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PubMed1996

Dihydroxyphenylglycol in pheochromocytoma: its diagnostic use for norepinephrine dominant tumor.

PURPOSE: To discern whether the 3,4-dihydroxyphenylglycol produced in adrenal medulla is associated with altered urinary excretion, we compared the tissue and urinary levels of this catecholamine metabolite, epinephrine and norepinephrine in 23 patients with pheochromocytoma and 26 subjects with renal cell carcinoma. MATERIALS AND METHODS: Tissue and urine concentrations of dihydroxyphenylglycol, epinephrine and norepinephrine were determined by catechol-O-methyl-transferase-based radioenzymatic method and high performance liquid chromatography with electrochemical detection. RESULTS: Contents of dihydroxyphenylglycol, epinephrine and norepinephrine in pheochromocytomas were higher than those in normal adrenal medullae. Removal of pheochromocytomas lowered urinary excretion of these catecholamines and dihydroxyphenylglycol. Norepinephrine content correlated closely with dihydroxyphenylglycol content in normal adrenal medullae and pheochromocytomas but not with epinephrine content in either tissue. Contents of norepinephrine and dihydroxyphenylglycol correlated significantly with urinary norepinephrine excretion and dihydroxyphenylglycol excretion in patients with pheochromocytoma but not in subjects with renal cell carcinoma. The norepinephrine-to-dihydroxyphenylglycol ratio in a 24-hour preoperative urine collection from patients with pheochromocytoma was higher than that from subjects with renal cell carcinoma, which was almost similar to that of epinephrine dominant pheochromocytomas. CONCLUSIONS: Our study suggests that dihydroxyphenylglycol in urine in subjects with renal cell carcinoma is predominantly of a neuronal origin and urinary dihydroxyphenylglycol in patients with pheochromocytoma is of tumor origin. A high level of norepinephrine-to-dihydroxyphenylglycol ratio in urine can be used to determine the diagnosis of the norepinephrine dominant type pheochromocytoma. Measurement of at least a few markers is preferable for precise biochemical diagnosis of pheochromocytoma.

Adrenal Gland Neoplasms↗

PubMed2002

Somatic VHL gene deletion and point mutation in MEN 2A-associated pheochromocytoma.

Multiple endocrine neoplasia type 2 (MEN 2) is an inherited cancer syndrome that includes pheochromocytoma. Germline mutations in RET are responsible for MEN 2 but the precise pathogenetic mechanisms of tumorigenesis are unknown. We have recently identified possible mechanisms of tumor formation in patients with MEN 2A-related pheochromocytoma. Two of nine tumors investigated, however, did not reveal either of these mechanisms. In the present study, we therefore searched for other possible mechanisms underlying the pathogenesis of MEN 2A-related pheochromocytoma. Hereditary pheochromocytoma also occurs in patients with von Hippel-Lindau (VHL) disease, a syndrome consisting of tumors caused by inactivation of the VHL tumor suppressor gene. A subset of sporadic pheochromocytomas have somatic mutations in RET or VHL, suggesting that both genes contribute to pheochromocytoma pathogenesis in a subset of tumors. It is unknown, however, whether VHL gene alterations would be associated with tumorigenesis in hereditary, MEN 2-related pheochromocytoma. We therefore investigated four pheochromocytomas from patients with MEN 2A and RET germline mutations for the presence of allelic deletion and/or somatic mutation of the VHL gene. LOH analysis using the polymorphic markers D3S1038 and D3S1110 that map to the VHL gene locus 3p25/26, revealed evidence for somatic VHL gene deletion in all four MEN 2A-related pheochromocytomas. Mutation analysis of the VHL gene showed frameshift mutations in two tumors and a splice acceptor mutation in one tumor. The remaining tumor did show LOH but not mutation of the VHL gene. These results suggest that somatic genetic alterations of the VHL gene may play a role in the tumorigenesis of some MEN 2A-related pheochromocytomas.

Adult↗

PubMed1999

Clinical experience over 48 years with pheochromocytoma.

OBJECTIVE: To analyze the presentation, localization, surgical management, pathology, and long-term outcome of a large series of patients with pheochromocytomas. SUMMARY BACKGROUND DATA: There are several areas of controversy pertaining to pheochromocytomas. Although many studies report a higher rate of malignancy for extraadrenal pheochromocytomas than for adrenal pheochromocytomas, the number of patients with the former tumor are small and statistical analysis is lacking. There has also been recent debate as to whether microscopic features of the tumor may be predictive of future behavior. METHODS: From 1950 to 1998, the authors observed 108 pheochromocytomas in 104 patients. The outcome of these patients has been followed prospectively. The medical records of these patients were reviewed for data on the presentation, localization, surgical management, pathology, and outcome. Patient survival was analyzed using Kaplan-Meier survival distributions. RESULTS: This study included 66 female patients and 38 male patients. The average age at surgery was 42.3 years. Sporadic cases accounted for 84% of the patients; the other 16% had multiple endocrine neoplasia type 2, von Recklinghausen's disease, von Hippel-Lindau disease, or Carney's syndrome. Of 64 adrenal tumors, 55 were initially considered benign, 6 had microscopic malignant features, and 3 had malignant disease. Mean patient follow-up was 12.6 years. To date, in five additional patients (none with microscopic disease) malignant disease developed (13% overall rate of malignancy). Recurrence occurred as late as 15 years after resection. Of 26 extraadrenal pheochromocytomas, 14 were initially considered benign, 8 had microscopic malignant features, and 4 had malignant disease. Thus, 46% of patients had either malignant disease or tumors with malignant features. Mean patient follow-up was 11.5 years. In one patient with benign disease and in one patient with malignant features, malignant disease developed (23% overall rate of malignancy). The difference in the rate of malignancy was not statistically significant between adrenal and extraadrenal pheochromocytomas. Patients with adrenal and extraadrenal pheochromocytomas also had similar rates of survival (p = NS). CONCLUSIONS: The data suggest that patients with extraadrenal pheochromocytomas have the same risk of malignancy and the same overall survival as patients with adrenal pheochromocytomas. Lifelong follow-up of these patients is mandatory.

Adolescent↗

PubMed2003

Diabetes as a marker of pheochromocytoma in hypertensive patients.

OBJECTIVE: To assess the prevalence of diabetes in patients with pheochromocytoma and the probability of pheochromocytoma occurring in hypertensive patients with or without diabetes. SETTING: A tertiary, referral hypertension department. PATIENTS AND METHODS: We compared age, body mass index and the frequency of diabetes in 191 patients with pheochromocytoma and a random sample of 880 patients with essential hypertension. Diabetes was defined as current antihyperglycemic treatment or two fasting blood glucose concentrations >or= 7 mmol/l. For patients with pheochromocytoma, we also recorded plasma catecholamine concentrations, the urinary excretion of metanephrines, and tumor characteristics. RESULTS: Diabetes was present in 68 (35.6%) patients with pheochromocytoma and 192 (21.8%) patients with essential hypertension (P < 0.001). Pheochromocytoma patients with or without diabetes did not differ in body mass index, plasma noradrenaline concentration, metanephrine excretion or tumor characteristics. Age, duration of hypertension and plasma adrenaline concentration were significantly and independently associated with diabetes in patients with pheochromocytoma. They were younger, more likely to be female and had a lower body mass index than those with essential hypertension (P < 0.01). After adjustment for these three variables, the odds ratio for pheochromocytoma in hypertensive patients with diabetes was 5.5 (95% confidence interval, 3.5-8.7). For patients younger than the age of 51 years with a body mass index < 25 kg/m2, the odds ratio was 18.9 (95% confidence interval, 5.9-58.8). CONCLUSION: Diabetes is present in one in three patients with pheochromocytoma. In young patients with hypertension and normal body weight, the presence of diabetes is a clinical clue to the diagnosis of pheochromocytoma.

Adult↗

PubMed1997

Immunohistochemical localization of endothelin-1, endothelin-3 and endothelin receptors in human pheochromocytoma and paraganglioma.

Endothelin (ET) and its receptor system have been shown to exert various biological effects on different types of cells in addition to their well-known vasoconstrictor activity. Recently ET-1, ET-3 and the ETB receptor have been shown to play an important role in the development of neural crest-derived cells and, in this context, pheochromocytomas have been reported to harbor ET-1. Endothelin-3 or ET receptor subtypes, however, have not been examined in pheochromocytoma and paraganglioma so far. In the present study the immunohistochemical localization of ET-1/big ET-1, ET-3/big ET-3 and the ETA and ETB receptors were investigated to clarify the biological characteristics of these two tumors using 32 pheochromocytomas and 11 extra-adrenal paragangliomas. Endothelin-1/big ET-1 was detected in 19 pheochromocytomas (59%) and eight paragangliomas (72%), while ET-3/big ET-3 was detected in 10 pheochromocytomas (31%) and three paragangliomas (27%). The ETA receptor was found in 21 pheochromocytomas (66%) and in eight paragangliomas (73%), while the ETB receptor was found in 25 pheochromocytomas (78%) and in eight paragangliomas (73%). Normal adrenomedullary cells lacked each antigen examined. Endothelin-immunoreactive tumor cells were distributed focally or in a manner scattered, while receptor-immunostained tumor cells were distributed with a focal pattern for the ETA receptor and with a focal or diffuse pattern for the ETB receptor. Endothelin and its receptor coexisted in the same tumor in 21 of 28 ET-positive pheochromocytomas and in eight of 10 ET-positive paragangliomas. In addition, seven pheochromocytomas and two paragangliomas revealed positivity of the receptor(s) irrespective of the absence of ET-immunoreactivity. In conclusion, ET and its receptor are frequently and concomitantly expressed in the pheochromocytoma and paraganglioma. From the highly frequent expression of this system or the receptor(s), ET-receptor-mediated signal transduction of these tumors concerning growth and/or cell survival is expected, although definite biological significance of this ligand-receptor system in these tumors awaits further investigation.

Adrenal Gland Neoplasms↗

PubMedDate not supplied

The association between severe nephropathy and pheochromocytoma in the male F344 rat -- the National Toxicology Program experience.

The possible correlation between the severity of chronic progressive glomerulonephropathy (CPN) and the incidence of adrenal pheochromocytoma was examined in selected studies of male Fischer 344 (F344) rats at the National Toxicology Program (NTP). The NTP historical control database was first examined in order to determine whether there was association between the severity of CPN and the occurrence of adrenal pheochromocytoma in unexposed animals. Following this analysis, the 125 most recent NTP studies conducted in F344 rats were examined in order to determine how frequently chemicals that cause increased severity of CPN showed an increased incidence of pheochromocytoma. Finally, we examined the association between the incidence of pheochromocytoma and the severity of CPN in those NTP studies with chemically related increased rates of pheochromocytoma. In control male F344 rats surviving beyond 21 mo, the incidence of adrenal pheochromocytoma was consistently higher in animals with more severe CPN. This association was significant (p < 0.05) both for 900 NTP inhalation study controls and 900 NTP feeding study controls. An association was not consistently observed when dosed groups were considered. Although 22% (28/125) of NTP studies reported a chemically related increased severity of CPN, only 3 of these reported a corresponding significant increase in the incidence of pheochromocytoma. Of 6 NTP studies that reported increased incidence of pheochromocytoma, animals with pheochromocytoma from 5 of those studies had some degree of increased severity of CPN. However, the estimated strength of the correlation with the severity of CPN varied from study to study and was often quite different from that indicated by an analysis of the more extensive NTP control databases. The possible correlation between the severity of CPN and the incidence of pheochromocytoma may influence interpretation of carcinogenic effects observed at this site.

Administration, Inhalation↗

PubMedDate not supplied

High plasma levels of human chromogranin A and adrenomedullin in patients with pheochromocytoma.

AIMS AND BACKGROUND: The aim of our study was to investigate the plasma chromogranin A (CgA) and adrenomedullin (AM) levels in patients with pheochromocytomas. METHODS AND STUDY DESIGN: We collected blood samples for measurement of plasma CgA and AM in 21 patients with pheochromocytomas, 43 healthy subjects and 26 patients with solid non-functioning adrenocortical adenomas. In 11 patients with pheochromocytomas plasma CgA and AM were measured again four weeks after tumor removal. CgA and AM were measured by means of a novel solid-phase two-site immunoradiometric assay based on monoclonal antibodies (CgA-RIA CT, CIS bio international) and by a specific radioimmunoassay (RIA, Phoenix Pharm. Inc.), respectively. RESULTS: The mean plasma CgA level (+/- SD) in patients with pheochromocytomas (204 +/- 147.9 ng/mL) was significantly higher (P < 0.001) than that in healthy subjects (41.6 +/- 10.7 ng/mL) and in patients with non-functioning adrenocortical adenomas (47.3 +/- 17.6 ng/mL). The mean plasma AM concentration (+/- SD) in patients with pheochromocytomas (27.5 +/- 10.4 pg/mL) was significantly higher (P < 0.001) than that in HS (13.8 +/- 4.5 pg/mL) and in patients with non-functioning adrenocortical adenomas (16.6 +/- 7.3 pg/mL). Plasma CgA levels correlated with plasma AM levels (r = 0.501; P < 0.02) and with plasma metanephrine levels (r = 0.738; P < 0.0001) in patients with pheochromocytomas. In 11 patients with pheochromocytomas plasma CgA and AM concentrations significantly decreased after tumor removal (P < 0.001 for both). Circulating CgA and AM had a sensitivity of 76.2% and 81%, a specificity of 97.7% and 90.7%, and an accuracy of 91% and 88%, respectively. CONCLUSION: This study demonstrates that circulating CgA and AM levels are increased in pheochromocytoma patients compared with healthy subjects and patients with non-functioning adrenocortical adenomas. Moreover, at the time of diagnosis plasma CgA levels correlated with plasma AM levels and with plasma metanephrine levels in all patients with pheochromocytomas. In conclusion, plasma CgA and AM concentrations may represent additional biochemical parameters for clinical monitoring of patients with pheochromocytomas.

Adrenal Cortex Neoplasms↗

PubMed2006

Risk-oriented approach to hereditary adrenal pheochromocytoma.

Hereditary adrenal pheochromocytoma is caused by germline mutations in RET, VHL, SDHB, SDHD, and NF1. As these genes differ in function, so may their pheochromocytoma phenotypes, suggesting gene-specific patterns of age-related progression to pheochromocytoma. This possibility was explored for gene carriers with a lifetime risk of pheochromocytoma in excess of 50%. Published age-standardized penetrance rates of VHL-, SDHB-, and SDHD-associated pheochromocytoma were gauged against age-standardized penetrance rates of MEN2-associated pheochromocytoma in 219 institutional carriers of RET mutations conferring highest (codon 918), high (codons 609, 611, 618, 620, 630, and 634) and least high risk (codons 768, 790, 791, 804, and 891). The highest-risk category included SDHB, SDHD, and the highest-risk RET genotype; the high-risk category VHL missense mutations and the high-risk RET genotypes; and the least-high risk category VHL truncating mutations and least-high risk RET genotypes. Detailed information on recurrence rates and intervals was available only for the RET carriers (19-31% and means of 4.3-5.5 years; all RET risk categories combined). Ipsilateral recurrences in adrenal remnants, and contralateral recurrences in virgin adrenals were comparable in incidence (27% and 39%, P=0.69; high-risk RET category) and time to recurrence (means of 4.3 vs. 5.4 year; P>0.99; high-risk RET category), discounting a major effect of tumor spillage at primary subtotal adrenalectomy on pheochromocytoma recurrence. The risk of malignancy usually is low, except for SDHB (38%). For most hereditary pheochromocytomas, endoscopic subtotal adrenalectomy is the procedure of choice. Grouping hereditary pheochromocytoma into preliminary risk categories may improve the management of gene carriers at risk of developing pheochromocytomas.

Adrenal Gland Neoplasms↗

PubMed2005

AKT is highly phosphorylated in pheochromocytomas but not in benign adrenocortical tumors.

CONTEXT: Activation of AKT plays a major role in a variety of human neoplasias. In mice, a heterozygous deletion of the Pten gene is associated with increased activation of AKT and with development of pheochromocytomas. OBJECTIVE: The objective of this study was the investigation of the role of AKT in the pathogenesis of pheochromocytomas and adrenocortical tumors. DESIGN, SETTING, AND PARTICIPANTS: Total AKT and phosphorylated AKT (pAKT) in 15 pheochromocytomas, nine aldosterone-producing adenomas, nine cortisol-producing adenomas, eight adrenocortical carcinomas (ACC), and 15 normal adrenals were investigated by Western blot analysis. Immunohistochemistry for total AKT and pAKT was performed in pheochromocytomas (n = 8), ACC (n = 4), and normal adrenal glands (n = 2). In addition, in pheochromocytomas PTEN protein expression and PTEN loss of heterozygosity were analyzed. MAIN OUTCOME MEASURES: Determination of pAKT/total AKT ratio in adrenal tissues was the main outcome. RESULTS: In comparison to normal adrenals, total AKT expression was elevated in both pheochromocytomas (193 +/- 22%) and ACC (176 +/- 36%). The pAKT/AKT ratio was significantly increased in pheochromocytomas (338 +/- 49% vs. 100 +/- 11%) but not in ACC, aldosterone-producing adenomas, and cortisol-producing adenomas. No loss of heterozygosity of PTEN and no decrease in PTEN protein was detected in pheochromocytomas. Immunohistochemistry showed strong and homogeneous AKT and pAKT staining in pheochromocytomas and focal staining in ACC. CONCLUSION: Our findings provide evidence for increased activation of AKT in pheochromocytomas but not in adrenocortical adenomas.

Adrenal Cortex Neoplasms↗

PubMed2004

Animal models of pheochromocytoma.

Pheochromocytomas are neuroendocrine tumors of adrenal chromaffin cells. They are rare in all species except rats but occur with increased frequency in several human familial tumor syndromes. Concurrence of pheochromocytoma with other tumors sometimes parallels these human syndromes in rats, bovines, horses and dogs but a shared genetic basis for human and spontaneously occurring animal pheochromocytomas has thus far not been established. Pheochromocytomas are inducible in rats by a variety of non-genotoxic substances that may act indirectly by stimulating chromaffin cell proliferation. They are not known to be similarly inducible in other species but arise with increased frequency in transgenic and knockout mice that to varying degrees recapitulate human tumor syndromes. Preliminary evidence suggests that homologous somatic genetic changes might contribute to pheochromocytoma development in humans and some mouse models. The nerve growth factor-responsive PC12 cell line, established from a rat pheochromocytoma, has for almost 30 years served as a research tool for many aspects of neurobiology involving normal and neoplastic conditions. Recently developed pheochromocytoma cell lines from neurofibromatosis knockout mice supplement the PC12 line and have generated additional applications. Advantages of the mouse lines include expression of substantial levels of the epinephrine-synthesizing enzyme, phenylethanolamine N-methyltransferase and expression of high levels of the receptor tyrosine kinase, Ret, which is characteristic of sporadic and familial human pheochromocytomas but not of PC12 cells. Disadvantages include an apparently less stable phenotype. It is difficult to establish pheochromocytoma cell lines from any species, although the tumor cells persist in culture for many months. Understanding of factors that permit pheochromocytoma cells to proliferate might itself provide important insights for tumor biology.

Adrenal Gland Neoplasms↗

PubMed2000

Expression of activin A and its receptors in human pheochromocytomas.

Activin A (a homodimer of two activin betaA subunits) has been shown to induce the neuronal differentiation of rat pheochromocytoma PC12 cells. We studied activin A and its receptor gene expression in human pheochromocytomas in vivo and in vitro to clarify the potential involvement of activin A in the pathophysiology of these tumors. We first screened 20 pheochromocytomas and nine normal adrenal tissues for activin betaA mRNA expression. Northern blots hybridized with specific oligonucleotide probes detected weak signals for activin betaA transcripts in pheochromocytomas. Both type I and type II activin receptor (ActR-I, ActR-IB and ActR-II) mRNA expression was also detectable in the pheochromocytoma tissues. In primary cultures of pheochromocytoma cells, expression of activin betaA mRNA was readily detectable by Northern blotting, and secretion of activin A into the conditioned medium was confirmed by an enzyme-linked immunosorbent assay. The expression of activin betaA mRNA and secretion of activin A were induced by (Bu)(2)cAMP after 1 and 3 days of treatment (all P<0.05). A protein kinase inhibitor, staurosporine, inhibited the basal and (Bu)(2)cAMP-induced accumulation of activin betaA mRNA (P<0.05). In addition, induction of chromaffin phenotype by dexamethasone also inhibited the basal and (Bu)(2)cAMP-induced expression of activin A at both mRNA and protein levels (all P<0.05). In contrast, the expression of ActR-I and ActR-IB mRNAs was not affected by these agents in cultured pheochromocytoma cells. In summary, activin betaA subunit and activin receptors are expressed in human pheochromocytomas. Production of activin A in cultured pheochromocytoma cells is induced through the protein kinase A pathway, but reduced during chromaffin differentiation. Therefore, activin A may function as a local neurotrophic factor via an auto/paracrine manner in human pheochromocytomas.

Activin Receptors↗

PubMedDate not supplied

[Bilateral pheochromocytoma associated with duodeno-jejunal GIST in patient with von Recklinghausen disease: report of a clinical case].

The authors present the case of a 60-year-old male patient suffering from von Recklinghausen's disease (neurofibromatosis type I, NF1) with bilateral pheochromocytoma and occasional intraoperative reports of duodenojejunal GIST (GastroIntestinal Stromal Tumour). Through a review of the literature the authors analyze the frequency and the features of bilateral pheochromocytoma and its rare histological variant, the so-called composite pheochromocytoma, characterized by the combination of pheochromocytoma and ganglioneuroma or ganglioneuro-blastoma. Bilaterality of pheochromocytoma is more frequent in patients with familiarity for pheochromocytoma without NF1. Composite pheochromocytoma accounts for about 3% of total pheochromocytomas. In addition, the authors summarize the present knowledge about gastrointestinal stromal tumours and investigate the possible association between them and NF1 or pheochromocytoma, concluding that any such association is purely casual, while confirming the well known, genetically determined association between NF1 and pheochromocytoma.

Adrenal Gland Neoplasms↗

PubMed2003

Clinical features and anesthetic management of multiple endocrine neoplasia associated with pheochromocytoma.

OBJECTIVE: To investigate clinical features and anesthetic management of multiple endocrine neoplasia (MEN) associated with pheochromocytoma. METHODS: Medical records of patients who were diagnosed as multiple endocrine neoplasia associated with pheochromocytoma in our hospital from April 1977 to April 2001 were reviewed retrospectively. The demographic data, clinical presentations, family history, biochemical examinations, type of MEN, sequence of different surgical procedures, anesthetic methods and hemodynamics during surgery were analyzed. RESULTS: Thirteen cases of MEN associated with pheochromocytoma were investigated, accounting for 6% (13/213) of the pheochromocytoma patients admitted into our hospital. Nine of the 13 patients presented as type IIa MEN (Sipple syndrome), one as type IIb MEN, and three as mixed MEN. Four patients with type IIa MEN had a family history of similar disease. Five patients with other coexisting endocrine disorders first underwent excision of the pheochromocytomas, although only two had hypertensive symptoms at the time of admittance. Seven patients without histories of hypertension received surgical treatment for pheochromocytoma secondly. The excision of pheochromocytoma was performed under general anesthesia in 8 patients and epidural block in 4 patients. Marked hemodynamic fluctuation was recorded in 8 patients. No perioperative death was recorded. CONCLUSION: Pheochromocytoma may be linked to other endocrine disorders during MEN, either as the main clinical presentation or most frequently as an occult tumor. Recognition of this feature of pheochromocytoma is of importance to the improvement of diagnosis and treatment both for pheochromocytoma and MEN.

Adrenal Gland Neoplasms↗

PubMed2003

Incidental adrenal pheochromocytoma. Report on 5 operated patients and update of the literature.

INTRODUCTION: Adrenal masses discovered by imaging techniques for reasons unrelated to adrenal diseases are called incidentalomas with pheochromocytomas accounting for about 20%. The aim of this study was to report on our experience of 5 patients operated for incidentally discovered adrenal pheochromocytoma and update the literature concerning this subject. PATIENTS AND METHODS: From September 1976 to December 2000 we operated on 28 for adrenal incidentaloma, and pheochromocytoma was detected in 5 patients (19%). Average age was 61.4 years (range 54-65). All patients underwent adrenal hormonal as well as imaging investigation. Preoperative care for pheochromocytoma was performed in 3 patients. All patients were followed up. RESULTS: Average tumor diameter, as detected by CT, was 5.5 cm (range 3-10). Urinary 24/h excretion of VMA resulted elevated in 2 cases (40%). Two patients during adrenalectomy developed paroxysmal hypertension which was readily controlled by intravenous administration of nitroprussiate and trandate. Postoperative outcome was uneventful. Average tumor size, as detected by pathology, resulted 8.9 cm (range 3-12). Mean follow-up was 60.4 months (range 32-122) and overall survival rate was 60%. Three patients are alive and disease-free and 2 died after a follow-up of 32 and 36 months for reasons unrelated to the primary disease. CONCLUSIONS: Every incidentally discovered adrenal mass should be investigated for pheochromocytoma since accounting for about 20%. Undetected pheochromocytomas undergoing surgery without preoperative care for avoiding cardiovascular disturbances confer a high risk for morbidity and mortality, especially in pregnancy and childhood. Nonfunctional and small tumors sized 3 cm or lesser may be closely followed up by imaging and hormonal investigation. Surgery is indicated when tumors are functional or larger than 3 cm. Actually, laparoscopy is the new gold-standard in treating adrenal pheochromocytomas. Adrenal sparing surgery as well as autotransplantation of adrenal tissue are both effective and safe in treating patients with bilateral pheochromocytomas, thus improving quality of life since avoiding lifelong steroid dependency. When managed appropriately, the outlook for pheochromocytoma is excellent with a free survival rate of 92% and 80% at 5 and 10 years respectively. Unfortunately, malignant pheochromocytomas are still difficult to treat.

Adrenal Gland Neoplasms↗

PubMed1985

Iodine-131 metaiodobenzylguanidine for the locating of suspected pheochromocytoma: experience in 400 cases.

The efficacy of the newly developed pheochromocytoma-seeking radiopharmaceutical, [131I]MIBG, was examined in the first 400 patients (441 studies) investigated for suspected pheochromocytoma at our institution. The results of [131I]MIBG scintigraphy were classified as true positive, false positive, true negative, and false negative. Using this classification the sensitivity was found to be 78.4% in primary, sporadic pheochromocytoma, 92.4% in malignant pheochromocytoma, and 94.3% in familial pheochromocytoma giving an overall sensitivity of 87.4%. The specificity was 98.9% in primary, sporadic pheochromocytoma, 100% in malignant pheochromocytoma, and 100% in familial pheochromocytoma. The overall specificity was 98.9%. Iodine-131 MIBG scintigraphy was thus found to be a safe, noninvasive, and efficacious technique for the location of pheochromocytomas, especially for those arising from nonadrenal sites, recurring postoperatively, and exhibiting malignant metastatic disease. We find that, where available, [131I]MIBG scintigraphy is the study of choice to initiate the location of suspected pheochromocytoma.

3-Iodobenzylguanidine↗

PubMed1984

Oncologic aspects of pheochromocytoma: the importance of follow-up.

During a 33-year period at Vanderbilt University Medical Center and its long-standing affiliated hospitals, Veterans Administration Medical Center and Metropolitan Nashville General Hospital, 69 patients with pheochromocytoma were seen. Despite the fact that hypertension was present in all patients at initial admission, in 11 patients seen during the 1950s the clinical diagnosis of pheochromocytoma was not made and the existence of this tumor was discovered at autopsy examination. In 58 patients the clinical diagnosis of pheochromocytoma was made and surgical excision of pheochromocytoma (or biopsy only in two cases) was carried out. One patient died during the initial postoperative period (1.7%). All of the operative survivors were followed to date or the death, with the exception of three who were lost to follow-up at 18 months and 7 and 11 years, respectively. The incidence of malignant disease in this carefully followed group of patients with pheochromocytoma was impressive. Nine of 69 patients (13%) proved to have malignant pheochromoblastoma, all but one of whom have died. Additional malignant tumors have occurred in two of these nine patients (22%) with proved malignant pheochromoblastoma and in 14 of 60 patients (23%) with benign pheochromocytoma. The overall incidence of additional malignant tumors in the collective group of patients with the histopathologically confirmed diagnosis of pheochromocytoma was thus 23% (16/69 patients). It is also interesting that a variety of benign neoplasms occurred in the 60 patients with benign pheochromocytomas (9/60; 15%). If the nine patients with malignant pheochromoblastoma are added, the overall incidence of malignant disease in these 69 patients with pheochromocytoma is 25/69 or 36%. Follow-up of patients with pheochromocytoma is mandatory on a lifetime basis and preferably by the operating surgeon.

Adolescent↗

PubMed1993

Three-decade investigation of familial pheochromocytoma. An allele of von Hippel-Lindau disease?

BACKGROUND: Usually sporadic, pheochromocytoma can, on occasion, complicate genetic disorders, such as neurofibromatosis 1, von Hippel-Lindau disease, and multiple endocrine neoplasia 2; some families seem to have just pheochromocytoma, where it may have occurred by chance. The natural history of a large kindred believed to have an excess of pheochromocytoma 34 years ago was followed with the hypothesis that the predisposition was, in fact, present and that family education and surveillance would decrease mortality. METHODS: Prospective observation and diagnostic surveillance for pheochromocytoma were conducted on the inception cohort, defined as three branches of the kindred in 1960. Of 619 descendants of three (of 11) siblings of German origin, 333 were evaluated in person at least once in the three decades of surveillance. No pheochromocytomas were known to have occurred in the eight other branches. A total of 522 persons from the 11 branches were evaluated. RESULTS: Five of the eight initial patients with pheochromocytoma died of cardiovascular complications attributable to the tumor. In follow-up, eight additional relatives were newly diagnosed with pheochromocytomas (at an average age of 19 years), and others had additional or recurrent pheochromocytomas, meningioma, para-adrenal paraganglioma, and a functioning glomus vagale; none died. CONCLUSIONS: A continuing excess of pheochromocytoma seems present in the family. Whether the incompletely penetrant gene in this family is allelic to the von Hippel-Lindau gene on chromosome 3 or is a distinct locus remains to be resolved with molecular studies. Meanwhile, education and surveillance seem to decrease mortality from pheochromocytoma in this family.

Adolescent↗

PubMed1993

[Anesthetic management of intraoperatively diagnosed pheochromocytoma--a case report].

Pheochromocytoma is a catecholamine secreting tumor originating from the adrenal medulla (up to 90%), or from the chromaffin tissue along the paravertebral sympathetic chain. The hallmark of pheochromocytoma is paroxysmal hypertension associated with diaphoresis, headache, tremulousness, and palpitations. The triad of diaphoresis, tachycardia, and headache in hypertensive patients is highly suggestive of pheochromocytoma. Other symptoms like flushing, nausea, vomiting, personality changes, and visual disturbances may however cast doubt on the diagnosis of pheochromocytoma. Death resulting from pheochromocytoma is usually due to congestive heart failure, myocardial infarction, or intracerebral hemorrhage. Although less than 0.1 percent of patients with hypertension have a pheochromocytoma, nearly 50 percent of the mortality with unsuspected pheochromocytoma occurred during anesthesia and surgery or parturition. Patients of unsuspected pheochromocytoma have higher risk for surgery, because some mandatory pre-op medical treatments might have been ignored. It is also a challenge to anesthesiologists to handle unsuspected hypertensive crisis during anesthesia and surgery. We presented such a case of unexpected Pheochromocytoma which was mis-diagnosed by the surgeon and was treated as an ordinary adrenal gland tumor and was scheduled for surgical operation. When the patient was undergoing excision of the tumor, manipulations of the tumor initiated an tremendous elevation of the blood pressure. Upon reviewing her history of normotension with visual disturbance, nausea and restlessness, she was immediate treated as with a pheochromocytoma. Appropriate managements were applied to control her abnormally high fluctuating blood pressure with success and with no complications or adverse effect.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Gland Neoplasms↗