Spontaneous ruptured pheochromocytoma: a case report

We present a rare case of intra-abdominal hemorrhage due to a ruptured pheochromocytoma. Our patient presented with signs of shock. By emergency surgery, an hemorrhagic pheochromocytoma of the left adrenal gland was removed. Recovery was uneventful. In cases of suspected hemorrhagic pheochromocytoma with severe shock, prompt surgery is mandatory and catecholamines administration may be crucial to resolve hypotension and guarantee an uneventful recovery.     

Acute abdominal symptoms caused by hemorrhagic necrosis of a pheochromocytoma: Report of a case

The diagnosis of pheochromocytoma is often difficult in a patient presenting with acute illness but without the classical constitutional symptoms. We report herein the case of a woman who developed acute abdominal symptoms following hemorrhagic necrosis of a previously unsuspected pheochromocytoma. This patient underwent successful emergency resuscitation followed later by elective surgical resection of a right adrenal pheochromocytoma. A review of 27 other cases indicates that a fatal outcome is likely to result without the initiation of prompt and appropriate emergency treatment, followed later by removal of the tumor. Thus, early diagnosis is important not only for controlling hypertension, but also because performing a laparotomy on a patient with unrecognized hemorrhagic necrosis of pheochromocytoma can create a potentially lethal situation.     

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.     

腹腔镜肾上腺嗜铬细胞瘤切除术的围手术期护理

目的探讨腹腔镜肾上腺嗜铬细胞瘤患者围手术期护理措施。 方法回顾性分析2012年6月至2017年6月在北京安贞医院收治的56例肾上腺嗜铬细胞瘤行腹腔镜手术治疗的患者临床资料,总结腹腔镜肾上腺嗜铬细胞瘤患者围手术期护理经验。 结果56例患者均康复出院,住院时间(6.9 ± 1.2)d,术后出现低血糖1例、术后腹胀4例、术后皮下气肿6例。无低血容量性休克、心功能衰竭、术后出血及心脑血管事件发生。所有患者术后病理均证实为嗜铬细胞瘤,随访6个月,患者血压恢复至正常范围。 结论对于肾上腺嗜铬细胞瘤患者,完善术前检查,充分术前准备和围手术期管理,加强术后监测和护理,可以有效减少和避免相关并发症的发生。     

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.     

Differences between sporadic pheochromocytoma and pheochromocytoma in multiple endocrime neoplasia, type 2

We studied the gross and microscopic pathology of the adrenal gland in 69 cases of sporadic phenochromocytoma in order to develop a profile of the neoplasm to compare with that of pheochromocytoma observed in the syndrome of multiple endocrine neoplasia, type 2 (MEN 2). The results showed that sporadic pheochromocytoma was a unicentric (93%), unilateral (100%) neoplasm, which was associated with normal extratumoral adrenal medulla (100%). The findings contrast with those encountered in the adrenal gland in MEN 2, in which the tumor involvement is frequently multicentric, usually bilateral, and associated with extratumoral medullary hyperplasia in cases of early involvement. Therefore, the interpretation of the results of pathologic examination of a pheochromocytoma should be immediately communicated to the surgeon.     

Hemorrhagic necrosis of pheochromocytoma associated with reversible renal artery stenosis

An unusual case of pheochromocytoma associated with renal artery stenosis is described. Despite the removal of bilateral adrenal pheochromocytoma, laboratory findings suggested the presence of residual pheochromocytoma and abdominal aortography revealed more pronounced stenosis of the right renal artery. Two months later, the undetected residual pheochromocytoma underwent hemorrhagic necrosis with acute cessation of catecholamine release. Thereafter, the patient's blood pressure decreased to a normal level with marked improvement in hypertensive symptoms. No remaining stenosis was demonstrated on follow up renal angiography. Our case suggests that constant local secretion of catecholamines may be responsible for the development of renal artery stenosis.     

Hemorrhagic necrosis of pheochromocytoma associated with reversible renal artery stenosis

An unusual case of pheochromocytoma associated with renal artery stenosis is described. Despite the removal of bilateral adrenal pheochromocytoma, laboratory findings suggested the presence of residual pheochromocytoma and abdominal aortography revealed more pronounced stenosis of the right renal artery. Two months later, the undetected residual pheochromocytoma underwent hemorrhagic necrosis with acute cessation of catecholamine release. Thereafter, the patient's blood pressure decreased to a normal level with marked improvement in hypertensive symptoms. No remaining stenosis was demonstrated on follow up renal angiography. Our case suggests that constant local secretion of catecholamines may be responsible for the development of renal artery stenosis.     

Simultaneous bilateral laparoscopic adrenalectomy for pheochromocytoma in multiple endocrine neoplasia (MEN) syndrome: Case report with review literature

INTRODUCTION

Laparoscopic adrenalectomy has gained favour as a preferred surgical approach in the multiple endocrine neoplasia (MEN) type 2 patients. Currently, there is limited literature on bilateral simultaneous laparoscopic adrenalectomy in MEN 2 syndrome. We reported two cases of bilateral pheochromocytoma associated with MEN 2 syndrome cured by simultaneous bilateral laparoscopic adrenalectomy.

PRESENTATION OF CASE

First patient presented with big lips since childhood and episodic abdominal pain. On investigations, he was diagnosed with features of MEN 2B syndrome. Second patient was hypertensive and presented with abdominal pain. On evaluation she had features of MEN 2A syndrome.

DISCUSSION

Minimally invasive approach was preferred in both cases. Bilateral simultaneous adrenalectomies were uneventfully done with acceptable operative time and blood loss with rapid perioperative recovery. These cases highlighted the feasibility of laparoscopic simultaneous bilateral adrenalectomy for pheochromocytomas in MEN 2 syndrome.

CONCLUSION

Laparoscopic simultaneous bilateral adrenalectomy is a safe feasible and preferable technique for pheochromocytomas associated with MEN 2 syndrome.     

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.     

Anesthetic Management of Clinically Silent Familial Pheochromocytoma with MEN 2A: A Report of Four Cases

Familial pheochromocytomas are commonly associated with multiple endocrine neoplasia type 2 (MEN 2) syndrome. Majority of the patients present with normal clinical and biochemical parameters in the preoperative period, the incidence of hypertension being only 50 %. Even though patients may be clinically asymptomatic, surveillance and proper preoperative evaluation is important, as surgery for associated tumors may precipitate a hypertensive crisis and result in severe complications. A family of 19 members, of which 12 were positive for MEN 2A syndrome, presented to our hospital. Seven of the 12 patients had pheochromocytoma and medullary thyroid carcinoma (MTC), while the other 5 had only raised plasma calcitonin levels. Two of the 7 patients presented with bilateral pheochromocytoma and underwent an open adrenalectomy. The other 5 patients had a left-sided adrenal tumor and underwent left laparoscopic adrenalectomy under combined general and epidural anesthesia. We present our experience with four of these five cases. We here state that how paucity of literature on perioperative preparation of clinically and biochemically silent pheochromocytomas led to serious intraoperative complications in one of four cases.     

2型多发内分泌腺瘤病的诊断及外科治疗

目的探讨2型多发内分泌腺瘤病的临床特点、治疗方法及疗效。方法回顾分析我院1980～2002年诊治的8例2型多发内分泌腺瘤病患者的临床特点、诊断及治疗方法与疗效。结果7例有阵发性高血压,5例最高血压超过200mmHg(1mmHg=0．133kPa),3例查体发现肿瘤。经B超、CT及24h尿儿茶酚胺检查确定诊断,2A型6例,2B型2例;表现为甲状腺髓样癌合并嗜铬细胞瘤伴或不伴甲状旁腺腺瘤或增生(6例),1例伴多发黏膜神经瘤,1例为嗜铬细胞瘤伴有马凡综合征;双侧肾上腺肿瘤7例;分期行肾上腺与甲状腺、甲状旁腺结节切除或次全切除术。术后平均随访9年,效果良好。结论2型多发内分泌腺瘤病的诊断主要依赖相应的内分泌检查及B超和CT检查,手术是主要的治疗手段,当嗜铬细胞瘤与其他肿瘤同时存在时,宜首先切除嗜铬细胞瘤。     

Pheochromocytoma manifested by traumatic adrenal hemorrhage

The presentation of pheochromocytoma is quite variable. We report a case of previously undiscovered pheochromocytoma which was manifested by traumatic retroperitoneal hemorrhage. A 79-year-old man fell from his bicycle and was admitted to the emergency room complaining of right flank pain. Enhanced computed tomography (CT) revealed hemorrhage around the right adrenal gland. Since the plasma catecholamine levels were elevated, we suspected the presence of pheochromocytoma. After absorption of the hematoma, the tumor appeared clearly. The diagnosis of pheochromocytoma was confirmed through urine catecholamine testing and 131I-MIBG scintigraphy. Six months after the injury, the tumor was surgically resected. Traumatic hemorrhage of pheochromocytoma is extremely rare; only 3 cases have been reported in the literature.     

An operated case of multiple endocrine neoplasia type IIb and review of the Japanese literature

Multiple endocrine neoplasia, type IIb (MEN IIb) is a rare syndrome characterized by the occurrence of medullary thyroid carcinoma (MTC), pheochromocytoma and mucosal neuroma. A 35-year-old male patient with MEM IIb having megacolon, marfanoid habitus and no family history of the disease underwent surgery. Because MTC was present in both lobes, total thyroidectomy and modified neck dissection were performed. Pheochromocytoma was found bilaterally and bilateral adrenalectomy with adrenal autotransplantation in the rectus abdominis muscle was carried out. Postoperative course was satisfactory except for transient hypocalcemia and mild ileus. After the slow corticosteroid weaning process, his adrenocortical function was at the lower level within a normal range. In August 1986 (24 postoperative months), he was maintained by the administration of 10mg of hydrocortisone every three days, and calcitonin and CEA levels in sera were normal. We collected 15 cases reported in Japanese literatures. MTC and mucosal neuroma were found in all cases, whereas pheochromocytoma was present in 9 cases. Bilateral and multicentric occurrences were usual, and total thyroidectomy and bilateral adrenalectomy were, warranted. We believe that autotransplantation following bilateral adrenalectomy is a worthy alternative.     

Laparoscopic right adrenalectomy for spontaneous hemorrhage in an adrenal incidentaloma,presenting as a case of acute surgical abdomen

Spontaneous adrenal hemorrhage is a rare cause of acute abdominal pain. Systemic disorders that deregulate normal hemostasis and the use of anticoagulants are both considered predisposing factors for this unusual condition. Questions still remain in the case of hemorrhage in previously diagnosed incidentalomas. Spontaneous hemorrhage in this setting increases the suspicion of occult malignancy or pheochromocytoma. On the other hand, the presence of a retroperitoneal hematoma can make laparoscopic excision of such lesions technically challenging in the acute setting. In the present study we report the case of spontaneous adrenal hemorrhage associated with oral anticoagulant use in a previously diagnosed incidentaloma. After initial stabilization, the patient was subjected to laparoscopic right adrenalectomy within 72 hours of the onset of symptoms. The patient was discharged on the second postoperative day. Biopsy revealed a benign adrenal adenoma. Prompt laparoscopic adrenalectomy is the treatment of choice in hemodynamically stable patients presenting with hemorrhage in a known adrenal tumor.     

Hemorrhagic necrosis of pheochromocytoma associated with phentolamine administration.

A case of ruptured pheochromocytoma is presented, the pathophysiology discussed, and the literature reviewed. Evidence is presented that the use of alpha-adrenergic blockade in general, and phentolamine in particular, may predispose to this complication. Twelve cases of massive hemorrhagic necrosis with or without rupture were found in the literature, including the present case. Six had no operation; one survived. Six had immediate operation; 4 survived. An additional case of hemorrhage into a small pheochromocytoma following phentolamine is presented. This tumor was neither ruptured nor massively necrotic, but the case supports the hypothesis that alpha-adrenergic blockade may cause hemorrhage within the pheochromocytoma.     

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.     

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.     

Traumatic hemorrhage of occult pheochromocytoma: a case report and review of the literature

Pheochromocytoma usually presents with gradual onset and mild to moderate symptoms, but may present acutely with severe symptoms. Hemorrhage into pheochromocytoma is a rare cause of acute presentation that is often devastating to patients. We describe the case of a 34-year-old woman with hemorrhage into a previously undiscovered pheochromocytoma following a fall on a patch of ice. This is the first reported case of hemorrhagic pheochromocytoma associated with traumatic injury. Despite removal of the tumor within 18 hours of presentation, the patient suffered severe complications of massive catecholamine excess, including shock, cardiomyopathy, and adult respiratory distress syndrome. Animal studies have shown that early treatment with alpha blockers can prevent some, if not all of these complications. Proper management of hemorrhagic pheochromocytoma should include a high index of suspicion with early diagnosis and treatment with alpha blockers and surgical resection of the tumor when the patient is stable enough to tolerate the procedure.     

Laparoscopic resection of periadrenal paraganglioma in a patient with multiple endocrine neoplasia type 2A

We report here a case of recurrent pheochromocytoma successfully managed by laparoscopic surgery in a patient with multiple endocrine neoplasia type 2A. A 25-year-old man presented with the contralateral adrenal mass after earlier right adrenalectomy. For the preoperative diagnosis of left adrenal pheochromocytoma, adrenal sparing surgery was considered. From the intraoperative laparoscopic view, we found that the tumor originated in periadrenal sympathetic ganglia, and laparoscopic excision of paraganglioma was successfully performed that preserved the integrity of normal adrenal gland. Extra-adrenal pheochromocytoma is rather rare in MEN 2A and it is important to identify precise intraoperative localization of pheochromocytoma with laparoscopic surgery.