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At least 19 recordsLinked to original sources
PubMed1999

Clinical and genetic characterization of pheochromocytoma in von Hippel-Lindau families: comparison with sporadic pheochromocytoma gives insight into natural history of pheochromocytoma.

PURPOSE: Families with von Hippel-Lindau disease have variable risk of pheochromocytoma. Patients with von Hippel-Lindau disease and pheochromocytoma identified by screening can have no characteristic signs or symptoms. Families with von Hippel-Lindau disease were screened and followed to describe the natural history of von Hippel-Lindau pheochromocytoma, and to correlate these findings with von Hippel-Lindau germline mutation. MATERIALS AND METHODS: Between 1988 and 1997, 246 individuals with von Hippel-Lindau disease were identified (von Hippel-Lindau group). Between August 1990 and June 1997, 26 consecutive patients with sporadic pheochromocytoma were evaluated (sporadic group). RESULTS: A total of 64 patients with von Hippel-Lindau disease had manifestations of pheochromocytoma, including 33 newly diagnosed during screening at the National Institutes of Health and 31 previously treated (93 adrenal and 13 extra-adrenal pheochromocytomas). Germline von Hippel-Lindau gene missense mutation was associated with extra-adrenal pheochromocytoma, younger age at presentation and the only patient with metastases. Of the 33 newly diagnosed patients with von Hippel-Lindau disease 4 had pheochromocytoma 2 times (37 pheochromocytomas) during followup. Of these pheochromocytomas 35% (13 of 37) were associated with no symptoms, normal blood pressure and normal catecholamine testing. Comparison of urinary catecholamines in the von Hippel-Lindau and sporadic groups demonstrated increased epinephrine, metanephrines and vanillylmandelic acid in the sporadic group. Analysis of urinary catecholamine excretion in the von Hippel-Lindau and sporadic groups together demonstrated a correlation between tumor size, and urinary metanephrines, vanillylmandelic acid, norepinephrine, epinephrine and dopamine. In 12 patients without signs or symptoms of pheochromocytoma 17 newly diagnosed pheochromocytomas were followed for a median of 34.5 months without morbidity. Median tumor doubling time was 17 months. CONCLUSIONS: Von Hippel-Lindau gene missense mutation correlated with the risk of pheochromocytoma in patients with von Hippel-Lindau disease. These findings support a von Hippel-Lindau disease clinical classification, wherein some families are at high risk for manifestations of pheochromocytoma. Von Hippel-Lindau disease pheochromocytomas identified by screening were smaller and less functional than sporadic pheochromocytomas.

Adrenal Gland Neoplasms↗
PubMed1986

[Catecholamines in the cardiovascular expression of pheochromocytoma. II--Study of free urinary catecholamines in 14 pheochromocytomas. Classification of pheochromocytomas according to type of secretion].

Studies were conducted in 14 patients with pheochromocytoma over a 3-year period. Circumstances of detection of these tumors varied greatly and were sometimes misleading, hypertension being an inconstant finding in the clinical history and was not always the predominant feature. Biologic exploration involved assay of excretion of free urinary noradrenaline (NA), adrenaline (AD) and dopamine (DA) using a HPLC technique as well as assay of total methoxy derivatives and urinary vanilmandelic acid. Validity of each assay in the diagnosis of pheochromocytoma could be evaluated and only the total free methoxy derivatives gave false negative results. Hormonal secretion of pheochromocytoma is often mixed, but sometimes predominant or exclusive for a single catecholamine. Relative increases of the different catecholamines, evaluated from the ratios DA/NA and DA/NA + AD, are an important factor since a relation exists between blood pressure induced symptomatology and equilibrium between hypotensive hormone (DA) and pressor amines (NA + AD); 3 types of pheochromocytoma can be described: NA-induced with paroxysmal or permanent hypertension but without typical metabolic and cardiac disorders, and with a very reduced DA/NA + AD ratio during hypertensive crises; AD-induced without permanent hypertension but with a mainly orthostatic hypotension and episodes of cardiovascular collapse following hypertensive attacks and with an AD/NA ratio greater than 1; finally the DA-induced lesion in which hypertension is never associated and manifestations are misleading and atypical with an elevated DA/NA + AD ratio.

Adrenal Gland Neoplasms↗
PubMed1990

Immunoreactive corticotropin-releasing hormone, growth hormone-releasing hormone, somatostatin, and peptide histidine methionine are present in adrenal pheochromocytomas, but not in extra-adrenal pheochromocytoma.

CRH, GH-releasing hormone (GHRH), somatostatin (SRIH), and peptide histidine methionine (PHM) were measured by RIA in extracts of normal adrenal glands and in extracts from adrenal and extraadrenal pheochromocytomas. In normal adrenal glands, immunoreactive (IR) CRH, IR-SRIH, and IR-PHM were detectable, while IR-GHRH was undetectable. In all 11 cases of adrenal pheochromocytomas, the tumors contained 2 or more of these four IR-peptides. In particular, IR-CRH was found in 73% (n = 8) of adrenal pheochromocytomas, IR-GHRH in 91% (n = 10), IR-SRIH in 91% (n = 10), and IR-PHM in 82% (n = 9) of adrenal pheochromocytomas. There was no significant correlation among the concentration of these peptides in each tumor, i.e. the concentrations of the IR-peptides were independent of each other. In contrast to the adrenal pheochromocytomas, none of these 4 IR-peptides was detectable in 5 extraadrenal pheochromocytomas. Gel filtration of pooled extracts from adrenal pheochromocytomas showed that the major component of the IR-CRH, IR-GHRH, IR-SRIH, and IR-PHM eluted in the position of their synthetic counterparts. Our results suggest that 1) the normal adrenal gland contains IR-CRH, IR-SRIH, and IR-PHM, but not IR-GHRH; 2) all of the adrenal pheochromocytomas we examined contained a number of hypothalamic releasing or inhibiting hormones; 3) their tissue concentrations were independent of each other; and 4) all of the extraadrenal pheochromocytomas we examined contained no such IR-peptides. The presence of hypothalamic hormones in adrenal pheochromocytomas and their absence in extraadrenal pheochromocytomas may be due to the differences in the chromaffin cells of their origin. Our data may be helpful in the differential diagnosis between adrenal and extraadrenal pheochromocytomas.

Adrenal Gland Neoplasms↗
PubMed1997

Antiproliferative effect of suramin on primary cultures of human pheochromocytomas and rat PC12 pheochromocytoma cells.

BACKGROUND: Suramin inhibits growth of neural crest-derived cells and is used to treat adrenocortical cancer and neuroblastoma in clinical trials. The antiproliferative effect of suramin was evaluated in primary cultures of human pheochromocytoma and the PC12 rat pheochromocytoma cell line in vitro and in vivo. METHODS: Human pheochromocytoma and PC12 rat pheochromocytoma cells were grown in medium supplemented with suramin at concentrations of 1-1,000 micrograms/ml (1.43-1.43 mM) for up to five generations. Suramin did not induce neuronal differentiation, but inhibited growth of cultured human pheochromocytoma cells with IC50 (inhibitory concentration at which a 50% reduction of proliferation is observed) of 50-250 micrograms/ml. Also, suramin inhibited proliferation of PC12 cells with IC50 of 228 micrograms/ml after 5 days and 161 micrograms/ml at 10 days of treatment. Colony formation assays demonstrated these effects to be cytotoxic rather than cytostatic. Thus when reproductive integrity of PC12 cells was taken into account, IC50 was calculated with 118 micrograms/ml and 129 micrograms/ml, respectively. In vivo experiments were performed with subcutaneously xenotransplanted PC12 cells (BALB/c NCR-NU mice). Suramin did not alter tumorigenicity and did not inhibit local tumor growth. RESULTS: These data determine for the first time an antiproliferative effect of suramin in pheochromocytoma cells. Suramin is cytotoxic to pheochromocytoma cells in vitro at levels that are clinically achievable. CONCLUSIONS: Suramin may have potential as an antiproliferative drug in nonresectable pheochromocytoma.

Adrenal Gland Neoplasms↗
PubMed2002

Pheochromocytoma in von hippel-lindau disease: distinct histopathologic phenotype compared to pheochromocytoma in multiple endocrine neoplasia type 2.

Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin tissue. In a small subset of patients, pheochromocytomas occur as a manifestation of von Hippel- Lindau (VHL) disease. The histology of VHL-associated pheochromocytomas has not been reported in detail. In this article, we describe histopathologic features of 14 pheochromocytomas in eight patients with VHL disease and demonstrate that VHL-associated pheochromocytomas have a distinct histologic phenotype as compared with pheochromocytomas in patients with multiple endocrine neoplasia type 2 (MEN 2). VHL tumors are characterized by a thick vascular tumor capsule; myxoid and hyalinized stroma; round, small to medium tumor cells intermixed with small vessels; predominantly amphophilic and clear cytoplasm; absence of cytoplasmic hyaline globules; and lack of nuclear atypia or mitoses. In contrast to MEN 2, there is no extratumoral adrenomedullary hyperplasia in the VHL adrenal gland. Our findings of a distinct histologic phenotype of VHL pheochromocytoma may further help in subdividing patients who clinically present with multiple, bilateral pheochromocytomas.

Adrenal Gland Neoplasms↗
PubMed1999

Pheochromocytoma associated with neuroendocrine carcinoma. A new type of composite pheochromocytoma.

The coexistence of pheochromocytoma and other tumor types in a single adrenal gland has been rarely documented. This type of pheochromocytoma is designated "composite" or "mixed," depending on whether the pheochromocytoma and the nonpheochromocytoma components show the same embryologic origin. The nonpheochromocytoma components reported in the composite pheochromocytoma include ganglioneuroma, ganglioneuroblastoma, neuroblastoma, and malignant schwannoma. The components found in the mixed pheochromocytoma include adrenal cortical neoplasms and spindle cell sarcoma. We report a unique case of composite pheochromocytoma in which the nonpheochromocytoma element is a neuroendocrine carcinoma. The histologic and the immunohistochemical profiles of the 2 distinct components of this tumor were typical for those of pheochromocytoma and neuroendocrine carcinoma. This dual differentiation was also supported by ultrastructural findings. This case not only broadens the morphologic spectrum of composite pheochromocytoma but also provides some additional insight into the histogenesis of this rare but fascinating type of tumor.

Adrenal Gland Neoplasms↗
PubMed1999

Kappa1-opioid binding sites are the dominant opioid binding sites in surgical specimens of human pheochromocytomas and in a human pheochromocytoma (KAT45) cell line.

The adrenal medulla produces opioids which exert paracrine effects on adrenal cortical and chromaffin cells and on adrenal splanchnic nerves, via specific binding sites. The opioid binding sites in the adrenals are detectable mainly in the medullary part of it and differ in type between species. Thus, the bovine adrenal medulla contains mostly kappa-opioid binding sites and fewer delta- and mu-opioid binding sites while primate adrenals contain mainly delta sites and few kappa-opioid binding sites. Most chromaffin cell tumors, the pheochromocytomas, produce opioids which suppress catecholamine production by the tumor. The aim of the present work was to identify the types of opioid binding sites in human pheochromocytomas. For this purpose, we characterized the opioid binding sites on crude membrane fractions prepared from 14 surgically excised pheohromocytomas and on whole KAT45 cells, a recently characterized human pheochromocytoma cell line. Our data showed that human pheohromocytomas are heterogeneous, as expected, with regard to the production of catecholamines and the distribution and profile of their opioid binding sites. Indeed, only one out of the 14 pheochromocytomas expressed exclusively delta and mu opioid sites, while in the remaining 13 tumors kappa-type binding sites were dominant. The KAT45 cell line possessed a significant number of kappa1 binding sites, fewer kappa2-opioid binding sites and kappa3-opioid binding sites, and minimal binding capacity for delta- and mu-opioid receptor agonists sites. More specifically, the kappa1 sites/cell were approximately 18,000, the kappa2 4500/cell and the kappa3 sites 2000/cell. Our findings for the surgical specimens and the cell line combined with previously published pharmacological data obtained from KAT45 cells suggest that kappa sites appear to be the most prevalent opioid binding sites in pheochromocytomas. Finally, in normal bovine adrenals the profile of opioid binding sites differs in adrenaline and noradrenaline producing chromaffin cells. To test the hypothesis that the type of catecholamine produced by a pheochromocytoma depends on its cell of origin, we compared our binding data with the catecholamine content of each pheochromocytoma examined. We found no correlation between the type of the predominant catecholamine produced and the opioid binding profile of each tumor suggesting that this hypothesis may not be valid.

Analgesics, Opioid↗
PubMed2000

Laparoscopic adrenalectomy for pheochromocytoma: comparison with open adrenalectomy and comparison of laparoscopic surgery for pheochromocytoma versus other adrenal tumors.

OBJECTIVE: To compare the efficacy of laparoscopic adrenalectomy for pheochromocytoma with that of conventional open adrenalectomy for pheochromocytoma and laparoscopic surgery for other adrenal tumors. PATIENTS AND METHODS: Fifty-four patients with adrenal tumors, including 10 cases of pheochromocytoma, 18 cases of Cushing's syndrome, 20 cases of primary aldosteronism, and 6 cases of nonfunctioning tumors, were evaluated. A historical group of 7 consecutive patients who underwent conventional open adrenalectomy for pheochromocytoma was also studied. RESULTS: Laparoscopic adrenalectomy for pheochromocytoma was successful in 9 of the 10 patients. There was no difference in tumor size, operation time, estimated blood loss, or occurrence of hypertensive episodes during surgery between patients treated with laparoscopic procedures and those treated with open surgery. However, the number of days to first postoperative oral feeding and first ambulation, length of hospitalization, and number of patients requiring parenteral analgesics were significantly smaller after laparoscopic surgery than after open surgery. There was no significant difference in operation time, estimated blood loss, incidence of intraoperative complications, or postoperative recovery between patients who underwent laparoscopic adrenalectomy for pheochromocytoma and those who underwent laparoscopic surgery for other adrenal lesions. CONCLUSIONS: Laparoscopic adrenalectomy does not increase the specific risks associated with surgery for pheochromocytoma. It is a minimally invasive alternative to conventional open adrenalectomy.

Adolescent↗
PubMed1983

[Case of intrathoracic pheochromocytoma occurring 9 years after resection of intraabdominal paraaortic pheochromocytoma: effect of metoclopramide and sulpiride on catecholamine secretion in vitro].

A 29-year-old female who had undergone resection of an abdominal paraaortic pheochromocytoma weighing 33 g at the age of 20 had had severe headaches, hypertension and hyperhidrosis 3 years prior to the surgery. Postoperatively, her symptoms completely disappeared and urinary catecholamines were normalized. She was well and had married and had had 2 children. She was admitted to our hospital on August 22, 1982, for further evaluation of hypertension (154/100), which had been diagnosed 2 months previously. Endocrinological studies confirming the presence of a pheochromocytoma were as follows: 1) Plasma noradrenaline level was significantly elevated to 1750 pg/ml. 2) Urinary catecholamine and their metabolites (Metanephrines and VMA) were markedly elevated. Her blood pressure was borderline hypertension and its diurnal rhythm was lost. Her blood pressure decreased to normal values after the oral administration of labetalol (100 mg). Plasma noradrenaline level was still high at 180 minutes after the oral administration of clonidine (150 micrograms). Hypertensive response to insulin-induced hypoglycemia (regular insulin 0.1 u/kg i.v.) was observed, but blood pressure returned to normal after the infusion of glucose alone. Hypertensive response to both metoclopramide (5 mg i.v.) and sulpiride (50 mg per os) was observed accompanying the significant elevation of plasma noradrenaline. Computed tomography and ultrasonography revealed a tumor localized between the aorta and the vena cava inferior. Selective venous sampling also revealed an intrathoracic pheochromocytoma. On October 8, 1982, a 28 g mass was removed from the mediastinum just above the diaphragma. Histologically, it was typical of a pheochromocytoma. Electron microscopy showed large polygonal cells with numerous large secretory granules characteristic of noradrenaline-granules. Postoperative blood pressure was normal, but repeated measurements of plasma and urinary catecholamines were still slightly high. We, therefore, followed her case carefully at our out-patient clinic. In order to clarify the mechanism of catecholamine release by metoclopramide and sulpiride, tissue cultures of removed pheochromocytoma with and without these drugs were carried out. The in vitro studies revealed that metoclopramide released noradrenaline eight-fold and sulpiride 13-fold as compared with noradrenaline in a control medium. We concluded that both drugs stimulated catecholamine secretion directly from the tumor and thus, careless administration of these drugs should be avoided when pheochromocytoma was suspected, large or small. Finally, the rate of tumor growth seemed to be very slow because it took 9 years to ach

Abdominal Neoplasms↗
PubMed1998

A unique allogenic model of metastatic pheochromocytoma: PC12 rat pheochromocytoma xenografts to nude mice and establishment of metastases-derived PC12 variants.

Local invasion and metastatic spread to distant sites are major causes of death in patients with malignant pheochromocytoma. Since appropriate in vivo models do not exist, little is known about the underlying mechanisms of tumor growth and invasion. We, therefore, developed an animal model of malignant pheochromocytoma and established organotropic metastatic variants of PC12 rat pheochromocytoma cells. PC12 cells were established as xenografts to BALB/c NCR-NU mice. Subsequent to development of tumors or metastases, primary cultures from local tumors, metastases to lymph nodes, lungs and liver were established. These were subcultured in vitro and reinjected for up to five successive in vivo/in vitro cycles. Xenografted PC12 cells grew tumors with a doubling time of 6.78 +/- 0.58 days during log phase of tumor growth, killing hosts within 5-12 weeks depending on the experimental conditions. Tumors reproducibly metastasized to lymph nodes and the lung. Spontaneous metastases to the liver were not observed, but were achieved by intrasplenic injection of parent PC12 cells. In vitro, the metastatic cell lines displayed striking differences in morphology, overall growth patterns and nutritional requirements as well as binding to purified extracellular matrix proteins compared to the parent cell line. In vivo, the metastatic variants showed marked enhancement of metastatic ability. This is the first report of PC12 rat pheochromocytoma cells to exhibit the malignant phenotype in vivo. We also established variant PC12 cell lines that preferentially metastasized to specific sites and that had acquired different in vitro behavior and ability to metastasize. This unique model system should be useful for further studies relating to the invasion and metastases of pheochromocytoma and may prove valuable for investigations of novel antineoplastic therapies in vitro and in vivo.

Animals↗
PubMed1999

[A comparative study of 9 cases of adrenal pheochromocytoma and 11 cases of extra-adrenal pheochromocytoma].

Twenty patients with the diagnosis of pheochromocytoma were studied from January 1990 to January 1998. Nineteen patients had a pathologic investigation performed. The mean age of patients was 49 +/- 16 years (range: 24-71 years), 8 males and 12 females. Nine cases corresponded to adrenal and eleven to extra-adrenal (paragangliomas) pheochromocytomas. The anatomic location was similar in both adrenal glands and for paragangliomas the cervical location predominated. The most consistent clinical finding in our patients was maintained arterial hypertension, followed by headache, palpitations and flushing. Biochemically, an increase in urine catecholamine levels or their metabolites was found in 82.2%. The clonidine test was very useful, particularly for cases where biochemical results were discrepant. CT, NMR and gammagraphy with 123I-MIBG have a high sensitivity for locating pheochromocytomas. NMR and gammagraphy with 111In-pentetreotide located cases in which CT or 123I-MIBG were negative. Preoperative treatment with adrenergic alpha-blockers allowed to surgery with no arterial tension complications. Only one patient with multiple abdominal paragangliomas relapsed. No differences regarding clinical manifestations, biochemical parameters or imaging studies were found between pheochromocytomas and paragangliomas, except their location.

3-Iodobenzylguanidine↗
PubMed1986

Growth of human pheochromocytomas in the anterior eye chamber of the rat. A histochemical study on amine and peptide content of pheochromocytoma tumour cells.

Tissue pieces from seven benign human pheochromocytomas have been successfully transplanted to the anterior eye chamber of cyclosporin-treated rats. In vivo observations showed that 74-99% of the tumour transplants were vascularized within one to two days after transplantation. No increase in the size of the transplants was noted during the observation period (1-4 weeks). Tumour transplants grown in non-immunosuppressed rats were initially vascularized but rejection started to occur one week after transplantation. Histochemical analysis of tumour transplants grown in immunosuppressed rats demonstrated numerous tumour cells with strong catecholamine fluorescence, some of which formed long cell processes on the host iris. Immunocytochemical analysis of tumour transplants demonstrated positively labelled tumour cells after incubation with antisera against neuropeptide Y, enkephalin, vasoactive intestinal polypeptide, somatostatin, substance P, dopamine-beta-hydroxylase, tyrosine hydroxylase and serotonin. A similar histochemical and immunocytochemical pattern was observed in primary tumours but tumour cells sending out cell processes were observed less frequently. Human pheochromocytomas may thus be successfully grown in oculo in cyclosporin-treated rats. This may prove to be a suitable model for the study of storage and release of catecholamines and neuropeptides from pheochromocytoma tumour cells.

Adrenal Gland Neoplasms↗
PubMed1980

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.

Adolescent↗
PubMed1999

[Right adrenal recurrence of pheochromocytoma discovered 24 years after ablation of two ectopic pheochromocytomas].

The classical localisation of chromaffin cell tumours is intra-adrenal. Ectopic or multiple tumours are not rare and are commonly observed in children. The authors report a case of ectopic pheochromocytoma with a double localisation in a 14 year old child (renal pedicle and right retropleural space), in which surgical ablation resulted in an immediate and sustained correction of the hypertension. Hypertension recurred 24 years later and a classical right adrenal pheochromocytoma was demonstrated by methyl-iodo-benzylguanidine (M.I.B.G.) scintigraphy and abdominal CT scan. Right adrenalectomy resulted in normalisation of the hypertension once again without antihypertensive therapy with a follow-up of three years. Regular follow-up is necessary after ablation of a pheochromocytoma, especially in children, even in the absence of a phacomatosis or multiple endocrine neoplastic syndromes.

Adolescent↗
PubMed1986

[Malignant pheochromocytoma associated with Recklinghausen's disease. Apropos of a case. Value of new methods in the diagnosis of pheochromocytoma].

After having reported the case of a pheochromocytoma associated to Recklinghausen's disease, the authors define the best criteria of detection and localization of the pheochromocytoma and study its association to phacomatoses. Headaches, bouts of tachycardia and excessive inappropriate diuresis are the most evocative clinical signs of a pheochromocytoma. The different hormones and their urinary metabolites must be titrated separately and repeatedly. Two other examinations, scanner and scintigraphy with MIBG, visualize quite reliably the tumor foci. Calcium inhibitors are quite effective in sudden blood pressure rises. The association described here, may be explained by the fact that the two pathologies belong to the group of neurocristopathies.

Adrenal Gland Neoplasms↗
PubMed1986

Dopamine-secreting pheochromocytoma: an unrecognized entity? Classification of pheochromocytomas according to their type of secretion.

A pheochromocytoma that exclusively secretes dopamine (DA) rather than predominantly DA among a blend of catecholamines is as yet unreported. Of the 50 patients with pheochromocytoma who have undergone surgery, 32 underwent treatment within the last 5 years (when DA assay has been available). One half of these patients (15/32) exhibited DA secretion either in mixed catecholamines (12 patients) or exclusively (three patients). All three patients with exclusive DA-secreting tumors were normotensive. Without hypertension, the clinical investigation was a diagnostic challenge (unexplained cough or flank mass with inflammatory features). All three tumors were malignant and two were ectopic. Five of the 12 patients with mixed catecholamine-secreting tumors whose secretions included DA were hypertensive. Five other patients had flank mass and one had an unexplained cough. Tumors were rather large, and three of the tumors with mixed secretion were ectopic. Of the 12 patients, seven had tumors that were judged to be malignant. Three patients exhibited a dramatic decrease in blood pressure under alpha-blockade, which was not used in subsequent cases. Predominant or exclusive secretion of DA would explain the lack of hypertension due to its antiadrenergic action that inhibits the vasoconstrictive effects of other amines. Hypertension in patients with pheochromocytoma might depend on the ratio of DA/noradrenaline + adrenaline.

Adrenal Gland Neoplasms↗
PubMed1995

A case of massive adrenal malignant pheochromocytoma: management of a large pheochromocytoma.

A 69-year-old man was referred to our hospital for a left abdominal tumor measuring about 30 cm in diameter. Laboratory examination revealed an elevation of norepinephrine in plasma and of its metabolites in urine. CT scan disclosed a huge cystic tumor in the retroperitoneal space and an enlarged aortocaval lymph node, suggesting a diagnosis of malignant pheochromocytoma. The hemodynamic studies showed low blood volume and high vascular resistance, and therefore, he was treated with vasodilators and volume expansion. His hemodynamic status normalized and a complete excision was performed. Pathological examination revealed that the patient had a pheochromocytoma with metastasis to a lymph node. The total weight of the tumor was 5,930 g. Since pheochromocytomas can become a large with a risk of malignancy, they should be surgically excised as completely as possible with further treatment after making a definite diagnosis.

Adrenal Gland Neoplasms↗