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A Series Of Five Cases Of Adrenocortical Carcinoma Norasyikin AW, Nor Shafinas NA, Norhaliza MA, Wong Ming, Foo, Suezhazlyn Z, Norlaila M, Rohaizak M, Nor Azmi K Departments of Medicine and Surgery, UKM Medical Center, Kuala Lumpur. Abstract Adrenocortical carcinoma (ACC) is an extremely rare tumor. Although it is potentially curable in the early stages, only a third is confined to the adrenal gland at the time of diagnosis. Five cases of ACC with various presentations, managed in our institution within the last 2 years are discussed in the light of associated issues in their management. Of the three ACC cases that presented with Cushing’s syndrome, two were complicated by uncontrolled hypertension and psychosis as a result of excessive corticosteroid levels. Three of the patients had large tumours, two of whom had left adrenal tumor measuring greater than 6 cm in diameter (fig 1) whilst the third had a right adrenal tumour with the same dimension (fig 2). Figure 1 Figure 2 Of the three functioning tumours, surgical intervention was not performed in two as they had succumbed to overwhelming sepsis despite being on adrenalytic therapy. The remaining patient underwent a successful debulking surgery followed by mitotane therapy. The 4th case was a middle-aged Chinese man who presented with a back pain of two year duration. The abdominal CT scan revealed an inhomogeneous right adrenal mass measuring 3x4x6 cm. He was subjected to a successful adrenalectomy. Unfortunately, he declined any further treatment after the surgery. The last case was that of a young Malay school girl who presented with pulmonary embolism. Abdominal and thoracic CT scans revealed a huge ACC measuring 10x12x13cm with inferior vena cava and right atrial extensions (fig 3). She underwent an uneventful radical thoracoabdominal surgery with the aid of a cardio-pulmonary bypass procedure. Mitotane therapy was started before the operation and the dose was titrated as tolerated (This case is described separately in detail elsewhere in this proceeding). Figure 3 Conclusion: Surgery seems to offer the only satisfactory mode of treatment in ACC regardless of the stages while adrenocorticolytic therapy should be commenced as soon as possible. Efforts to control excessive corticosteroid levels in functioning cases should be an immediate primary concern as overwhelming sepis seems to carry the day. 25
Bilateral Adrenal Hyperplasia In Multiple Endocrine Neoplasia Type 2A Gunasekaran R, Zanariah H Endocrinology Unit, Department of Medicine, Hospital Putrajaya Multiple Endocrine Neoplasia (MEN) 2A is an autosomal dominant disease, characterized by the development of Medullary Thyroid Carcinoma, Pheochromocytoma and hyperparathyroidism. Genetic studies have identified the presence of germline mutations in the RET proto-oncogene in almost 100 percent of MEN 2 patients. In MEN 2A, pheochromocytoma occurs in 40 to 50 percent of patients, usually arising in the adrenal glands and rarely occurs at extra-adrenal sites. In contrast to sporadic pheochromocytoma, the adrenal involvement within MEN 2A begins with adrenal medullary hyperplasia and is multicentric and bilateral in more than 50 percent of cases. Pheochromocytomas are almost always benign, but some tend to recur locally. These tumors usually produce adrenaline disproportionately to noradrenaline, in contrast to sporadic cases. Hypertension in MEN 2A patients with pheochromocytoma is more often paroxysmal than sustained, in contrast to the usual sporadic case. MEN 2A patients with pheochromocytomas may have paroxysmal palpitations, anxiety, headaches, or sweating but many remain asymptomatic. We describe a 52-year-old man who was referred to us for further evaluation and follow-up of his elevated urinary catecholamines and adrenal abnormalities on the CT scan. He was initially diagnosed to have medullary thyroid carcinoma with regional lymph node metastases. He underwent total thyroidectomy and neck dissection 9 years back and is currently on thyroxine replacement with persistent mild elevation of serum calcitonin levels in the absence of clinically discernible disease. Serum calcium levels have remained within normal range. Genetic testing had detected a mutation on codon 634. Regular screening of urinary catecholamines revealed progressive elevation above normal and most recently, urinary adrenaline levels were elevated five-fold without significant elevation of noradrenaline. Serial CT scan of the adrenals showed gradual progressive nodularity of both adrenals in keeping with probable adrenal medullary hyperplasia. An additional extra-adrenal lesion was identified adjacent to the left adrenal gland. Further investigation with I-131-metaiodobenzylguanidine (MIBG) scintigraphy is planned prior to considering the timing and extent of surgical approach for the adrenal disease. 26
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: A Case Of Persistent Parathyroid Ca
Page 31 and 32: Carney Complex: Cardiac Myxoma And
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

JAFES-Booklet (English - pdf - 1103 Kb) - MEMS

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