conclusions PET scanning for pheochromocytoma offers improved quality and resolution over current diagnostic approaches. PET may significantly influence the clinical management of patients with a suspicion of these tumors and warrants further investigation. Presented at the 58th Annual Meeting of the Society of Surgical Oncology, Atlanta, Georgia, March 2005.     

Lethal posttraumatic rupture of a pheochromocytoma: lesson from a postmortem diagnosis

Pheochromocytoma is challenging to diagnose, and a life-threatening situation may occur if pheochromocytoma is incorrectly diagnosed and treated. We report the first case of a lethal posttraumatic rupture of an undiagnosed pheochromocytoma in a 46-year-old man who presented with peripheral vasoconstriction and signs of peritoneal irritation after being kicked by a horse. Computed tomography, arteriography, and two exploratory laparotomies were performed. The patient died of multiorgan failure 8 h after the trauma. The diagnosis of ruptured pheochromocytoma of the left adrenal gland was made during the postmortem examination. Pheochromocytoma should be considered in cases of blunt abdominal trauma associated with unstable blood pressure, peripheral vasoconstriction and adrenal hematoma.     

Composite pheochromocytoma associated with adrenal neuroblastoma in an infant: a case report

A 5-year-old boy presented with headache and hypertension. Screening abdominal ultrasonography showed a 6-cm-sized mass in the left adrenal gland. Laboratory data included urine vanillylmandelic acid/creatinine (208.3 μg/mg), urine homovanillic acid/creatinine (114.3 μg/mg), serum epinephrine (237 pg/mL), and serum norepinephrine (1842 pg/mL). Metaiodobenzyl guanidine I 123 scintigram, abdominal computed tomography, and abdominal magnetic resonance imaging revealed only left adrenal tumor with no metastasis. Left adrenalectomy was performed transperitoneally, and histopathological diagnosis was an adrenal composite pheochromocytoma associated with neuroblastoma. Immunoreactivity for vimentin was positive in pheochromocytoma, which was negative in neuroblastoma. After operation, the symptom of hypertension disappeared. We should examine the identification of composite pheochromocytoma by immunohistochemistry method in patients with neuroblastomatous elements complicated by severe hypertension, and it is important to carefully monitor urine vanillylmandelic acid and urine homovanillic acid in the examination tests and hypertension in the clinical symptoms, and follow up this patient because of some risk of recurrence and metastasis.