Adrenal Tumors PDF

Adrenal Tümörler Adrenal Tumors Istinye University, School of Medicine  Cortex Zona Glomerulosa: Aldosterone Zona Fasiculata: Cortisol Zona Reticularis: DHEA ve androstenodione  Medulla Chromaffin cells: Epinephrine...

Adrenal Tümörler Adrenal Tumors Istinye University, School of Medicine  Cortex Zona Glomerulosa: Aldosterone Zona Fasiculata: Cortisol Zona Reticularis: DHEA ve androstenodione  Medulla Chromaffin cells: Epinephrine and Norepinephrine  Adrenocortical Tumors Of adults 50%, of children 90% hormonal active The clinic occurs according to the hormone it secretes. Salgıladığı hormona göre klinik oluşur Cushing Conn’s Virilism or ada feminization  Cushing’s Incidence 5 in million and F/M: 9 1 out of 5 origin is adrenal gland Unresponsive to dexamethason suppression test, in case of low ACTH level 95% adrenal origin  Conn No significant phenotypic appearance 70% adenoma, 30% hyperplasia, 1% malignancy Unresponsive Hypertension Hipokalemia, hipernatremia Plazma aldosterone (ng/dL)/ renin activity (ng/mL/s) > 20 Aldosterone level >15ng/dL Adrenal Medulla Tumors  Pheochromacytoma 90% are adrenal origin, 10% extra adrenal location (aorta- symphatetic chain) Unresponsive hypertension is the main presenting sign, headache, fever, palpitation, sweating, nausea, pollakuria and weight loss  Pheo’s 10 percent rules 10 % familial 10 % malignant 10 % extraadrenal origin 10% in children 10% bilateral, multicentric  Pheochromacytoma Pheo can be a compenent of some syndromes Type 2 multiple endocrine neoplasia (MEN-2) and von Hippel- Lindau syndrome, rarely type 1 neuorofibromatosis MEN-2, autosomal dominant disease caused by a mutation in RET proto-oncogene in 10th chromosome In MEN-2, medullary thyroid cancer and bilateral benign pheo in 50% probability A mutation in vHL gene of 3rd chromosome causes VHL syndrome resulting in retinal angioma, hemangioblastoma in central nervous system, renal cyst and tumor, pancreatic cysts  Imaging Contrast enhanced CT scan and MRI If any doubt, for further diagnostic sensitivity MIBG (Metaiodobenzylguanidine scintigraphy) scan  Management Surgery is the definitive treatment Perioperative mortality is around 10% - Blood pressure monitorization and controlling most important parameter Alpha blockers premedication  Persistent Hypertension Persistent hypertension after surgical treatment warrants a research for other origins than adrenal gland Metastatic regions if the pathological result is malignant  Adrenal venous sampling Bilateral adenoma, right or left? Choosing the dominant side In all hormonally active adrenal diseases  Adrenal Myelipoma Autopsy incidence 0.08-0.4% MRI –fat density Hormonally inactive The size matters and if it is (>6cm), then the probability of sarcoma component increases and surgery needed  Management Two approach for adrenal gland tumors Clinical approach (Cushing, Conn etc) and surgical management If adrenal gland mass is visualized insidentally, then the nature is evaluated Management Adrenal Mass Endocrinological evaluation: Hormonall y Active Yes Pre-op medication and No surgery Malignancy suspicion Yes No Pre-op staging and surgery Size> 4cm No Yes Surgery Takip  Adrenalectomy Open Laparoscopy Robotic  Adenomectomy Selected cases (no suspicion of malignancy ) Smaller than 2-2,5 cm Previous contralateral adrenalectomy (VHL)