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Cardiac Paraganglioma In A Familial Paraganglioma Syndrome - Coronary Artery embolization For Nonresectable Tumor. Zanariah H 1 , Alfazir O 2 , Rosli MA 2 1 Endocrinology Unit, Department of Medicine, Hospital Putrajaya 2 Department of Cardiology, National Heart Institute (IJN) Primary cardiac paraganglioma is a very rare tumor with less than 60 reported cases in literature to date. Clinical presentation is variable,most commonly manifested by hypertension and symptoms related to catecholamine excess such as palpitations, headache and sweating. Most are benign and most commonly arise from the left atrial wall. The tumor is often poorly encapsulated and local invasion can occur with metastases reported in up to 10% of cases. Surgical excision is the treatment of choice, often complex and necessitating cardiopulmonary bypass. It is often difficult to detect preoperatively the degree of extension of the tumor and its resectability. Patients with non-resectable, intracardiac tumors have been considered for cardiac transplantation. Embolization of feeding coronary arteries to the tumor has been performed, mainly as a preoperative procedure to reduce perioperative bleeding. We describe a case of non-resectable functioning cardiac paraganglioma who underwent embolization of coronary arteries with subsequent improvement in clinical status, normalization of urinary catecholamines and reduction in tumor size. A 42-year-old Chinese male presented with recent onset hypertension and diabetes with an abnormal chest radiograph. He was otherwise asymptomatic. He was on metformin monotherapy with a HbA1c of 7.6% and had severe hypertension of 180/140 mmhg with a postural drop to 150/120 mmHg at presentation to our center. He had previous surgical excision of bilateral carotid paragangliomas. His sister was recently diagnosed to have abdominal paraganglioma with liver metastases. Initial CT scan noted a mediastinal mass adjacent to the main pulmonary artery measuring 6.3 x 3.9 cm and normal appearance of both adrenal glands. Coronary angiogram revealed a highly vascular cardiac mass adjacent to pulmonary artery with feeding vessels from the Right coronary (RCA) and Left anterior descending (LAD) arteries. Twenty-four hour urinary noradrenaline levels were elevated at 19236nmol (normal 40 – 780 nmol) with normal urinary adrenaline levels. MIBG scan revealed a faint tracer uptake in the heart and otherwise physiological uptake elsewhere. Cardiothoracic consultation concluded that the tumor was inoperable. The patient was referred to the cardiologist for consideration of tumor embolization. He was prepared with oral phenoxybenzamine and later addition of propranolol. Coronary angiogram was performed with alcohol ablation of the tumor and placement of a helical coil in the main feeder artery to the tumor. On follow-up, blood pressure gradually normalized and antihypertensives were withdrawn. Urinary catecholamines had normalized at three months follow-up with CT scan showing tumor size reduction. Follow-up to detect regrowth of cardiac tumor and development of additional paragangliomas at other sites is important. He most likely has a succinate dehydrogenase (SDH) gene mutation giving rise to familial and multifocal disease. Genetic studies are necessary for him and his family to enable early diagnosis and disease prognostication. 27
Carney Complex: Cardiac Myxoma And Cushing’s Syndrome Norhaliza MA, Zanariah H, Masni M Endocrinology Unit, Department of Medicine, Hospital Putrajaya Carney complex is a rare multiple neoplasia syndrome, comprising the association of cardiac and cutaneous myxomas, pigmented skin lesions, endocrine tumors, and melanocytic schwannomas, first reported by Carney in 1985. Endocrine tumors observed include growth hormone secreting pituitary adenomas, thyroid adenomas or carcinomas, testicular tumors and ovarian cysts. Adrenocorticotropic hormone (ACTH)-independent Cushing's syndrome due to primary pigmented nodular adrenal disease (PPNAD) is observed in 25 to 30 % of patients. It is transmitted as an autosomal dominant trait. We report two cases, both presenting with Cushing’s syndrome due to bilateral nodular adrenal hyperplasia and cardiac myxoma. The first case is a 39-year-old lady, recently diagnosed with ACTH-independent Cushing’s syndrome, presented with uncontrolled hypertension and excessive weight gain. She had previous surgical resection of cardiac myxoma at age 17 years and later underwent cystectomy for recurrent left ovarian cyst. Her elder sister has acromegaly due to a pituitary adenoma. Computer tomography (CT) showed a nodular right adrenal gland and normal appearance of the contralateral gland. With a probable diagnosis of Carney complex and likelihood of bilateral adrenocortical hyperplasia, she underwent laparoscopic bilateral adrenalectomy, which confirmed the diagnosis of primary pigmented nodular adrenal disease (PPNAD) in both adrenals. The second case is a 23 year-old-lady with initial presentation of Cushing’s syndrome, nondetectable ACTH levels and CT scan evidence of bilateral nodular adrenal enlargement. She underwent a bilateral adrenalectomy which confirmed bilateral macronodular adrenal hyperplasia in keeping with PPNAD. She also had spotty facial hyperpigmentation. Six years later, she presented with acute breathlessness and palpitations. Echocardiography showed a left atrial tumor, confirmed to be an atrial myxoma following resection. She remained well until recent review revealed recurrence of cardiac myxoma , 3 years after the initial removal. She had no family history of any of the components of Carney complex. Cardiac myxoma and PPNAD are characteristic components of the Carney complex. Cardiac tumors and are often multi-centric, having a high risk for recurrence. Awareness of the Carney complex and of the importance of its various components will facilitate identification of affected patients and screening of their first-degree relatives. 28
Page 1 and 2: Vol 24, No 1 (SUPPLEMENT) ISSN 0857
Page 3 and 4: CLINICAL TRIALS AND STUDIES
Page 5 and 6: A Randomized Control Trial To Asses
Page 7 and 8: Abnormal Glucose Tolerance In Tanju
Page 9 and 10: Binge Eating Behavior And The Prefe
Page 11 and 12: Gene Expression Profiling Associate
Page 13 and 14: Growth Hormone Deficiency And Cogni
Page 15 and 16: Prolonged QTc Interval Is Not Uncom
Page 17 and 18: Prevalence Of Glucose Intolerance I
Page 19 and 20: Role of Adrenal Venous Sampling in
Page 21 and 22: The Prevalence Of Metabolic Syndrom
Page 23 and 24: Treatment Outcome And Complications
Page 25 and 26: CASE REPORTS 22
Page 27 and 28: A Case Of Persistent Parathyroid Ca
Page 29: Bilateral Adrenal Hyperplasia In Mu
Page 33 and 34: McCune-Albright Syndrome Dr. Suhaim
Page 35 and 36: The Case Of A Troubled Patient, Tro
Page 37: TSH- Secreting Pituitary Macroadeno

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