MyMedR

A case of glomus jugulare tumour

Ghee LT, Devaraj TP

Med J Malaya, 1972 Sep;27(1):69-72.

PMID: 4345647

Matched MeSH terms: Paraganglioma, Extra-Adrenal/radiography*

Fulltext Ectopic ACTH syndrome - Experience with Etomidate

Tong CV, Hussein Z

J ASEAN Fed Endocr Soc, 2017;32(1):54-56.

PMID: 33442086 DOI: 10.15605/jafes.032.01.10

For ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS), when surgery is not feasible, or in cases of severe biochemical disturbances, immunosuppression or mental instability, medical therapy with agents such as etomidate is indicated. We present our experience in using etomidate for a 41-year old female with EAS secondary to a malignant mediastinal paraganglioma. We were able to demonstrate that etomidate can be used effectively to control severe hypercortisolism in a lower dose than previously described.

Matched MeSH terms: Paraganglioma; Paraganglioma, Extra-Adrenal

Education and imaging. Hepatobiliary and pancreatic: A huge liver paraganglioma

Koh PS, Koong JK, Westerhout CJ, Yoong BK

J Gastroenterol Hepatol, 2013 Jul;28(7):1075.

PMID: 23782121 DOI: 10.1111/jgh.12254

Matched MeSH terms: Paraganglioma, Extra-Adrenal/pathology*; Paraganglioma, Extra-Adrenal/radiography*; Paraganglioma, Extra-Adrenal/surgery

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: Paraganglioma

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: Paraganglioma/diagnosis*

Surgical management of tympanojugular paragangliomas with intradural extension, with a proposed revision of the Fisch classification

Sivalingam S, Konishi M, Shin SH, Lope Ahmed RA, Piazza P, Sanna M

Audiol. Neurootol., 2012;17(4):243-55.

PMID: 22584244 DOI: 10.1159/000338418

Tympanojugular paragangliomas (TJPs) with intradural extension can be successfully treated by a single or staged procedure with low surgical morbidity.

Matched MeSH terms: Paraganglioma, Extra-Adrenal/classification; Paraganglioma, Extra-Adrenal/surgery*

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: Paraganglioma/complications; Paraganglioma/diagnosis*

Malignant paraganglioma of frontoethmoidal region

Sharma HS, Madhavan M, Othman NH, Muhamad M, Abdullah JM

Auris Nasus Larynx, 1999 Oct;26(4):487-93.

PMID: 10530746

Nonchromaffin paragangliomas are unusual tumours arising from widely distributed paraganglionic tissues probably of neural crest origin. In the head and neck region they are usually seen as carotid body or jugulotympanic tumours. Other rarely reported sites in the head and neck region are the orbit, nose and larynx. This report deals with a case of sinonasal paraganglioma which was initially treated with surgery and radiotherapy. Twenty two years later the tumour recurred and showed a rapid growth due to malignant transformation which we believe is late effect of radiotherapy. The clinical features, histopathology and role of radiotherapy in sinonasal paragangliomas together with a review of the medical literature have been discussed.

Matched MeSH terms: Paraganglioma/pathology*; Paraganglioma/therapy

Fulltext Surgical access to parapharyngeal space tumours--the Manipal experience

Hazarika P, Dipak RN, Parul P, Kailesh P

Med J Malaysia, 2004 Aug;59(3):323-9.

PMID: 15727377

A few series of parapharyngeal space tumours have been reported earlier but recently not many series have been published in English literature. It is rare for any medical center, let alone an individual surgeon, to develop sufficient experience in evaluating these tumours. We present our experience in the treatment of 41 cases of parapharyngeal tumours from January 1992 to December 2001. FNAC, ultrasound and CT scan of the presenting mass was done in most of the patients as the main pre-operative work-up. The strategic location and extension of the tumour may occasionally alter the surgical approach for tumour excision.

Matched MeSH terms: Paraganglioma/complications; Paraganglioma/diagnosis; Paraganglioma/pathology; Paraganglioma/surgery

Fulltext Extra-adrenal paraganglioma: presentation in three uncommon locations

Mun KS, Pailoor J, Chan KS, Pillay B

Malays J Pathol, 2009 Jun;31(1):57-61.

PMID: 19694315 MyJurnal

Extra-adrenal paragangliomata are uncommon entities. They can be classified into four basic groups according to their anatomical sites, i.e. branchiomeric, intravagal, aorticosympathetic and visceral autonomic. Similar tumours may arise in sites away from the usual distribution of the sympathetic and parasympathetic ganglia, e.g. orbit, nose, small intestine and even in the pancreas. We report three instructive cases of extra-adrenal paraganglioma which were found in unusual sites such as urinary bladder, thyroid gland and on the wall of the inferior vena cava.

Matched MeSH terms: Paraganglioma, Extra-Adrenal/metabolism; Paraganglioma, Extra-Adrenal/pathology*; Paraganglioma, Extra-Adrenal/surgery

Fulltext Glomus tympanicum

Appannan VR, Md Daud MK

Malays Fam Physician, 2018;13(1):45-48.

PMID: 29796211

Glomus tympanicum is a tumour classified under the group glomus tumours, and is also known as paragangliomas. It is thought to commonly occur in women in the fifth to sixth decades of life. Here, we report a case of a 77-year-old lady with multiple co-morbids and a diagnosis of glomus tympanicum presenting to us. Her symptoms included pulsatile tinnitus, and reduced hearing, and the management of the case was done with consideration for her underlying multiple co-morbidities. This paper also describes the best modality of treatment for this patient with regard to her background history. The treatment goal was to improve her quality of life and control the disease.

Matched MeSH terms: Paraganglioma

Fulltext Glomus tympanicum

Appannan, V.R., Md Daud, M.K.

Malaysian Family Physician, 2018;13(1):45-48.

MyJurnal

Glomus tympanicum is a tumour classified under the group glomus tumours, and is also known
as paragangliomas. It is thought to commonly occur in women in the fifth to sixth decades of life.
Here, we report a case of a 77-year-old lady with multiple co-morbids and a diagnosis of glomus
tympanicum presenting to us. Her symptoms included pulsatile tinnitus, and reduced hearing,
and the management of the case was done with consideration for her underlying multiple comorbidities. This paper also describes the best modality of treatment for this patient with regard
to her background history. The treatment goal was to improve her quality of life and control the
disease.

Matched MeSH terms: Paraganglioma

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: Paraganglioma/pathology; Paraganglioma/secretion; Paraganglioma/surgery

Fulltext The value of pre-operative embolisation in primary inferior vena cava paraganglioma

Zaki FM, Osman SS, Abdul Manaf Z, Mahadevan J, Yahya M

Malays J Med Sci, 2011 Apr;18(2):70-3.

PMID: 22135590

We report a case of a 13-year-old boy who complained of progressive abdominal distension and symptoms of anaemia. Radiological investigations revealed that the child had a hypervascular tumour of the inferior vena cava (IVC). Unfortunately, the child presented with acute lower gastrointestinal bleed soon after the investigation. He underwent an urgent pre-operative embolisation, aimed to reduce the tumour vascularity. A total resection of the tumour, right nephrectomy, and partial duodenal resection were done within 24 hours post-embolisation. The child was stable postoperatively. The histopathological examination revealed chromogranin-positive paraganglioma originating from the IVC. We highlight the radiological findings of rare primary IVC paraganglioma and the role of embolisation prior to surgical removal of the tumour.

Matched MeSH terms: Paraganglioma

Paroxysmal Hypertension Associated With Urination

Lou Y, Fan L, Hou X, Dominiczak AF, Wang JG, Staessen JA, et al.

Hypertension, 2019 11;74(5):1068-1074.

PMID: 31564165 DOI: 10.1161/HYPERTENSIONAHA.119.13140

Matched MeSH terms: Paraganglioma/pathology; Paraganglioma/surgery*

Fulltext Bilateral Carotid Body Tumours

Amran, A.R., Jayaram, G., Kumar, G.

International Medical Journal Malaysia, 2006;5(1):1-6.

MyJurnal

Paragangliomas are slow growing hypervascular tumour arising from neural crest cell derivatives throughout the body. In the head and neck region, the major paraganglial cells are located at the carotid bifurcation (carotid body), along the ganglia of the vagus nerve and along the nerves supplying the middle ear and jugular bulb. Less common locations include the larynx, orbit, nose and the aortic arch. Carotid body tumours are very rare neoplasms constituting less than 0.5% of all tumours. The true nature of the tumour is established at the time of attempted biopsy or surgical resection, sometimes with disastrous consequences. Only a few of the more than 500 cases reported in the literature have been studied and diagnosed preoperatively. These tumours must be considered in the evaluation of any lateral neck mass, even one located far from the carotid bifurcation. We report a case of bilateral carotid body tumours detected using 16-slice MDHCT in a patient who presented with pulsatile neck swelling for two years. This tumour is rarely malignant; however it produces serious problems by its progressive enlargement and impairment of adjacent structures in the neck. The treatment of choice is surgical resection; preferably to excise this lesion when it is small, because large, very vascular tumours are intimately attached to the carotid vessels and make surgical resection more hazardous. Pre-operative embolization can be performed in large tumours, as to facilitates surgery and reduce complication. In most of the cases, there is strong attachment of the tumour to the carotid arteries. When this is the situation, removal of the tumour means complete excision of both great arteries as well, and the mortality rate is 40 to 45 %. When surgical removal is not feasible, radiotherapy may help to control the tumour growth, although the results have not been encouraging. As it is very slow growing tumour with the growth rate of less than 5 mm per year tumours in old patients with significant risk factors for surgical intervention can be managed by observation alone.

Matched MeSH terms: Paraganglioma

Fulltext An Undiagnosed Non Functioning Retroperitoneum Paraganglioma, Complicating A Gynaecological Surgery; A Case Report

Noraziana, A.W., Hakim, B., Mokhtar, A., Alik, R.

International Medical Journal Malaysia, 2010;9(1):59-61.

MyJurnal

Paragangliomas are uncommon tumors arising from the neuroendocrine elements (chief cells) of the paraganglia and symmetrically distributed along the aorta in close association with the sympathetic chain. Although functional tumors are easier to diagnose due to the clinical fi ndings caused by excess secretion of catecholamines and an elevation of the urinary catecholamine levels, however the diagnosis of nonfunctioning paraganglioma of the retroperitoneum is diffi cult and only few cases have been made before exploratory laparotomy. Although our patient was initially planned and operated by our gynaecological team, the incidental findings had to involve the general surgeons. She is fortunate that the tumour did not show any features of malignancy nor metastasis, however a long time follow up is needed in case of any possible recurrence.

Matched MeSH terms: Paraganglioma

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: Paraganglioma/radiography*

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.

PMID: 33818409

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: Paraganglioma

Immediate selective laryngeal reinnervation in vagal paraganglioma patients

Mat Baki M, Clarke P, Birchall MA

J Laryngol Otol, 2018 Sep;132(9):846-851.

PMID: 30180919 DOI: 10.1017/S0022215118000476

OBJECTIVE: This prospective case series aimed to present the outcomes of immediate selective laryngeal reinnervation.

METHODS: Two middle-aged women with vagal paraganglioma undergoing an excision operation underwent immediate selective laryngeal reinnervation using the phrenic nerve and ansa cervicalis as the donor nerve. Multidimensional outcome measures were employed pre-operatively, and at 1, 6 and 12 months post-operatively.

RESULTS: The voice handicap index-10 score improved from 23 (patient 1) and 18 (patient 2) at 1 month post-operation, to 5 (patient 1) and 1 (patient 2) at 12 months. The Eating Assessment Tool 10 score improved from 20 (patient 1) and 24 (patient 2) at 1 month post-operation, to 3 (patient 1) and 1 (patient 2) at 12 months. There was slight vocal fold abduction observed in patient one and no obvious abduction in patient two.

CONCLUSION: Selective reinnervation is safe to perform following vagal paraganglioma excision conducted on the same side. Voice and swallowing improvements were demonstrated, but no significant vocal fold abduction was achieved.

Matched MeSH terms: Paraganglioma/surgery*

Displaying all 20 publications