Ectopic Pheochromocytoma Research Articles

A case of ectopic pheochromocytoma detected by ultrasonographic screening

A case of ectopic pheochromocytoma detected by ultrasonographic screening

Retroperitoneal neoplasms within the perirenal space in infants and children: Differentiation of renal and non-renal origin in enhanced CT images

Purpose To retrospectively demonstrate the specific CT findings of retroperitoneal neoplasms to diagnosis and differential diagnosis renal and non-renal tumors within the perirenal space in infants and children. Materials and methods We retrospectively reviewed the clinical data and CT images of 42 consecutive patients with surgically and pathologically proven retroperitoneal neoplasms within the perirenal space. The patients were divided into renal tumors group ( n = 16) and non-renal tumors group ( n = 26). The former included nephroblastoma ( n = 15) and renal lymphoma ( n = 1), while the latter included neuroblastoma ( n = 12), retroperitoneal teratoma ( n = 6), adrenal ganglioneuroma ( n = 4), retroperitoneal lymphoma ( n = 2), ectopic pheochromocytoma ( n = 1) and adrenal cortical carcinoma ( n = 1). The clinical information of these patients and the major CT imaging findings which were related to lesion localization in the two groups were compared and statistically analyzed using Pearson Chi-Square Test and Risk Estimate. Results The mean diameter of tumors was 9.82 ± 6.13 cm ( n = 42 range: 2.3–32 cm). The demographic data and chief clinical symptoms between the renal tumor group and the non-renal tumor group showed no statistically significant differences ( P > 0.05). 30.8% (8/26) of non-renal tumor patients presented elevated urinary vanillylmandelic acid (VMA) level, while no patient showed elevated VMA in renal tumor group ( P < 0.05). Some CT imaging signs of the renal tumors including “crescent sign” (odds ratio, OR = 52), “beak sign” (OR = 84), “embedded organ sign” (OR = 84), and “prominent feeding artery sign” (OR = 36) showed significantly higher incidence when compared to the non-renal tumors ( P < 0.001). The sign of “renal displacement and renal axis rotation” (OR = 0.059) was seen in 23 of 26 (88.5%) non-renal tumors, but in only 5 of 16 (31.3%) renal tumors ( P < 0.001). The sign of “extra-renal central plane of tumor” (OR = 0.038) was displayed in 24 of 26 (92.3%) non-renal tumors, but in only 5 of 16 (31.3%) renal tumors ( P < 0.001). The CT findings such as “pseudocapsule” (OR = 38.5), “necrosis and cystic change” (OR = 11.2), “vascularity” (OR = 16.867), “distant metastasis” (OR = 5.96), and “inferior vena cava tumor thrombus” which were thought to be characteristic of renal tumors were observed with significant higher incidence in renal tumors group than in the non-renal tumors group ( P < 0.05); while CT signs of “irregular mass” (OR = 0.045) and “intratumoral calcifications” (OR = 0.065) were observed with lower incidence in renal tumors group than in the non-renal tumors group ( P < 0.05). Conclusion The “crescent sign”, “beak sign”, “embedded kidney sign” and “renal arteries feeding” are the most specific CT signs suggestive of renal tumors and distinguish them from non-renal origin tumors within the perirenal space. Other CT signs, such as “pseudocapsule”, “hypervascular tumors” and “Inferior vena cava tumor thrombus”, when present, tumors of renal origin are strongly suggested. On the other hand, CT signs of “irregular mass”, “intratumoral calcifications”, and associated elevated urinary vanillylmandelic acid strongly suggest the non-renal tumors.

Pheochromocytoma of the urinary bladder: a case report

Urinary bladder pheochromocytoma is rare. From a case report of unsuspected pheochromocytoma and literature review, the authors develop a diagnostic and therapeutic algorithm for the management of this ectopic pheochromocytoma localization.

Extensive Ganglioneuromatosis of Gallbladder

A case of extensive ganglioneuromatosis (GN) of gallbladder is presented. A 38-year-old man presented with a hepatic hilar mass (Ø ~ 48 mm) and gall stones. He had undergone total thyroidectomy for medullary thyroid carcinoma 8 years earlier. The hepatic hilus tumor, which had been clinically suspected to be a lymph node metastasis from the medullary thyroid carcinoma, was found to be pheochromocytoma. The gallbladder, resected with a clinical diagnosis of cholelithiasis, showed extensive transmural GN despite a grossly normal appearance. Taking into account the past history, the patient was diagnosed as having multiple endocrine neoplasia 2b (MEN2b)-associated GN of gallbladder and ectopic pheochromocytoma. As GN of gallbladder in MEN2b has been rarely reported, the histological findings are described in detail and a brief review of literature is carried out.

Ectopic Pheochromocytoma: Does the Rule of Tens Apply?

The rule of "tens" is often stated to reflect the distribution and histology of pheochromocytomas, with 10% being bilateral, 10%; ectopic in origin, and 10%; malignant. The objective of this study was to review the ectopic pheochromocytomas in a tertiary endocrine center and to establish whether the rule of tens holds true. Retrospective data were collected on all adrenalectomies and ectopic pheochromocytoma resections performed between 1993 and 2005 at our institution. In total, 77 patients had pheochromocytomas: 75%; (58/77) adrenal and 25%; (19/77) ectopic. Of the adrenal pheochromocytomas, 10%; (6/58) were bilateral. The anatomic locations of the ectopic pheochromocytomas were as follows: 26%; (5/19) adjacent to the adrenals, 53%; (10/19) in the organ of Zuckerkandl, 11%; (2/19) in the bladder, 5%; (1/19) in the mediastinum, and 5%;(1/19) in the neck. Our series demonstrates an incidence of 10%; for bilateral pheochromocytoma, which is similar to that in the published reports. However, 25%; of the pheochromocytomas were ectopic. Zuckerkandl pheochromocytomas were the most common among the ectopic lesions. Rarely, these tumors present outside the abdominal cavity.

MicroCT for high‐resolution imaging of ectopic pheochromocytoma tumors in the liver of nude mice

Successful outcomes for patients with cancer often depend on the early detection of tumor and the prompt initiation of active therapy. Despite major advances in the treatment of many cancers, early-stage lesions often go undetected due to the suboptimal resolution of current anatomical and functional imaging modalities. This limitation also applies to preclinical animal tumor models that are crucial for the evaluation and development of new therapeutic approaches to cancer. We report a new mouse model of metastatic pheochromocytoma, generated using tail vein injection of the mouse pheochromocytoma cell (MPC) line that reproducibly generated multiple liver tumors in the animals. Furthermore, we show that in vivo microCT imaging enhanced using a hepatobiliary-specific contrast agent, glyceryl-2-oleyl-1,3-di-7-(3-amino-2,4,6-triiodophenyl)-heptanoate (DHOG), detected tumors as small as 0.35 mm as early as 4 weeks after the injection of the tumor cells. This model may be useful for in vivo studies of tumor biology and for development of new strategies to treat metastatic pheochromocytoma.

Pheochromocytoma in von Hippel–Lindau disease and neurofibromatosis type 1

Clinical and genetic understanding of chromaffin tumors has been greatly enhanced in the last few years. Although some pheochromocytoma genes may still be unknown, the role of RET, VHL, SDHB, SDHD and NF1 genes is unequivocal and phenotypes are also being better characterized. The loss of function of VHL and NF1 genes can lead to a variety of tumors including phechromocytoma and their mechanism of action is under intensive investigation. Many different mutations are responsible for VHL gene inactivation but only missense mutations have been described so far in families with pheochromocytoma. Because of its large size extensive mutation analysis of the NF1 gene has seldom been performed, and mutations have only been identified in about 15% of patients. Several point mutations have been found in exon 31. Differences in pheochromocytoma phenotype in VHL or NF1 are not very pronounced, but it may be of some interest to consider the two groups separately. In VHL, pheochromocytoma has an earlier onset than in sporadic forms, it is often multiple, and malignancy is less frequent. The mean age of diagnosis is 28 years, the youngest patient being 5 years old. In NF1 patients pheochromocytoma phenotype is similar to sporadic forms. The mean age of pheochromocytoma onset is 42 years; 84% of patients have solitary adrenal tumors, 9.6% have bilateral adrenal disease and 6.1% have ectopic pheochromocytomas; malignant pheochromocytomas were identified in 11.5% of the cases. The group of pheochromocytoma susceptibility genes includes, along with the tumor suppressor genes VHL and NF1, the proto-oncogene RET and the genes encoding succinate dehydrogenase subunit D and succinate dehydrogenase subunit B. Whether there is a common pathway among these different genes is still a matter of debate.

Simultaneous appearance of leg giant squamous cell carcinoma and ectopic pheochromocytoma

Simultaneous appearance of leg giant squamous cell carcinoma and ectopic pheochromocytoma

Les localisations extrasurrénaliennes du phéochromocytome : à propos de quatre cas

Extra-adrenal pheochromocytoma is rare. The diagnosis must be evoked in signs of pheochromocytoma with absence of tumor of the adrenal. We report four cases of ectopic pheochromocytoma while specifying their clinical particularities and diagnosis procedure.

Phéochromocytome extrasurrénalien découvert en peropératoire

In this study, an uncommon case has been reported of an ectopic pheochromocytoma without the presence of any clinical symptoms. The radiological investigations showed a right retroperitoneal tumor without any kidney involvement. The diagnosis was established by biopsy and subsequent histological findings. In the course of surgery as the large tumor mass was being removed, tachycardia was observed which caused the resection to be performed as rapidly as possible. Once the tumor had been removed, bradycardia occurred, followed by cardiac arrest: although the latter was stabilized after cardiac massage, the patient died one hour after the operative field had been closed. In addition to this case report, the diagnosis, therapeutic strategy and prognosis regarding an ectopically located pheochromocytoma have been discussed.

Le phéochromocytome extrasurrénalien. À propos de trois observations et revue de la littérature

The authors report three new cases of ectopic pheochromocytoma: latero-aortic, bladder and near the kidney. From the review of the literature, they propose a diagnostic and therapeutic strategy for the management of ectopic localisation of pheochromocytoma.

A CASE OF ECTOPIC PHEOCHROMOCYTOMA

A 65-year-old woman was admitted to the hospital for exploration for a tumor in the left upper abdomen which was palpated when she was receiving treatment for hypertension on an ambulant basis. On admission an elastic soft tumor was palpated in the left upper abdomen. Endocrine examination revealed elevations in blood noradrenaline and urinary vanillylmanderic acid (VMA). Abdominal ultrasonography visualized a tumor of 8cm in diameter with heterogenous internal component at the anterior to the left kidney and the left-side to the aorta. Abdominal CT also revealed a tumor with heterogenous internal component consisting of multiple cysts in the same region. A possible diagnosis of ectopic pheochromocytoma was made, and the tumor was excised. The excised tumor was 9.0×7.0×5.4cm in size and 300g in weight. The tumor had an irregular surface. The section revealed sporadical nodules in different sizes and cysts with the maximum diameter of about 2cm. Ectopic pheochromocytoma associating with cysts rarely occurs in this country and only 24 cases have been reported so far. This paper describes such a rare case with a review of the literature.

A CASE OF ASYMPTOMATIC ECTOPIC PHEOCHROMOCYTOMA

A 44-year-old woman was admitted to the hospital because of an abdominal tumor. abdominal ultrasonography, CT and MRI revealed a well defined mass about 7cm in diameter around the aorta. The tumor was diagnosed as neurogenic tumor or hemangioma, and an operation was performed. Histological diagnosis was ectopic pheochromocytoma. The patient had no clinical symptoms suggestive of pheochromocytoma. This was an extremely rare case of asymptomatic ectopic pheochromocytoma.

A CASE OF ECTOPIC PHEOCHROMOCYTOMA

A 72-year-old man was admitted because of right hypochondralgia. A tumor in the right retroperitoneal space was revealed by abdominal ultrasonography, CT and MRI. Blood pressure was normal (140/86 mmHg) before operation, but during operation, systolic blood pressure suddenly rose to 240 mmHg. At this time, it was suspected that the tumor was an ectopic pheochromocytoma. Histological findings showed pheochromocytoma and bilateral normal adrenal glands were visualized on postoperative131I-adosterol scintigraphy.

A CASE OF ASYMPTOMATIC ECTOPIC PHEOCHROMOCYTOMA

A case of asymptomatic ectopic pheochromocytoma, a rare retoroperitoneal tumor, is reported. A 21-year-old female arrived at a local hospital with the chief complaints of vomiting and epigastric discomfort. Abdominal ultrasonography and CT revealed a mass abour 5cm in diameter around the aorta. At the exploratory laparotomy an ectopic pheochromocytoma was suspected because of hyperternsion and ventricular premature beat which occurred by palpation of the mass. The operation was discontinued and the patient was referred to our hospital. On admission, blood pressure was normal and a remarkable increase of norepinephrine in serum and urine was observed. This case was diagnosed asymptomatic ectopic pheochromocytoma as a hot nodule in scintigram using 131I-metaiodobenzyl guanidine (131I-MIBG). After the extirpation of the mass, catecholamine levels in serum and urine decreased to normal range and symptoms completely disappeared.

Localization of ectopic pheochromocytomas by magnetic resonance imaging

Magnetic resonance imaging (MRI) was utilized to localize ectopic pheochromocytomas in two patients. In both cases, MRI proved superior to computer tomography. Differentiation from surrounding tissue was excellent. MRI permits spatial localization and by assessment of signal contrast intensity provides a suggestion as to the characteristics of the tumor without the use of invasive procedures or intravenous injections. MRI should be considered as an alternative to computed tomography when evaluating patients with a suspected pheochromocytoma.

Plasma gut hormone levels in 37 patients with pheochromocytomas

AbstractPheochromocytomas are usually recognized by the effects of overproduction of catecholamines, but there are clinical features that cannot be ascribed to catecholamine excess that may be due to vasoactive peptides. We, therefore, measured blood levels of vasoactive intestinal peptides (VIP), substance P, somatostatin (SS), and motilin in 50 instances in 37 patients with pheochromocytomas‐21 malignant, 10 benign intra‐adrenal, and 6 ectopic (5 paracardial and 1 perirenal). Hormone levels were considered raised if the level was more than 3 S.D. above the mean value found in 52 healthy subjects. Of the 37 patients, 20 (54%) had an abnormality in 1 or more gut hormone levels. The most common abnormality was a raised SS in 9/37 (24%). In addition to these, however, 3 (8%) others had raised VIP, 5 (13.5%) raised motilin, and 3 (8%) raised substance P. Patients with benign adrenal adenomas had raised levels of SS and substance P. Ectopic pheochromocytomas produced only SS in addition to catecholamines, but malignant pheochromocytomas could secrete all 4 peptides, and more than 1 in the same patient. We conclude that pheochromocytomas may secrete multiple vasoactive peptides, and they are more likely to do so if malignant. Somatostatin is the most commonly secreted peptide and is found with benign adrenal and ectopic (paracardiac) tumors. If the level of more than 1 peptide is elevated, the likelihood of malignancy is significantly increased.

A Case Report of an Ectopic Pheochromocytoma located on the Abdominal Aorta and on the Vena Cava

A Case Report of an Ectopic Pheochromocytoma located on the Abdominal Aorta and on the Vena Cava

A Case of Multiple Ectopic Pheochromocytomas Occurring in the Urinary Bladder and the Para-Aortic Region

A Case of Multiple Ectopic Pheochromocytomas Occurring in the Urinary Bladder and the Para-Aortic Region

The management of ectopic pheochromocytoma producing ureteral obstruction : M. A. Immergut, R. Boldus, C. P. Kollin and P. Rohlf. J. Urol. 104:337–341 (August), 1970

The management of ectopic pheochromocytoma producing ureteral obstruction : M. A. Immergut, R. Boldus, C. P. Kollin and P. Rohlf. J. Urol. 104:337–341 (August), 1970