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  1. Fulltext An audit of management of patients with borderline increased plasma-free metanephrines
    Anas SS, Vasikaran SD
    Ann. Clin. Biochem., 2010 Nov;47(Pt 6):554-8.
    PMID: 20926470 DOI: 10.1258/acb.2010.010131
    BACKGROUND: Measurement of plasma-free metanephrine plus normetanephrine (PFM) is the best screening test for phaeochromocytoma. While clearly raised levels are diagnostic, borderline increases may be associated with factors such as stress and medications, and should prompt a repeat study after interfering factors are withdrawn.
    METHODS: PFM results reported by a teaching hospital laboratory over a 12-month period were extracted from the laboratory information system. All borderline raised results were examined for a subsequent repeat test (as recommended by attached interpretative comment) and those not repeated were followed up by examination of case-notes or questionnaires to doctors.
    RESULTS: Of 111 patients with borderline increased PFM which did not normalize on repeat measurement, 33 were from the hospital and 78 from outside locations. Hospital notes for 27 out of 33 hospital-patients (82%) could be reviewed, and 49 completed questionnaires (63% of 78 sent out) were received from outside locations. Of these 76 patients thus followed up, the test was not repeated in 55 (72%) cases with borderline increased PFM. Of 10 patients with an adrenal mass and borderline PFM, only three had PFM repeated. Of another nine patients with undetermined final diagnosis and borderline PFM, only three had the test repeated.
    CONCLUSION: Seventy-two per cent of borderline increased PFM results were not followed up with appropriate repeat testing, potentially leading to missed detection of phaeochromocytoma. A stronger interpretative comment may encourage appropriate repeat testing in more cases with borderline increased PFM and suspected phaeochromocytoma.
    Matched MeSH terms: Pheochromocytoma/blood; Pheochromocytoma/diagnosis
  2. Fulltext Phaeochromocytoma of the urinary bladder
    Naqiyah I, Rohaizak M, Meah FA, Nazri MJ, Sundram M, Amram AR
    Singapore Med J, 2005 Jul;46(7):344-6.
    PMID: 15968447
    The occurrence of urinary bladder paragangliomas is rare. A 12-year-old Chinese girl who presented with history of blurring of vision was found to have grade IV hypertensive retinopathy. Investigations revealed a phaeochromocytoma on the posterior wall of the urinary bladder. A partial cystectomy with right ureter reimplantation was undertaken and her hypertension was promptly controlled. The diagnosis and management of this rare tumour is discussed.
    Matched MeSH terms: Pheochromocytoma/diagnosis; Pheochromocytoma/surgery*
  3. Fulltext Ectopic adrenocorticotrophic hormone syndrome (EAS) with phaeochromocytoma: a challenging endocrine case with a happy ending
    Wan Muhamad Hatta SF, Lekkakou L, Viswananth A, Buch H
    BMJ Case Rep, 2019 Aug 20;12(8).
    PMID: 31434676 DOI: 10.1136/bcr-2019-230636
    Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is rarely caused by a phaeochromocytoma. We report a case of a 51-year-old woman with an 8-year history of severe constipation who underwent extensive investigations including gastroscopy, colonoscopy, ultrasonography, colonic transit studies and isotope defeacography, which did not reveal any pathology other than slow colonic transit time. The unifying diagnosis of ectopic ACTH and phaeochromocytoma was made after the case was initially investigated for an adrenal incidentaloma. Multiple challenges had to be overcome prior to surgery for the functioning adrenal adenoma including management of refractory hypokalaemia, poor nutritional status, persistent hyperglycaemia, labile blood pressure and florid hypercortisolaemia driving the metabolic derangements. She underwent an uneventful left-sided adrenalectomy and required no medication thereafter with normal blood pressure, blood glucose and serum potassium and resolution of constipation and abdominal symptoms. In conclusion, patients with EAS related to phaeochromocytoma are rare and present with distinctive diagnostic and management challenges but if diagnosed successfully and managed intensively, they are curable.
    Matched MeSH terms: Pheochromocytoma/complications; Pheochromocytoma/diagnosis*; Pheochromocytoma/surgery
  4. Fulltext A case of Familial Phaechromocytoma- was it?
    Siti Yazmin Zahari Sham, Nor Aini Umar, Khalidah Mazlan
    Malaysian Journal of Medicine and Health Sciences, 2015;11(2):85-88.
    MyJurnal
    With advancement in genetic studies, familial phaeochromocytoma (PCC) and paraganglioma (PGL)
    are increasingly being recognized. Characteristically, correlations exist between genotypes and clinical
    and biochemical phenotypes. We report a phaeochromocytoma in a young patient with intriguing family
    histories, raising the possibility of his being a familial case.
    Matched MeSH terms: Pheochromocytoma
  5. Screening for phaeochromocytoma in patients with acute cerebrovascular disease: Is it necessary?
    Mohamed Zainuddin N, Sthaneshwar P, Ratnasingam J
    Malays J Pathol, 2020 Aug;42(2):203-207.
    PMID: 32860372
    INTRODUCTION: Phaeochromocytoma may present with uncontrolled hypertension leading to haemorrhagic stroke (HS), ischaemic stroke (IS) and transient ischaemic attack (TIA). False elevation in the levels of CATS/ METS has been reported in acute cerebrovascular disease. Our aim was to analyse the frequency and pattern of elevations of CATS/METS in patients with acute cerebrovascular disease and to determine associated factors.

    MATERIALS AND METHODS: This is a retrospective study of 112 samples of CATS/ METS received by the laboratory over a two-year period, from patients with acute cerebrovascular disease. CATS/METS were measured using LC/MS/MS method. Clinical details and CATS/METS level were obtained from the database. Mann-Whitney U test and Kruskal Wallis test were used for statistical analysis. These statistical analyses were performed using SPSS v.20.0 (IBM Corp., Armonk, NY, USA).

    RESULTS: Of the 112 patients, 39% had HS, 54% had IS and 7% had TIA. A total of 29% of patients had elevated CATS/ METS. Elevated levels of CATS/METS were noted in 41% and 25% of HS and IS patients, respectively (p=0.53). Median norepinephrine, epinephrine and metanephrine levels in HS were significantly higher than IS (p< 0.05). Systolic blood pressure was higher in those who had elevated CATS/ METS (p=0.04). Only for two patients with elevated CATS/METS repeat testing was performed. Age, diastolic blood pressure and the time of sample collection in relation to the presentation, for CATS/METS were not significantly different between groups that had elevated levels of CATS/ METS versus those who did not.

    CONCLUSION: We noted that CATS/METS were elevated in one-third of patients, especially in patients with high systolic blood pressure. Increase in CATS/METS should be appropriately followed up with repeat testing. Since false elevation in CATS/METS has been reported in cerebrovascular disease, screening for phaeochromocytoma is best deferred for a month.

    Matched MeSH terms: Pheochromocytoma/diagnosis*
  6. The challenge of improving the diagnostic yield from metanephrine testing in suspected phaeochromocytoma and paraganglioma
    Samsudin I, Page MM, Hoad K, Chubb P, Gillett M, Glendenning P, et al.
    Ann. Clin. Biochem., 2018 Nov;55(6):679-684.
    PMID: 29660998 DOI: 10.1177/0004563218774590
    Background Plasma-free metanephrines (PFM) or urinary fractionated metanephrines (UFM) are the preferred biochemical tests for the diagnosis of phaeochromocytoma and paraganglioma (PPGL). Borderline increased results should be followed up to either exclude or confirm diagnosis. Methods We extracted all PFM and UFM results reported by our laboratory over a six-month period from the laboratory information system. We categorized patients with borderline increased results according to whether follow-up testing had been performed as suggested in the initial laboratory report. Questionnaires were then sent to all requesting doctors and medical notes reviewed where available. Results Two hundred and four patients with borderline increased PFM or UFM were identified. Sixty-five (38.5%) of 169 patients with borderline increased PFM had a repeat test out of which 36 were normal and 29 did not normalize. Of 35 patients with borderline increased UFM, 17 (48.6%) had subsequent PFM measurement, out of which 15 were normal. Questionnaires were returned to 106 (52%) patients. Of these, the most frequent indication for testing was hypertension ( n = 50); 15 patients had an incidental adrenal mass and two of these patients were diagnosed with a phaeochromocytoma. Conclusion Only 38% of patients with borderline increased PFM had a repeat PFM measurement. This was not significantly higher when compared with the 28% in a previous audit that we reported in 2010 ( P = 0.10). Forty-nine per cent of patients with a borderline increased UFM had a repeat UFM or PFM measurement. There remains a substantial possibility of missed detection of PPGL.
    Matched MeSH terms: Pheochromocytoma/diagnosis*
  7. Epinephrine-secreting pheochromocytoma in a normotensive woman with adrenal incidentaloma
    Sukor N, Saidin R, Kamaruddin NA
    South. Med. J., 2007 Jan;100(1):73-4.
    PMID: 17269532
    Pheochromocytomas are rare neuroendocrine tumors that produce, metabolize, and usually secrete catecholamines. Although hypertension is a common presenting feature of pheochromocytoma, the tumors occur (or are present) in only 0.1% of patients with hypertension. The variability of symptoms and rarity of occurrence render these tumors difficult to diagnose; many are discovered incidentally during radiological examination or at autopsy. A patient is presented with a pheochromocytoma that was discovered incidentally when she presented with abdominal pain and a normal blood pressure.
    Matched MeSH terms: Pheochromocytoma/diagnosis; Pheochromocytoma/metabolism*; Pheochromocytoma/surgery
  8. The surgical treatment of adrenal diseases
    Meah FA, Abdullah T, Jasmi AY, Hisham AN, Tan TT, Khalid BA, et al.
    Ann Acad Med Singap, 1996 Mar;25(2):251-4.
    PMID: 8799016
    Between January 1978 to December 1993, 130 cases of adrenal diseases were diagnosed and surgically treated at the National University of Malaysia. They were 58 cases (44.6%) of Conn's syndrome, 40 cases (30.7%) of Cushing's syndrome, 20 cases (15.3%) of phaeochromocytoma and 12 cases (9.2%) of adrenocortical carcinoma (ACC). The commonest cause of Conn's syndrome was an adenoma (96.5%) which affected the left gland four times more than the right gland. Cushing's syndrome was caused by adrenocortical adenoma (32.5%), diffuse bilateral adrenal hyperplasia (40.0%), pigmented macronodular hyperplasia (20.0%) and adrenal carcinoma (7.5%). Twenty-five percent of the phaeochromocytomas were extraadrenal in origin arising mainly from the abdominal sympathetic chain. More than 50% of ACCs were non-functioning tumours. Fifty percent of the patients with ACC had inoperable tumours. The prognosis was poor even with adjuvant chemoradiotherapy. The main surgical approach was the anterior transabdominal route. There was no operative mortality or morbidity in all operated cases.
    Matched MeSH terms: Pheochromocytoma/diagnosis; Pheochromocytoma/epidemiology; Pheochromocytoma/surgery*
  9. Uncontrolled hypertension, palpitation and sweating in young female - A rare cause
    Sandeep HV, Sarat KS, Ng LT
    Med J Malaysia, 2016 02;71(1):39-40.
    PMID: 27130746
    Extra-adrenal /retroperitoneal paraganglioma is a rare cause of hypertension in young with increased incidence of metastasis as compared to adrenal pheochromocytoma. We present a case of a young female with history of headache, nausea/vomiting, palpitations, uncontrolled hypertension, heat intolerance and diaphoresis. The 24-hour urine catecholamine levels were elevated. Clinical diagnosis of pheochromocytoma was made and further evaluation with Computed Tomography (CT) scan of the adrenals revealed extradrenal para-aortic retroperitoneal mass in keeping with paraganglioma. Gallium-68 DOTATE positron emission tomography-CT scan (PET-CT) confirmed the diagnosis without evidence of metastatic foci.
    Matched MeSH terms: Pheochromocytoma/complications; Pheochromocytoma/diagnosis*
  10. Fulltext Phaeochromocytoma during pregnancy: ultrasound and MRI appearances
    Puvaneswary M, Beckhouse M
    Med J Malaysia, 1992 Mar;47(1):81-5.
    PMID: 1387457
    Matched MeSH terms: Pheochromocytoma/diagnosis*; Pheochromocytoma/ultrastructure
  11. Cystic pheochromocytoma mimicking a malignant pancreatic cyst
    Lee J, Raman K, Sachithanandan S
    Gastrointest Endosc, 2011 Sep;74(3):712-3.
    PMID: 21111418 DOI: 10.1016/j.gie.2010.09.027
    Matched MeSH terms: Pheochromocytoma/diagnosis*; Pheochromocytoma/surgery
  12. Fulltext Renal cell carcinoma in a von Hippel-Lindau syndrome: when should phaeochromocytoma be anticipated?
    Azarisman SM, Nor Azmi K
    Singapore Med J, 2007 Aug;48(8):779-82.
    PMID: 17657389
    A 39-year-old man was diagnosed with von Hippel-Lindau syndrome, which was associated with retinal haemangioblastoma, cervical cord haemangioblastoma and bilateral renal cell carcinoma. He subsequently underwent an arterial embolisation and cervical laminectomy, following a spinal angiogram of the cervical lesion. He also had a right radical nephrectomy, with no perioperative complications. However, on admission for the left radical nephrectomy, he was noted to have preoperative hypertension. Further investigation revealed an enlarged left adrenal gland on abdominal computed tomography scan and raised urinary catecholamines. We discuss the risk of renal cell carcinoma and phaeochromocytoma arising concomitantly in von Hippel-Lindau syndrome, and how best to investigate and manage them.
    Matched MeSH terms: Pheochromocytoma/complications*; Pheochromocytoma/diagnosis
  13. Unusual Bone Superscan, MIBG Superscan, and 68Ga DOTATATE PET/CT in Metastatic Pheochromocytoma
    Tan TH, Wong TH, Hassan SZ, Lee BN
    Clin Nucl Med, 2015 Nov;40(11):867-8.
    PMID: 26252329 DOI: 10.1097/RLU.0000000000000920
    A 17-year-old adolescent boy with biochemically raised 2-hour urinary metanephrine and normetanephrine as well as CT findings of retroperitoneal soft tissue mass and bony metastases was referred for further assessment. Apart from Ga DOTATATE PET/CT evaluation, pretargeted systemic radionuclide therapy assessment with I-MIBG scintigraphy showed unusual phenomenon of MIBG superscan. Postsurgically, restaging Tc-MDP bone scintigraphy showed typical bone superscan features. The MIBG superscan was better delineated on post-I-MIBG therapy images.
    Matched MeSH terms: Pheochromocytoma/pathology; Pheochromocytoma/radionuclide imaging*
  14. Large adrenal leiomyoma presented as adrenal incidentaloma in an AIDS patient: A rare entity
    Huei TJ, Lip HT, Rahman MS, Sarojah A
    Med J Malaysia, 2017 02;72(1):65-67.
    PMID: 28255146 MyJurnal
    The literature on adrenal gland tumour in HIV-infected patients is scarce. We report a 46-year-old Malay man with HIV and Hepatitis C infection presenting with a large nonfunctioning adrenal tumour. Computed tomography showed a large right adrenal tumour with heterogeneous enhancement and central necrosis. A high index of suspicion of a malignant tumour or pheochromocytoma led us to surgical removal of the adrenal gland. In this case report, we highlight important features to look for during pre-op evaluation of a large adrenal mass. Appropriate action should be taken when there is a suspicion of a pheochromocytoma or malignancy.
    Matched MeSH terms: Pheochromocytoma
  15. Fulltext Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features and management
    Foo SH, Chan SP, Ananda V, Rajasingam V
    Singapore Med J, 2010 May;51(5):e89-93.
    PMID: 20593136
    Most functional phaeochromocytomas/paragangliomas produce noradrenaline and/or adrenaline. Those that produce dopamine are rare. We describe the distinguishing clinical features of dopamine-secreting phaeochromocytomas and paragangliomas from those that secrete noradrenaline/adrenaline and the impact on their management. We present a case of a dopamine-secreting paraganglioma from our institution and review 14 case reports of dopamine-secreting phaeochromocytomas/paragangliomas published between 1984 and 2008. As observed in the literature, 80% of the tumours were extra-adrenal. Most patients presented with non-specific symptoms or mass effect without the classical presentation of catecholamine excess. The majority were diagnosed with urinary or plasma dopamine. Five patients had malignant tumours and 12 patients underwent surgical resection of the primary tumours. Unlike noradrenaline/adrenaline-secreting phaeochromocytomas/paragangliomas, dopamine-secreting tumours lack a classical presentation, are extra-adrenal and have a higher malignant potential. A routine inclusion of urinary or plasma dopamine as part of catecholamine screening in all suspected phaeochromocytomas and paragangliomas is recommended.
    Matched MeSH terms: Pheochromocytoma/pathology; Pheochromocytoma/secretion*; Pheochromocytoma/surgery
  16. Secondary hypertension: a condition not to be missed
    Sukor N
    Postgrad Med J, 2011 Oct;87(1032):706-13.
    PMID: 21746730 DOI: 10.1136/pgmj.2011.118661
    Hypertension is a chronic disorder which often entails debilitating cardiovascular and renal complications. Hypertension mostly arises as a complex quantitative trait that is affected by varying combinations of genetic and environmental factors. Secondary hypertension has been encountered with increasing frequency. The common causes of secondary hypertension include renal parenchymal disease, renal artery stenosis, primary aldosteronism, phaeochromocytoma, and Cushing's syndrome. The detection of a secondary cause is of the utmost importance because it provides an opportunity to convert an incurable disease into a potentially curable one. Early identification and treatment will provide a better opportunity for cure, prevent target organ damage, reduce socioeconomic burden and health expenditure associated with drug costs, and improve patients' quality of life. Hence, it is a condition not to be missed.
    Matched MeSH terms: Pheochromocytoma/complications*
  17. Fulltext A rare case of Von Hippel Lindau disease
    Abdul Ghani R, Norazmi MK, Norlaila M
    Med J Malaysia, 2006 Jun;61(2):254-7.
    PMID: 16898326 MyJurnal
    Pheochromocytoma is a rare catecholamine-secreting tumour typically arising within the adrenal medulla. It may occur sporadically or be associated as part of a tumour syndrome including Von Hippel Lindau (VHL), Multiple Endocrine Neoplasia (MEN) 2 and Neurofibromatosis Type 1. VHL is associated with multi-organ involvement of benign and malignant tumours characterized by the presence of retinal angiomas, hemangioblastomas of the cerebellum and spinal cord, renal cell carcinomas, pheochromocytomas and other cystic lesions in the kidneys, pancreas, and epididymis. It is a rare disorder with prevalence estimated at 2-3 per 100,000. This case report describes a 37 years old Chinese gentleman who presented to our institution for further management of bilateral pheochromocytoma and retinal angioblastoma with problems of duodenal ulcer and anaemia. There was no family history of similar problems. With these features the criteria for the diagnosis of von Hippel Lindau disease was established.
    Matched MeSH terms: Pheochromocytoma/diagnosis
  18. Transcriptome Pathway Analysis of Pathological and Physiological Aldosterone-Producing Human Tissues
    Zhou J, Lam B, Neogi SG, Yeo GS, Azizan EA, Brown MJ
    Hypertension, 2016 12;68(6):1424-1431.
    PMID: 27777363
    Primary aldosteronism is present in ≈10% of hypertensives. We previously performed a microarray assay on aldosterone-producing adenomas and their paired zona glomerulosa and fasciculata. Confirmation of top genes validated the study design and functional experiments of zona glomerulosa selective genes established the role of the encoded proteins in aldosterone regulation. In this study, we further analyzed our microarray data using AmiGO 2 for gene ontology enrichment and Ingenuity Pathway Analysis to identify potential biological processes and canonical pathways involved in pathological and physiological aldosterone regulation. Genes differentially regulated in aldosterone-producing adenoma and zona glomerulosa were associated with steroid metabolic processes gene ontology terms. Terms related to the Wnt signaling pathway were enriched in zona glomerulosa only. Ingenuity Pathway Analysis showed "NRF2-mediated oxidative stress response pathway" and "LPS (lipopolysaccharide)/IL-1 (interleukin-1)-mediated inhibition of RXR (retinoid X receptor) function" were affected in both aldosterone-producing adenoma and zona glomerulosa with associated genes having up to 21- and 8-fold differences, respectively. Comparing KCNJ5-mutant aldosterone-producing adenoma, zona glomerulosa, and zona fasciculata samples with wild-type samples, 138, 56, and 59 genes were differentially expressed, respectively (fold-change >2; P<0.05). ACSS3, encoding the enzyme that synthesizes acetyl-CoA, was the top gene upregulated in KCNJ5-mutant aldosterone-producing adenoma compared with wild-type. NEFM, a gene highly upregulated in zona glomerulosa, was upregulated in KCNJ5 wild-type aldosterone-producing adenomas. NR4A2, the transcription factor for aldosterone synthase, was highly expressed in zona fasciculata adjacent to a KCNJ5-mutant aldosterone-producing adenoma. Further interrogation of these genes and pathways could potentially provide further insights into the pathology of primary aldosteronism.
    Matched MeSH terms: Pheochromocytoma/genetics; Pheochromocytoma/physiopathology
  19. Localization and prediction of malignant potential in recurrent pheochromocytoma/paraganglioma (PCC/PGL) using 18F-FDG PET/CT
    Fikri AS, Kroiss A, Ahmad AZ, Zanariah H, Lau WF, Uprimny C, et al.
    Acta Radiol, 2014 Jun;55(5):631-40.
    PMID: 24037430 DOI: 10.1177/0284185113504330
    To our knowledge, data are lacking on the role of 18F-FDG PET/CT in the localization and prediction of neuroendocrine tumors, in particular the pheochromocytoma/paraganglioma (PCC/PGL) group.
    Matched MeSH terms: Pheochromocytoma/radiography*; Pheochromocytoma/radionuclide imaging*
  20. Fulltext Utility of alpha-blockade in a hypotensive pheochromocytoma patient with myocardial infarction
    Wahab NA, Zainudin S, AbAziz A, Kamaruddin NA
    Med Princ Pract, 2015;24(1):96-8.
    PMID: 25428406 DOI: 10.1159/000369021
    OBJECTIVE: The aim of this case study is to emphasize the importance of α-blockade in managing a rare complication of an untreated pheochromocytoma.

    CLINICAL PRESENTATION AND INTERVENTION: A 41-year-old man with previous bilateral pheochromocytoma presented with chest pain. He was suffering from cardiac failure and persistent hypotension requiring an inotrope. Cardiac markers, an electrocardiogram and an echocardiogram confirmed acute myocardial infarct with poor ejection fraction and global hypokinesia. An (18)F-fluorodeoxyglucose PET/CT scan showed progressive left suprarenal and organ of Zuckerkandl pheochromocytomas. Blood pressure stabilisation proved challenging but was achieved by titrating an incremental dose of α-blocker against a tapering inotropic dose.

    CONCLUSION: This case showed the efficacy of an α-blocker despite persistent hypotension in a patient with pheochromocytoma-induced cardiomyopathy.

    Matched MeSH terms: Pheochromocytoma/complications
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