Acute abdomen from ruptured adrenal pheochromocytoma: case report

The pheochromocytoma is a very rare neoplasm, which originates in 98% of cases in the adrenal medulla; it is often bilateral in familial syndromes. It is more frequent in syndromes like MEN2, von Hippel-Lindau disease, and neuofribromatosis type 1. In this article the Authors report a case of a young woman with a large adrenal pheochromocytoma, that presented by an acute abdomen; the treatment was explorative laparotomy with unilateral adrenalectomy. Therapy of this tumour is founded on surgery, plus chemiotherapy radiotherapy or treatment with 131I-MIBG (iodine-131-metaiodobenzylguanidine in malignant cases (10%). According with the absence of a correlation between pathological findings and clinical behaviour, a long-term follow up is indispensable.     

Metastatic pheochromocytoma associated with multiple endocrine neoplasia syndrome type II.

Pheochromocytoma is a rare adrenal medullary tumor of chromaffin cell origin that presents a syndrome of alpha- and beta-adrenergic receptor stimulation due to secretion of epinephrine and norepinephrine. This tumor occurs sporadically in the population and is also associated with multiple endocrine neoplasia syndrome type II (MEN II). Although malignant pheochromocytoma is associated with sporadic tumors, those associated with familial MEN syndromes are rarely malignant. We report a case of a rare metastatic pheochromocytoma in a patient with MEN IIA. Surgical debulking, which has been shown to benefit many patients with metastatic neuroendocrine tumors, was attempted in this patient. Palliation, with symptom relief, was provided. The options for treatment of metastatic pheochromocytoma are discussed.     

Surgical management of hereditary pheochromocytoma

BACKGROUND: Surgical treatment of hereditary pheochromocytoma remains controversial because of the need for lifelong corticosteroid therapy and the risk of Addisonian crisis associated with bilateral total adrenalectomy. We examined our large series of patients with hereditary pheochromocytoma to evaluate postsurgical outcomes, particularly in those who underwent cortical-sparing adrenalectomy. STUDY DESIGN: We retrospectively reviewed the outcomes of all patients with histopathologic diagnoses of hereditary pheochromocytoma treated at our institution from 1962 to 2003. Familial disease was initially determined by pedigree analysis, genetic testing, or both for multiple endocrine neoplasia (MEN) types 1, 2A, or 2B; von Hippel-Lindau disease (VHL); neurofibromatosis type 1 (NF-1); or familial paraganglioma syndrome (FP). RESULTS: Adrenal pheochromocytomas were present in 56 of 59 patients (95%): MEN2A (39), MEN2B (7), VHL (6), MEN1 (2), NF-1 (2). Paragangliomas (extraadrenal pheochromocytomas) were present in the remaining 3 of 59 patients (5%): FP (2) and NF-1 (1). Thirty-eight of 56 patients with pheochromocytomas had cumulative operations resulting in total or subtotal bilateral adrenalectomy. Acute adrenal insufficiency (Addisonian crisis) occurred in 4 of these 38 patients (11%). Cortical-sparing adrenalectomy was performed in 26 patients who underwent bilateral adrenal resection; 17 (65%) were corticosteroid independent at a median followup of 71 months. Recurrent pheochromocytoma developed in an adrenal remnant in 3 of 30 patients (10%) who underwent unilateral or bilateral cortical-sparing procedures. Metastatic disease did not develop in any patient with pheochromocytoma, but has occurred in two of three patients with paragangliomas. CONCLUSIONS: Our data suggest that a cortical-sparing adrenalectomy can successfully avoid the need for corticosteroid replacement in the majority of patients who undergo a bilateral adrenalectomy. Long-term followup should include monitoring of the remnant gland for recurrent pheochromocytoma with yearly biochemical screening studies.     

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.     

Adrenalectomy for familial pheochromocytoma in the laparoscopic era

OBJECTIVE: To report the results of treatment of patients with familial pheochromocytomas in the laparoscopic era. SUMMARY BACKGROUND DATA: The optimal surgical management of pheochromocytomas that arise in familial neoplasia syndromes may be complicated by bilateral involvement and associated endocrinopathies. METHODS: Twenty-one patients with familial pheochromocytomas (15 with multiple endocrine neoplasia [MEN] 2A, 4 with MEN 2B, 1 each with von Hippel-Lindau and neurofibromatosis type 1) underwent adrenalectomy between December 1993 and July 2001. Clinical, biochemical, and pathologic data were obtained by retrospective review of perioperative medical records, postoperative biochemical testing, and patient questionnaire. RESULTS: Mean age at diagnosis was 37 +/- 11 years. Twenty of the 21 patients had elevated urine catecholamines, and all had radiographic evidence of an adrenal tumor or tumors. Pheochromocytoma-related symptoms were present in 11 patients (52%). One patient with MEN 2B underwent open adrenalectomy due to previous adrenal surgery and megacolon. Laparoscopic adrenalectomy was attempted in the remaining 20 patients (9 right, 11 left, 2 bilateral). Two patients (9.1%) were converted to open adrenalectomy. Intraoperative hypertensive episodes occurred in 15 patients (71%) and were easily controlled medically. Mean operative time was 216 +/- 57 minutes, mean postoperative length of stay was 3.1 +/- 1.3 days, and mean tumor size was 3.1 +/- 1.0 cm. Minor complications occurred in three patients (14.3%) and major complications in two patients (9.5%). During a mean follow-up of 57 months, a contralateral pheochromocytoma developed in four patients with MEN 2 (33%); three of them underwent adrenalectomy. There have been no long-term complications related to hypertension or adrenalectomy. CONCLUSIONS: This study is the largest series of patients with familial pheochromocytoma undergoing adrenalectomy during the laparoscopic era. The results suggest that the laparoscopic approach is safe and effective for managing unilateral or bilateral adrenal medullary disease in this population.     

Diagnosis and treatment of pheochromocytoma

Early detection of a pheochromocytoma is necessary to avoid the cardiovascular complications of hypertension and to recognize the 10% of malignant neoplasms. Hypertensive changes, particularly hypertensive spikes and stable hypertension are the most common clinical features. A pheochromocytoma must be suspected in presence of various symptoms of which cephalea, tachycardia, diaphoresis are the most frequently encountered. Plasma catecholamines and 24-hours urine metabolites measurements have 60-100% and 72-99% sensitivity respectively. Radiologic procedures commonly used to locate a pheochromocytoma include CT and RM: they achieve 100% sensitivity for unilateral adrenal lesions and respectively 64% and 88% sensitivity for extra-adrenal lesions. Scintigraphic localization with MIBG provides functional informations and is recommended for follow-up of the recurrent or metastatic pheochromocytoma. Preoperative medical treatment using alpha and beta blocking agents or calcium channel blockers has contributed to reduce perioperative morbidity and mortality. Anterior transabdominal approach is advocated as the standard operative procedure; this approach allows ready access to any site where tumors are obviously present and permits thorough exploration of the abdominal cavity for additional contralateral adrenal or extra-adrenal lesions. An accurate preoperative localization of a sporadic, unilateral, of less than 7-10 cm, benign neoplasm constitutes the indication for the laparoscopic adrenalectomy. Pheochromocytoma-free survival were 92% and 80% at 5 and 10 years respectively; in living patients without recurrence, hypertension-free survival was 74% and 45% at 5 and 10 years respectively. Extra-adrenal pheochromocytomas are frequently malignant (36%) and are associated with a high incidence of persistent or recurrent disease (32%).     

Familial pheochromocytoma

OBJECTIVES: To identify disease characteristics, operative outcome, and prognosis in patients with familial pheochromocytoma. METHODS: Records of 29 patients with familial pheochromocytoma were retrospectively analyzed. Patients' age, gender, presenting symptoms and clinical signs, chemical and radiologic findings, and associated conditions were recorded. RESULTS: Of the 29 patients, 25 were adults and 4 were children. Age ranged from 18 to 52 years (mean age 30.8 years). Twenty patients were females. Of the 29 patients, 26 had adrenal tumors, 2 had extra-adrenal tumors, and 1 had a combined adrenal and extra-adrenal tumor. The patients included 17 with multiple endocrine neoplasia (MEN), 8 with von Hippel Lindeau disease (vHLD), and 4 with Von Recklinghausen disease (vRD). Two patients had malignant pheochromocytoma. All except one patient with MEN had thyroid carcinoma. The two patients with malignant pheochromocytoma had MEN II. All patients were managed by either open or laparoscopic adrenalectomy or excision of extra-adrenal tumors. There was no perioperative mortality. CONCLUSIONS: Associated conditions in patients with familial varieties are often the dominant or initial presentation. Presence of other manifestations of familial pheochromocytoma significantly influenced the clinical course and prognosis. However, it had no bearing on operative outcome of these patients.     

Can pheochromocytoma be considered a benign unilateral intra-adrenal sporadic hypertensive tumor ? Reflections on a series of 105 surgically treated subdiaphragmatic chromaffin tumors]

In a 20 year period, from 1971 through 1991, 105 chromaffin tumours--excluding cervical ones--were operated by the same surgeon: 50 during the first 15 years and 55 during the last 5 years. Pheochromocytomas are defined as intra-adrenal chromaffin tumours, and paragangliomas--or ectopic pheochromocytomas--as of extra-adrenal location. Among those tumours, 30 were malignant (i.e. metastatic) and 75 benign. Among the 30 malignant tumours, 14 were ectopic, 2 occurred in a MEN II A setting and were bilateral, 2 were associated with liver adenoma and liver hemangioma respectively suggesting Von Hippel-Lindau syndrome, and one case was associated with a seemingly sporadic primary hyperparathyroidism. 9 out of those 30 malignancies were not associated with hypertension. Among 75 benign pheochromocytomas or paragangliomas, 10 were ectopic, 7 occurred in a MEN setting (6 type II, 1 type I). 3 patients without evidence of MEN or other neuroectodermal abnormalities presented bilateral pheochromocytoma, either synchronous (2) or metachronous (1). 7 cases occurred in a Von Hippel-Lindau syndrome (3 bilateral) and 4 in a neurofibromatosis setting (1 bilateral). 3 other cases were familial without evidence of MEN (including a case of triple tumour: bilateral and ectopic and another ectopic case). 2 other cases were associated with seemingly sporadic hyperparathyroidism. As a whole, in 34 of 75 benign pheochromocytomas or paragangliomas, the tumour was not intra-adrenal, unilateral and sporadic. Among those 75 tumours, 22 were not overtly hypertensive, including 10 out of the 41 seemingly intra-adrenal, solitary and sporadic. The pheochromocytoma, benign, intra-adrenal sporadic, hypertensive accounts for no more than 30% of the subphrenic catecholamine-secretin chromaffin tumours.(ABSTRACT TRUNCATED AT 250 WORDS)     

Management of pheochromocytomas in patients with multiple endocrine neoplasia type 2 syndromes.

OBJECTIVE: The authors sought to determine the optimal surgical management of pheochromocytomas that develop in patients with multiple endocrine neoplasia (MEN) type 2 syndromes. SUMMARY BACKGROUND DATA: The performance of empirical bilateral adrenalectomy in patients with MEN 2A or MEN 2B, whether or not they have bilateral pheochromocytomas, is controversial. METHODS: The results of unilateral or bilateral adrenalectomy were studied in 58 patients (49 with MEN 2A and 9 with MEN 2B). Recurrence of disease was evaluated by measuring 24-hour urinary excretion rates of catecholamines and metabolites and by computed tomography (CT) scanning. RESULTS: The mean postoperative follow-up was 9.40 years. There was no operative mortality and malignant or extra-adrenal pheochromocytomas were not present. Twenty-three patients with a unilateral pheochromocytoma and a macroscopically normal contralateral gland underwent unilateral adrenalectomy. A pheochromocytoma developed in the remaining gland a mean of 11.87 years after the primary adrenalectomy in 12 (52%) patients. Conversely, 11 (48%) patients did not develop pheochromocytoma during a mean interval of 5.18 years. In the interval after unilateral adrenalectomy, no patient experienced hypertensive crises or other complications related to an undiagnosed pheochromocytoma. Ten (23%) of 43 patients having both adrenal glands removed (either at a single operation or sequentially) experienced at least one episode of acute adrenal insufficiency or Addisonian crisis, including one patient who died during a bout of influenza. CONCLUSIONS: Based on these data, the treatment of choice for patients with MEN 2A or MEN 2B and a unilateral pheochromocytoma is resection of only the involved gland. Substantial morbidity and significant mortality are associated with the Addisonian state after bilateral adrenalectomy.     

Pheochromocytoma: inherited associations, bilaterality, and cortex preservation

BACKGROUND: Hereditary pheochromocytoma (HP) is characterized by early onset, bilateral adrenal involvement, low malignancy rate, and genetic linkage with certain familial syndromes. This retrospective review is intended to show the high yield of surveillance, predictable bilaterality, and the challenge of cortex-sparing adrenalectomy. METHODS: From 1964 to 1999, 32 patients with HP were treated at a single institution and followed for a mean of 7 years. There were 15 cases of multiple endocrine neoplasia type 2A (MEN 2A), 12 cases of von Hippel-Lindau (VHL) disease, 3 cases of von Recklinghausen's disease (VRD), and 2 cases of familial pheochromocytoma. Twenty-four of 32 patients underwent bilateral adrenalectomy (9 metachronous). Subtotal resection with orthotopic cortex preservation was performed in 5 patients, and heterotopic autografting was performed in 14 patients. RESULTS: Pheochromocytoma was the first manifestation in 50% of patients with VHL disease and in 27% of patients with MEN 2A. Surveillance uncovered medullary thyroid cancer in 5 of 15 patients with MEN 2A and hemangioblastomas, renal cell carcinoma, and islet cell tumors in 7 of 15 patients with VHL disease and VRD. HP was bilateral in 24 of 32 patients (14/15 in patients with MEN 2A, 7/12 in patients with VHL disease, 2/3 in patients with VRD, and 1/2 in patients with familial pheochromocytoma). In 9 cases of metachronous adrenalectomy, the mean interval was 67 months (range, 9-156 months). Three of 5 patients who underwent orthotopic preservation of the adrenal cortex experienced recurrence compared with 0 of 14 patients with heterotopic autotransplantation of cortical tissue. CONCLUSIONS: Pheochromocytoma frequently heralds coexisting silent VHL disease or MEN-2, mandating surveillance for inherited associations. The long interval of metachronous pheochromocytoma argues against prophylactic removal of the contralateral "normal" adrenal gland. Total adrenalectomy and heterotopic autotransplantation of medulla-free cortex may diminish the need for lifelong steroid substitution and eliminates recurrence.     

肾上腺嗜铬细胞瘤的诊断与手术治疗

目的：提高肾上腺嗜铬细胞瘤的诊断与外科治疗水平。方法：对109例经手术治疗的肾上腺嗜铬细胞瘤患者的临床资料进行回顾性分析，其中具有典型儿茶酚胺症临床表现者104例，24h尿VMA定性检查106例，99例增高，阳性率为90．8％，B超、CT及MRI定位诊断阳性率分别为89．0％、92．7％及100％。106例经开放手术切除肿瘤，3例行后腹腔镜肿瘤切除术。结果：经病理检查证实，良性102例(93．6％)，恶性7例(6．4％)，本组无手术死亡病例。结论：肾上腺嗜铬细胞瘤主要根据临床表现、生化定性检查及影像学定位检查明确诊断。手术切除肿瘤是唯一有效的措施，术前充分准备是确保手术成功的重要因素。应用腹腔镜技术切除肾上腺肿瘤具有广阔的应用前景。     

Clinical observation of pheochromocytoma

Eleven cases of pheochromocytoma observed at our department between 1976 and 1985 are presented. There were 5 males and 6 females and they were between 18 and 59 years old. The site of the tumor was in the right adrenal in 4 cases, left adrenal in 3 cases, bilateral adrenals in 2 cases and extra adrenal in 3 cases, 1 of which had multiple lesions and involvement of the right adrenal. Clinical symptoms observed were hypertension in 10 cases, headache in 7 cases, palpitation in 3 cases and nausea in 2 cases. Atypical adrenal pheochromocytoma was seen in 1 case. Definitive diagnosis was established by determination of urinary catecholamine levels in the 24-hour sample. Urinary levels of catecholamine revealed higher adrenaline levels for paroxysmal type and higher nor-adrenaline levels for extra-adrenal cases. For localization of tumors, computed tomography was most useful with a diagnostic rate of 100%, followed by ultrasonography and adrenal scan. As preoperative treatment, blood transfusion and administration of adrenergic blocking agents were performed in 9 cases. In all but 2 cases, hypertension was improved and no recurrence was seen after the operation.     

Diagnosis and treatment of pheochromocytoma. Report of 49 cases

From 1958 to 1990, 49 patients with pheochromocytoma were treated surgically. The tumors were located in the adrenal gland in 34 cases (69.4%) and in the extra-adrenal gland in 15 cases (30.6%). In two cases the tumors were pheochromoblastoma and in 6 cases the tumors were asymptomatic. Determination of urine VMA has a screening value for patients with pheochromocytoma. The corresponding rate was 91.3% by B-ultrasonography to localize the tumor. CT scanning can clearly identify pheochromocytoma of its size and relation to the adjacent organs, which may provide a basis for the selection of operative methods. Administration of alpha-adrenoceptor blocking drugs and expanding blood volume are very important procedures, which can effectively reduce the mortality rate.     

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.     

Laparoscopic surgery for pheochromocytoma

OBJECTIVE: To evaluate the feasibility of laparoscopic adrenalectomy for pheochromocytoma. MATERIAL: Between January 1998 and March 2002, 12 patients aged from 19 to 76 (average age 54 years) underwent 13 adrenalectomies (7 right and 6 left, 1 bilateral) using laparoscopic surgery. A specific anti-hypertensive preparation was begun prior to the operation. Peaks of blood pressure were treated by bolus Nicardipine and sinus tachycardia by bolus Esmolol. Catecholamines were dosed at different times during the intervention. As far as surgery was concerned, the adrenalectomies were performed 11 times using the transperitoneal route and twice using the retroperitoneal route. The adrenal vein was found and ligatured before manipulation of the adrenal gland. RESULTS: Average length of operation was 127 minutes (75 to 195). Average blood loss was 105 ml (0 to 1000). Catecholamines dosed throughout showed a variable increase in plasma rates during peritoneal insufflation and manipulation of the gland. They were responsible for 5 cases of hypertensive bouts and 2 cases of tachycardia which were treated with medication. No surgical conversions were necessary. Average length of hospital stay was 4.18 days (3 to 6); average size of adrenal tumours was 44 mm (30 to 72); average follow-up, 18.4 months. CONCLUSION: This study showed the feasibility of adrenalectomy for pheochromocytoma using laparoscopic surgery, subject to specific medical preparation to reduce the consequences of peroperative bouts of hypertension and sinus tachycardia during peritoneal insufflation and manipulation of the adrenal gland despite initial ligature of the main adrenal vein.     

嗜铬细胞瘤临床诊治

目的 提高嗜铬细胞瘤的诊治水平。方法 总结１９５８年至１９９８年间经手术和病理证实的嗜铬细胞瘤２８６例。结果 本组２２０例患者肿瘤位于肾上腺,６６例位于肾上腺外。在２９例恶性嗜铬细胞瘤中,７例位于肾上腺,２２例位于肾上腺外。１９例为静止型嗜铬细胞瘤。手术切除肿瘤２８１例。结论 对潜在的儿茶酚胺性心肌病的防治可降低手术死亡率。恶性嗜铬细胞瘤的诊断要靠长期严密的随访。     

Estimated risk of pheochromocytoma recurrence after adrenal-sparing surgery in patients with multiple endocrine neoplasia type 2A

HYPOTHESIS: Adrenal-sparing adrenalectomy is considered the treatment of choice for hereditary bilateral pheochromocytoma in patients with multiple endocrine neoplasia type 2A (MEN 2A). DESIGN: Retrospective analysis of prospectively documented data with a mean +/- SD follow-up of 81.5 +/- 85.3 months. The PubMed database was searched for articles published between 1975 and 2004 to identify published series and/or case reports. SETTING: University hospital referral center. PATIENTS: In 17 (22%) of 77 patients with various mutations of the RET proto-oncogene, unilateral (n = 12) or bilateral (n = 5) pheochromocytomas were documented at the time of diagnosis or during the course of MEN 2A. Adrenal-sparing surgery was performed in 13 patients (group 1), synchronous bilateral total adrenalectomy in 4 patients (group 2A), and metachronous bilateral total adrenalectomy in 5 patients after adrenal-sparing adrenalectomy (group 2B). MAIN OUTCOME MEASURES: Measurement of 24-hour urinary catecholamine levels (noradrenaline, adrenaline, and dopamine) and, in case of high catecholamine levels, imaging studies to localize the tumors in 1 or both adrenal glands to determine the size and exclude extra-adrenal tumors and distant metastasis. RESULTS: The mean+/-SD estimated 5- and 10-year cumulative risk of developing recurrence in both groups was 38.5% +/- 15.7%. Five (38%) of 13 patients in group 1 developed recurrence in the contralateral gland. Two (22%) of 9 patients in groups 2A and 2B developed several episodes of an addisonian crisis, 1 of whom died. CONCLUSIONS: Substantial morbidity and mortality are associated with addisonian crisis after bilateral adrenalectomy. Adrenal-sparing adrenalectomy and close monitoring of the remnant may be the treatment of choice for hereditary bilateral pheochromocytoma in MEN 2A, since overall recurrence is low.     

Familial pheochromocytoma: a report of 2 cases in a kindred.

A kindred with 2 current cases of pheochromocytoma is reported. The proband had classical features of adrenal medullary hyperfunction, in addition to Raynaud's phenomenon. After surgical removal of the right adrenal gland, containing a pheochromocytoma and a small paraganglioma, the patient was free of symptoms. The 12-year-old son of the proband was discovered to have relatively asymptomatic sustained hypertension on routine examination. Biochemical and radiological tests confirmed the diagnosis of a left adrenal pheochromocytoma, which was removed successfully.     

肾上腺外嗜铬细胞瘤

目的 提高肾上腺外嗜铬细胞瘤的诊治水平。方法 总结１９５８￣１９９８年收治的肾上腺外嗜铬细胞瘤６６例,占同期嗜铬细胞瘤总数的２３．３％。结果 肾上腺外嗜铬细胞瘤恶性比例高,本组中３３％为恶性嗜铬细胞瘤。结论 １３１Ｉ－ＭＩＢＧ在肾上腺外嗜铬细胞瘤的诊治与随访中有重要意义,肾上腺外嗜铬细胞瘤术后需长期、严密随访。     

Asynchronous pheochromocytoma in childhood

A case of asynchronous pheochromocytoma in a 13-year-old boy is reported. Two years elapsed between excision of the right adrenal gland and the appearance of another tumour in the contralateral gland. Selective central venous sampling for plasma catecholamine measurement and adrenal phlebography were of diagnostic value. Excision of the tumour with partial adrenalectomy was the preferred surgical treatment in the remaining contralateral adrenal; no clinical or biochemical abnormalities were observed 2 years after removal of the second pheochromocytoma. This case stresses the importance of a closer follow-up in children for early detection of a second pheochromocytoma.