MyMedR

A Case of Paraganglioma with Cyanotic Congenital Heart Disease

Wahab NA, Chien BH, Omar MR, Aziz AA, Mustafa N, Sukor N, et al.

Acta Med Indones, 2021 Jan;53(1):77-81.

Co-occurrence of cyanotic congenital heart disease (CCHD) and phaeochromocytoma (PCC) and paraganglioma (PGL) are rare, although some cases have been reported. We report a case of left paraganglioma in a 20-year-old lady with an underlying CCHD who underwent palliative Glenn shunt, subsequently developed polycythaemia and cavernous sinus thrombosis presented with palpitation, sweating, headache and hypertension of 3-months duration at the age of 17. The abdominal CT scan revealed an enhancing left paraaortic mass measuring 5.2 cm x 4.4 cm x 3.8 cm. A 24-hour urine catecholamine demonstrated raised noradrenaline level to six times upper limit of normal and hence diagnosis of left sympathetic (sPGL) was made. In view of the delayed diagnosis and significant morbidity associated with her condition, surgical treatment is no longer an option. Therefore, vigilant screening and early treatment of PCC-PGL in patients with CCHD are crucial in order to avoid significant morbidity and ensure a good quality of life.

Matched MeSH terms: Adrenal Gland Neoplasms

Fulltext Diagnostic approach to a functional adrenal tumour in a Shih-Tzu Poodle mix

Tan, L.P., Thong, H.F., Lim, S.Y.

Jurnal Veterinar Malaysia, 2016;28(2):21-23.

MyJurnal

Hyperadrenocorticism is the excessive production of cortisol by the adrenal cortex. 15-20% of hyperadrenocorticism in dogs may be due to functional adrenal tumour. Here we present a case of functional adrenal tumour in a 10 years old Shih-Tzu Poodle mixed-breed dog that was presented to University Veterinary Hospital, Universiti Putra Malaysia. Physical examination findings include multiple cutaneous myiasis, a pendulous abdomen with comedones on the ventral thoracic region and thinning of the skin. Clinical signs shown were polyuria, polydipsia and polyphagia. Serum biochemistry result showed elevated alkaline phosphatase enzyme of 5 folds. Urinary specific gravity was minimally concentrated (1.015). Radiography showed generalised hepatomegaly. Meanwhile, ultrasonography showed unilateral enlargement of the right adrenal gland with mineralisation. Invasion of the right adrenal gland into the caudal vena cava was observed. A differentiation test (high-dose dexamethasone suppression test) was performed and a lack of suppression of the cortisol level was observed. Based on the appropriate clinical signs and physical examination findings, elevated alkaline phosphatase enzyme, minimally concentrated urinary specific gravity, generalised hepatomegaly, unilateral adrenal gland enlargement, and high-dose dexamethasone suppression test, a diagnosis of functional adrenal tumour was made.

Matched MeSH terms: Adrenal Gland Neoplasms

Fulltext An unusual case of shortness of breath

Wan Muhammad Hatta SF, Kandaswamy L, Gherman-Ciolac C, Mann J, Buch HN

Endocrinol Diabetes Metab Case Rep, 2018;2018.

PMID: 30087779 DOI: 10.1530/EDM-18-0074

Myopathy is a well-known complication of hypercortisolism and commonly involves proximal lower-limb girdle. We report a rare case of Cushing's syndrome in a 60-year-old female presenting with significant respiratory muscle weakness and respiratory failure. She had history of rheumatoid arthritis, primary biliary cirrhosis and primary hypothyroidism and presented with weight gain and increasing shortness of breath. Investigations confirmed a restrictive defect with impaired gas transfer but with no significant parenchymatous pulmonary disease. Respiratory muscle test confirmed weakness of respiratory muscles and diaphragm. Biochemical and radiological investigations confirmed hypercortisolaemia secondary to a left adrenal tumour. Following adrenalectomy her respiratory symptoms improved along with an objective improvement in the respiratory muscle strength, diaphragmatic movement and pulmonary function test.

Learning points: Cushing's syndrome can present in many ways, a high index of suspicion is required for its diagnosis, as often patients present with only few of the pathognomonic symptoms and signs of the syndrome.Proximal lower-limb girdle myopathy is common in Cushing's syndrome. Less often long-term exposure of excess glucocorticoid production can also affect other muscles including respiratory muscle and the diaphragm leading to progressive shortness of breath and even acute respiratory failure.Treatment of Cushing's myopathy involves treating the underlying cause that is hypercortisolism. Various medications have been suggested to hinder the development of GC-induced myopathy, but their effects are poorly analysed.

Matched MeSH terms: Adrenal Gland Neoplasms

Fulltext Co-Occurrence of Exogenous and Endogenous Cushing's Syndromes-Dilemma in Diagnosis

Tong CV, Rajoo S

Case Rep Endocrinol, 2019;2019:2986312.

PMID: 31737375 DOI: 10.1155/2019/2986312

Approach to patients who manifest with features of Cushing's syndrome often begin with exclusion of exposure to excessive exogenous source of glucocorticoids (GC). Most guidelines advocate no further assessment if excessive exogenous GC use is present. We present a case of a 66-year-old lady who was noted to have typical features of Cushing's syndrome. As she gave a very clear history of ingesting exogenous GC for a year, no further work up was undertaken. Despite cessation of GC for a year, she continued to have thin skin and easy bruising. Upon admission for hypertensive emergency, her clinician took note of her changes and investigated her for endogenous Cushing's syndrome. Her cortisol post overnight dexamethasone suppression test was 707 nmol/l. Post low dose dexamethasone suppression test yielded a cortisol of 1133.2 nmol/l. 24 hours urine cortisol was 432.2 nmol/l. Plasma ACTH was 1.1 pmol/l, indicating an ACTH independent Cushing's syndrome. We proceeded with Computed tomography scan (CT scan) of adrenals which revealed a right adrenal adenoma measuring 4.4 × 3.4 × 4.0 cm. Right retroperiteneoscopic adrenalectomy was done. Histopathology examination was consistent with adrenal cortical adenoma with foci of myelolipoma. Post adrenalectomy she developed hypocortisolism secondary to contralateral adrenal suppression which lasted up to the present date. Her cutaneous and musculoskeletal manifestations improved substantially. Co-occurrence of endogenous and exogenous Cushing's syndromes is uncommon but should be considered in patients whose Cushingnoid features do not resolve after cessation of exogenous GC.

Matched MeSH terms: Adrenal Gland Neoplasms

Unusual presentation of pheochromocytoma with ischemic sigmoid colitis and stenosis

Tan F, Thai AC, Cheah WK, Mukherjee JJ

South. Med. J., 2009 Oct;102(10):1068-70.

PMID: 19738530 DOI: 10.1097/SMJ.0b013e3181b571e6

A 45-year-old woman with poorly controlled hypertension and diabetes mellitus presented with left iliac fossa pain, constipation alternating with diarrhea, and weight loss. She had been diagnosed with idiopathic cardiomyopathy five years previously. Echocardiogram had shown a left ventricular ejection fraction (LVEF) of 35%; coronary angiogram was normal. Colonoscopy revealed sigmoid colitis with stenosis. Abdominal computed tomography revealed a 5 cm right adrenal tumor. Twenty-four hour urinary free catecholamines and fractionated metanephrine excretion values were elevated, confirming pheochromocytoma. Her colitis resolved after one month of adrenergic blockade. Repeat echocardiogram showed improvement of LVEF to 65%. After laparoscopic right adrenalectomy, the patient's hypertension resolved, and diabetic control improved. Timely management avoided further morbidity and potential mortality in our patient.

Matched MeSH terms: Adrenal Gland Neoplasms/diagnosis*; Adrenal Gland Neoplasms/therapy

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: Adrenal Gland Neoplasms/complications

Fulltext Early presentation of right adrenal mass, hepatoblastoma and hepatic cavernous haemangioma in Beckwith-Wiedemann Syndrome

Teh SH, Ong GB

Med J Malaysia, 2007 Oct;62(4):345-6.

PMID: 18551945 MyJurnal

Beckwith-Wiedemann Syndrome (BWS) is associated with early development of embryonal tumours usually in the first four years of life. We describe a patient who presented with a right adrenal cyst in the first month of life and hepatoblastoma in the third month of life. A cavernous haemangioma was subsequently found in the resected tumour.

Matched MeSH terms: Adrenal Gland Neoplasms/diagnosis

Fulltext Primary Hyperaldosteronism in End-Stage Renal Disease: Diagnostic Challenges and Treatment Considerations

Chand R, Tandukar S, Asmil S, Chico M

Cureus, 2020 Aug 07;12(8):e9599.

PMID: 32923203 DOI: 10.7759/cureus.9599

An adrenal incidentaloma is defined as an adrenal mass measuring at least 1 cm that is discovered surreptitiously in an imaging study done for reasons other than the evaluation of adrenal disease. The increase in the prevalence of adrenal incidentalomas has paralleled the increase in diagnostic imaging done for evaluation of other abdominal pathologies. However, most of these adrenal incidentalomas are benign non-hyperfunctioning adenomas. When an adrenal incidentaloma is discovered, the simultaneous presence of hypokalemia, metabolic alkalosis, mild hypernatremia, and mild to severe drug-resistant hypertension may alert a clinician to underlying primary hyperaldosteronism. We present a case of adrenal incidentaloma noted in a patient with end-stage renal disease on hemodialysis which presented a diagnostic challenge due to the correction of metabolic parameters with hemodialysis. The patient was found to have an aldosterone-producing adenoma based on an elevated aldosterone-to-renin ratio and was started on a mineralocorticoid antagonist.

Matched MeSH terms: Adrenal Gland Neoplasms

Fulltext A clinically silent tumour of adrenal myelolipoma: A case report

Azizan N, Myint O, Wynn AA, Thein TT, Hayati F, Nik Lah NAS

Int J Surg Case Rep, 2020;72:63-65.

PMID: 32506033 DOI: 10.1016/j.ijscr.2020.05.056

INTRODUCTION: Adrenal myelolipoma is a rare, non-functional, benign neoplasm which is constituted of mature haematopoietic elements and adipose tissues in various proportions. It is diagnosed accidentally and frequently with the widespread use of imaging modalities.

PRESENTATION OF CASE: We report a 63-year-old lady with incidental findings of adrenal tumour on computed tomography (CT) scan during a routine medical check-up. She underwent tumour resection in view of a large tumour of 7 cm in size.

DISCUSSION: CT scan is sensitive to diagnose adrenal myelolipoma in view of its fat-laden property and useful to monitor the tumour progress. Even previously she opted for conservative management; the decision for surgery was made in view of enlarging tumour and risk of surrounding tissue compression.

CONCLUSION: With increased awareness, the detection rate of this tumour is improving, hence able to prevent the complications of a large tumour such as compression, bleeding and tumour necrosis.

Matched MeSH terms: Adrenal Gland Neoplasms

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: Adrenal Gland Neoplasms/radiography*; Adrenal Gland Neoplasms/radionuclide imaging*

Fulltext Diffuse neonatal haemangiomatosis: a rare cause of haemorrhagic shock and refractory coagulopathy in the newborn

Rohana J, Boo NY, Hayati AR, Baizura J

Med J Malaysia, 2002 Sep;57(3):364-7.

PMID: 12440278

A term newborn infant developed hypovolaemic shock shortly after birth. She was pale with gross hepatomegaly. She required multiple boluses of intravenous fluids, blood products as well as inotropic support. Blood investigations showed persistent thrombocytopenia, anaemia and disseminated intravascular coagulopathy (DIC). She also developed heart failure. She finally succumbed on the eleventh day of life. Autopsy revealed haemangiomatosis involving the liver, lungs, gastrointestinal tract, kidneys and adrenals.

Matched MeSH terms: Adrenal Gland Neoplasms/complications*; Adrenal Gland Neoplasms/congenital*

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: Adrenal Gland Neoplasms/complications*

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: Adrenal Gland Neoplasms/diagnosis

18F-FDG Uptake in Benign Fibrous Histiocytoma of Adrenal Gland: A Potential Pitfall

Tan TH, Lai CNB

Clin Nucl Med, 2017 Aug;42(8):622-623.

PMID: 28632691 DOI: 10.1097/RLU.0000000000001730

A 47-year-old man with newly diagnosed nasopharyngeal carcinoma underwent staging F-FDG PET/CT. Apart from showing increased FDG uptake in the primary site and locoregional nodal and liver metastases, an unusual site of intense FDG focus was demonstrated in the left adrenal gland. He underwent CT-guided biopsy, and the histopathologic diagnosis was benign fibrous histiocytoma.

Matched MeSH terms: Adrenal Gland Neoplasms/metabolism*

Endogenous Cushing syndrome from an ectopic adrenocorticotropic hormone production as a rare cause of ocular hypertension

Khaw KW, Jalaludin MY, Suhaimi H, Harun F, Subrayan V

J AAPOS, 2010 Aug;14(4):356-7.

PMID: 20637667 DOI: 10.1016/j.jaapos.2010.04.009

Ocular hypertension caused by endogenous Cushing syndrome from an ectopic adrenocorticotropic hormone-producing tumor is rare. We report an 11-year-old boy who presented with intraocular pressures (IOPs) of 50 mm Hg in both eyes. Surgical resection of the tumor was performed with subsequent normalization of serum cortisol and IOP levels.

Matched MeSH terms: Adrenal Gland Neoplasms/complications*; Adrenal Gland Neoplasms/diagnosis; Adrenal Gland Neoplasms/metabolism

Fulltext Pregnancy, Primary Aldosteronism, and Adrenal CTNNB1 Mutations

Teo AE, Garg S, Shaikh LH, Zhou J, Karet Frankl FE, Gurnell M, et al.

N Engl J Med, 2015 Oct 08;373(15):1429-36.

PMID: 26397949 DOI: 10.1056/NEJMoa1504869

Recent discoveries of somatic mutations permit the recognition of subtypes of aldosterone-producing adenomas with distinct clinical presentations and pathological features. Here we describe three women with hyperaldosteronism, two who presented in pregnancy and one who presented after menopause. Their aldosterone-producing adenomas harbored activating mutations of CTNNB1, encoding β-catenin in the Wnt cell-differentiation pathway, and expressed LHCGR and GNRHR, encoding gonadal receptors, at levels that were more than 100 times as high as the levels in other aldosterone-producing adenomas. The mutations stimulate Wnt activation and cause adrenocortical cells to de-differentiate toward their common adrenal-gonadal precursor cell type. (Funded by grants from the National Institute for Health Research Cambridge Biomedical Research Centre and others.).

Matched MeSH terms: Adrenal Gland Neoplasms/genetics*; Adrenal Gland Neoplasms/metabolism; Adrenal Gland Neoplasms/pathology

Fulltext Partial adrenal suppression with prolonged use of Depo-Provera

Nur Aisyah Zainordin, Fatimah Zaherah Mohd Shah, Rohana Abdul Ghani

Journal of Clinical and Health Sciences, 2019;4(1):64-66.

MyJurnal

A 49-year old patient presented with symptoms of adrenal suppression following an attempt to
withdraw Depo-Provera or Depot Medroxyprogesterone Acetate (DMPA) injection. She had
been receiving DMPA injections for the past 16 years for contraception. She was initially
prescribed DMPA by her gynaecologist but later on began obtaining the medication directly
from a private pharmacy without prior consultation from her gynaecologist. Clinically, she had
been experiencing significant weight gain and appeared cushingoid. Blood investigations
confirmed partial adrenal suppression with presence of an adrenal incidentaloma. This case
reports a known side effect of DMPA but occurring at a much lower dose than previously
described. It also highlights the need to increase the awareness of the insidious side effect of
DMPA and to avoid unsupervised use of the drug.

Matched MeSH terms: Adrenal Gland Neoplasms

Fulltext Solitary adrenal metastasis from invasive infiltrating ductal carcinoma: A case report and review of literature

Sangeetha Poovaneswaran, Justin Zon Ern Lee, Whei Ying Lim, Navarasi S Raja Gopal, Fauziah Mohd Dali, Ibtisam Mohamad

International e-Journal of Science, Medicine & Education, 2013;7(1):33-36.

MyJurnal

Solitary adrenal metastasis is a rare presentation in breast cancer and it presents the clinician with a difficult therapeutic dilemma as there are no existing guidelines for optimal management. On literature review, we only found one published case report of solitary adrenal metastasis from infiltrating ductal carcinoma of the breast. Here we present a case of a 75 year-old lady who presented with a right breast lump which was subsequently confirmed to be infiltrating ductal carcinoma. She underwent a right mastectomy and axillary clearance. Computerised tomography (CT) staging revealed a solitary adrenal metastasis. She was treated with aromatase inhibitors and her tumour markers which were initially raised has now normalised.

Matched MeSH terms: Adrenal Gland Neoplasms

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: Adrenal Gland Neoplasms

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: Adrenal Gland Neoplasms/pathology; Adrenal Gland Neoplasms/secretion*; Adrenal Gland Neoplasms/surgery

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