Index

A occult tumors in, 52-53 Abdomen, computed tomography of, 29f, 30f, 51 Adrenogenital syndrome, 38 Achlorhydria (WDHR) syndrome. See VIP-oma of Aldosterone, effects of, 36 the pancreas Aldosterone and primary aldosteronism (Conn's ACTH. See Adrenocorticotropic hormone syndrome),155-159 Addison's disease, 31, 133 aldosterone suppression tests, 157-158 Adrenal carcinoma, arteriography of, 43f determination of plasma aldosterone precursors, Adrenal cortex 158 aldosterone-producing tumors of, 36 determination of plasma and urinary aldosterone diseases of, 33-38 levels, 156 Adrenal cortical carcinomas, 42-43 determination of plasma activity or Adrenal glands, 28-32 concentration, 155-156 adrenal scintigraphy in, 28 determination of plasma/serum electrolytes, 155 close interaction between gonads and, 21 individual basal levels, 158f computed tomography of, 28-30, 32 postural stimulation test, 156-157 in Cushing's disease, 35f response to spironolactone trial, 159 in macro nodular hyperplasia, 35 twenty-four-hour urinary aldosterone excretion magnetic resonance imaging (MRI) in, 30-32 rates, 158f malignant tumors of, 42-43 Aldosteronism, treatment of, 38 single adenoma of, 50f Aldosteronoma, in Conn's syndrome, 37f ultrasonography in, 28, 32 Amine-precursor uptake and decarboxylation venous sampling in, 32 (APUD),75 Adrenal hemorrhage, 31 Androgen-producing adrenal neoplasias, 162-163 Adrenal hormone excess, 36-38 Androgen-producing tumors, 38 Adrenal hyperplasia, 34f Androgen-secreting ovarian tumors, 25t Adrenal imaging, indications for, 28 Angiotensin I, 119 Adrenal medulla, diseases of, 39-44 Angiotensin II, 119 Adrenal scientigraphy, 28 APUD. See Amine-precursor uptake and Adrenal veins, anatomical variations and anomalies decarboxylation of,5f APUD cells, 87, Adrenocorticotropic hormone (ACTH) APUD concept, 17-78 in catheterization of IPS, 64-68 APUDomas, 78 in Cushing's syndrome, 33-36 Ascending lumbar vein, Ilf measuring, 52 Asymptomatic hyperparathyroidism, 167 178 Index

B Computed tomography (CT) Basal metabolic rate (BMR), 41 of abdomen, 29f, 30f, 51 Basilar plexus, 56 in adrenal glands, 28-30, 32 B cells, 76 of cranium, 51 Beta cells (B cells), 76 in hyperandrogenemia, 4 Bilateral adrenal hyperplasia, 34f in hyperparathyroidism, 137 Bilateral adrenal metastases, 44f in islet cell localization, 90-91 Bilateral renal artery stenosis, 123 in pheochromocytomas, 40f Blind pancreaticoduodenectomy, 110 Congenital adrenal hyperplasia, 162 Breast cancer, 128 Conn's syndrome, 36-38 aldosteronoma of, 37f C Corticotropin releasing factor, 63 Calcitonin (thyrocalcitonin), 129 in elevated prolactin levels (PRL) , 67-68 Calcium, absorption of, 131 testing, in Conn's syndrome, 49-51 Calcium set point, 127 Corticotropin releasing hormone (CRH) stimulation Captopril, 121, 170 \ test, 165 Captopril challenge test, 121 Cortisol and Cushing's syndrome, 151-155 Catheterization "classic" dexamethasone suppression test accuracy of, 11 (Liddle's test), 154-155 clinical findings and peripheral androgen levels in determination of plasma/serum cortisol levels, 75women,3t 152-153 normal women and clinical results of, 14-17 determination of urinary free cortisol excretion in pancreatic venous sampling, 97-99 rate, 152 selective bilateral ovarian-adrenal individual serum cortisol levels, 153f, 154f catheterization, 24-25 overnight dexamethasone suppression test (DST) , stress factor and hormone release during 153-154 sampling, 13-14 Cortisol measurement, 49 Catheterization in hyperandrogenemia, 1-4 Cranial CT, 51 angiographic catheters in, 14f CRF. See Corticotropin releasing factor four-vessel blood sampling technique, 15f C-terminal fragments/assays, 134-135 hormone analysis, 14 Cushing's disease influence of contrast media on ho~mone release, bilateral adrenal hyperplasia in, 34f 12 causes of, 48 procedure, 8-10 CRFin, 63 side effects and complications of, 12-13 diagnostic problems in, 52-53 statistical analysis, 14 laboratory tests, 48-51 Catheterization in IPS, 58- 63 pituitary adenoma in, 50 access, 58-59 plasma ACTH levels in, 66f catheters, 58 plasma prolactin levels in, 65f, 66f contrast media, 58 problems with endocrinologic testing, 52-53 endocrinological background for, 63 problems with hormone measurements, 52 endocrinological results in, 64-66 problems with radiological procedures, 53 facts to keep in mind about, 66-68 radiological procedures, 51 patients and methods, 63-64 thrombosis in, 59 procedure, 59-62 Cushing's syndrome, 33-34, 43. See also Cortisol schematic drawing of sampling catheters entering and Cushing's syndrome inferior petrosal sinuses, 59f ,clinical workup, 34-36 side effects and complications of, 62-63 IPS catheterization in, 63-64 technical results, 64 plasma cortisol and ACTH levels in, 165f Cavernous sinuses, anatomy of, 54-56 serum cortisol and 11-deoxycortisol levels in, afiluents and efiluents of, 55-56 165f Cimetidine therapy, 109 Circumaortic venous ring, 12f D "Classic" dexamethasone suppression test (Liddle'S D cell, 76 test),154-155 Dehydroepiandrosterone sulfate (DHEA-S), 2 Index 179

Deoxycorticosterone acetate (DOCA) suppression H test, 157 Hepatic artery embolization, 112 Dexamethasone (DXM), in hyperandrogenemia, 2 Hepatic metastases, 82 Dexamethasone suppression test (DST), 2-4, Hepatic veins, 8t 154-155 High-dose dexamethasone tests, 49, 52 in Cushing's syndrome, 49 Hirsutism and virilization, 159-163 in idiopathic hirsutism, 161 androgen-producing adrenal and ovarian overnight test, 49, 153-154 neoplasias, 162-163 Diabetes-dermatitis syndrome, 82 causes of, 159 Diazoxide suppression test, 81 congenital adrenal hyperplasia (CAH), 162 Distal blind pancreatic resection, 110 idiopathic hirsutism, 160-161 Human pancreatic polypeptide (hPP), 76 E Humoral hypercalcemia of malignancy (HHM), Ectopic ACTH syndrome, 34, 50f, 164 134 CRFin,63 Hyperandrogenemia. See also Catheterization in dexamethasone suppressi\m test in, 49 hyperandrogenemia diagnosing, 52-53 anatomy and anatomical variations in, 4-8 Ectopic insulinomas, 109 causes of, 1 Ectopic ulcerogenic syndrome. See Gastrinoma clinical features, 2 Endocrine pancreatic tumor, 75 diagnostic methods for localization of androgen• schematic representation of, 79f producing tumors, 4t Endocrinologic testing, problems with, 52-53 diagnostic procedures, 2-4 Extraadrenal pheochromocytomas (para• effects of contrast media on adrenal steroid gangliomas), 39 release in, 16f Extraadrenal tumors, 39 endocrinologic results, 13-14 Extrapancreatic tumors, localizing, 96 estimation of secretion rates in, 1 indications, 4 F laboratory tests, 2-4 Familial hypocalciuric hypercalcemia, 126-127, 133 nontumorous, 17-21 Female hyperandrogenemia. See Hyperandrogenemia ovarian hyperthecosis, 22 Feminizing tumors, 38 physical examination in, 2 Functioning islet cell tumors, 75 polycystic ovary syndrome, 21-22 radiographic procedures for, 1 G standardized protocol for ovarian and adrenal Gastrin vein sampling, 4t and gastrinoma, 166-167 tumorous, 23-24 hormone assay, 167 Hypercalcemia Gastrinoma, 84-86, 166-167 causes of, 133t clinical features, 85 differential diagnosis of, 131-134 diagnosis, 85-86 effects of, 125 diagnostic workup, 85-86 familial hypocalciuric, 133 head/body/tail ratio in, 108-109 idiopathic hypocalciuria of infancy, 133 histologic and architectural data, 85 of malignancy, 127-128, 133 preoperative localization methods, 167 milk-alkali syndrome, 133 Gastrin secreting cells (G cells), 76 myeloma, 133 G cells, 76 other causes of, 133-134 Glucagon hormone assay, 167 primary hyperparathyroidism as cause of, Glucagonoma, 79, 82-83, 167 126-127 diagnostic workup, 82-83 secondary hypocalcemia and, 127-128 histologic and architectural data, 82 Hypercatecholaminism, 39-42 Glucose tolerance test, 81 Hypercortisolis,m, major clinical features of, 48 Gonadal neoplasms, 24f HypercortisolisI11us,33-36 Gonads clinical workup, 34-36 close interaction between adrenals and, 21 Cushing's disease, 33-34 radiation exposure to, 13 Cushing's syndrome, 33 180 Index

Hyperinsulinism anatomy, 54~58 author's experience in, 114-115 functional anatomical aspects, 56-58 preoperative localization of pancreatic lesions hormonal concentrations in, 53 causing, 114t location and description of, 56 symptoms of, 81 sampling, reasons for performing, 53 Hyperparathyroidism (HPT) Inferior phrenic veins, 5, 6f catheter for upper part of right jugular vein, 140f Insulin clinical features, 125 hormone assay, 166 computed tomography (CT) in, 137 and insulinomas, 165-166 diagram for parathyroid sampling, 14lf production of, 76 extremely dilated right jugular vein, 14lf Insulinomas, 80-82, 165-166. See also Pancreatic four different catheters for parathyroid sampling, insulinoma blind distal pancreatectomy for, 110 139f categories of, 80-81 gadolinium (Gd)-D PTA enhanced Tl-weighted characteristics of, 80 spin-echo (SE) sequences, 138 clinical diagnosis of, 109 introduction, 125 1 clinical presentation of, 80 invasive diagnostic procedures, 138-144 clinical workup of, 166 magnetic resonance imaging (MRI) in, 137-138 diagnostic workup of, 81-82 noninvasive imaging in, 135-138 head/body/tail ratio of, 109 roadmapping of venous sampling, 140-141 histologic and architectural data, 80-81 scintigraphy in, 137 preoperative localization methods, 166 signs and symptoms, 125-126 Intraarterial DSA, 142-143 thermography in, 138 Intraoperative ultrasound, 96-97 treatment, 144-145 Intravenous dexamethasone, in Polycystic ovaries, ultrasound in, 135-137 22,23f venous sampling, 139-140 IPS. See Inferior petrosal sinuses Hyperthecosis (HYP), 22 IPS catheterization. See Catheterization in IPS androgen metabolism in, 25t Islet cell hyperplasia, 81 Hypoglycemia, 78 Islet cell localization causes of, 80 arteriography in, 91-95 in 49-year-old man, 112f-113f computed tomography (CT) in, 90-91 symptoms of, 81 intraoperative ultrasound in, 96-97 12-year-old boy with, 105f Islet cell tumor embolization, 110-114 2-month-old boy with, 107f Islet cell tumor localization, 89-90 Hypoglycemic syndrome, 109-110 Islet cell tumors, 78-80 cure rate of, 110 calcification of, 91 Hypokalemia. See VIP-oma of the pancreas clinical and surgical considerations, 108-110 Hypothalamic-pituitary-adrenal axis, 50f groupings of, 79-80 Hypovolemia, in aldosteronism, 38 localization of, 88-97 Islet of Langerhans, 76 I normal distribution of, 77f Idiopathic hirsutism, 160-161 determination of plasma/serum androgen profile, L 160 Langerhans, Paul, 79 dexamethasone suppression test for plasma Laparotomy, 108, 110 androgens, 161 Left , 40 Incidentalomas,43-44 Left adrenal vein, 4-5, 6f Infants Left ovarian vein, 5-8, 9f, lOf idiopathic hypercalciuria in, 133 Leucine tolerance test, 81 with nesidioblastosis, 110 Liddle's test, 154-155 primary hyperparathyroidism in, 127 Lysine-vasopressin (8-L VP) stimulation test, 165 Inferior petrosal sinuses (IPS), 48-68 affiuents and effiuents of, 55-56 M anatomical relationships of cavernous and, 55f Macronodular hyperplasia, 34, 35f anatomical variations in, 56, 57f Magnetic resonance imaging (MRI) Index 181

of ACTH-secreting adenomas, 51 p for adrenal glands and adrenal tumors, 30-32 Pancreas in hyperandrogenemia, 4 endocrine tumors, 75 in hyperparathyroidism, 137-138 islet cell neoplasms in, 79 in pheochromocytoma, 40f Pancreatectomy, 109-110 Metaiodobenzylguanidine (MIBG), 42 Pancreatic angiography, 91-95 Metastatic pancreatic endocrine tumors, 112 Pancreatic cholera syndrome. See VIP-oma of the Metyrapone (metopirone) test, 164-165 pancreas Metyrozine,42 Pancreatic hormones, 165-167 Milk-alkali syndrome, 133 gastrin and gastrinoma, 166-167 Multiple adeonmas, false diagnosis of, 106 insulin and insulinomas, 165-166 Multiple endocrine adenomatosis type I (MEA-I), Pancreatic insulinoma 79,87,126 algorithmic approach for diagnosis and Multiple endocrine adenomatosis type II (MEA-II), localization of, 88t 87,126 benign, with calcification, 93f pheochromocytomas in)44 diffuse metastatic disease in liver due to, 95f Multiple endocrine adenopathy (MEA), 87 in 50-year-old woman, 89f Multiple endocrine neoplasia (MEN) syndrome, 39 malignant, liver metastatic disease of, 95f Multiple endocrine neoplasia type I (MEN-I), 126 in 9-year-old girl, 103f Multiple endocrine neoplasia type II (MEN-IIA), in 79-year-old woman, 92f 126 in tail of pancreas, 94f Myeloma, 133 in 31-year-old man, 104f in 29-year-old woman, 96f Pancreatic islet cell hyperplasia, 108 N Pancreatic islet cells, hyperplasia of, 87 Nesidioblastosis, 81, 87 Pancreatic islet cell tumors, 75 false diagnosis of, 106 Pancreatic islets infants with, 110 cell types in, 76 2-month-old boy with, 107f neuroendocrine cells from, 75 Neuroendocrine cells, from pancreatic islets, 75 Pancreaticoduodenectomy, 109 Neurofibromatosis (von Recklinghausen's disease), Pancreatic polypeptide (PP), 76 39 Pancreatic polypeptide secreting tumors (PP-omas), Nonfunctioning pancreatic islet cell tumo'r, 90f 84 Nontumorous hyperandrogenemia, 17-21 Pancreatic venous sampling, 75-115 elevated OPGs and APGs in, 20f anatomy of pancreatic venous drainage, 99f, elevated peripheral vein steroids in, 19f 100-102 incidence of ovarian and/or adrenal involvement anterior inferior pancreaticoduodenal (AIPD) in etiology of, 20-21, 2lt vein, 99f, 100, 104 peripheral and glandular vein levels of T and anterior superior pancreaticoduodenal (ASPD) DHEA-S (DS) in, 19f vein, 98, 99f, 100, lOlf, 104, 105 steroid gradients reflecting glandular secretion in, APUD concept, 77-78 16t,19-20 caudal pancreatic (CP) veins, 98, 99f N-terminal fragments/assays, 134-135 complications of procedure, 107-108 distribution of hormone-producing islet cells, o 76-77 Oat cell carcinoma, 34 dorsal pancreatic (DP) vein, 98, 99f, lOlf Oral clonidine, 42 gastrocolic trunk (GT), 100, lOlf, 104 Orthoendocrine tumors, 80 interpretation of hormone data, 102-107 Osteitis fibrosa cystica, 125-126 introduction, 75 Ovarian hormone diseases, 151-163 left gastric vein (LGV), lOlf, 102, 105 Ovarian hyperthecosis, 22 percutaneo~s transhepatic access for, 97f Ovarian veins, anatomical variations and anomalies percutaneous transhepatic portography with of, Sf catheter tip in splenic vein, 98f Overnight dexamethasone suppression test (DST), posterior inferior pancreaticoduodenal (PIPD) 153-154 vein, 99f, 100, 104 182 Index

Pancreatic venous sampling (cant.) Pituitary adenoma, 61 posterior superior pancreaticoduodenal (PSPD) Pituitary blood supply, 54f vein, 98, 99f, 100, 101, 104, 105 Pituitary gland microadenoma, 34 sampling technique, 99-102 Pituitary hormones, 164-165 technique of catheterization and blood sampling, Pituitary hypercorticolism, 34 97-99 Pituitary microadenomas, 64f transverse pancreatic (TP) vein, 98, 99, 102f, 105 PlasmaACTH Paraendocrine tumors, 80 in Cushing's disease, 52 Paragangliomas, 39 determination of levels of, 164 Parathyroid anatomy and physiology, 128-135 Plasma aldosterone, measuring, 38 autotransplanted parathyroid tissue, 131 Plasma aldosterone precursors, 158 bilateral venography of superior vena cava, 132f Plasma renin activity (PRA) , 170 differential diagnosis of hypercalcemia, 131-134 in primary aldosteronism, 155-156 inferior trunc, 130f Plasma/serum cortisol levels, 152-153 laboratory findings, 130-131 Plasma/serum electrolytes, in primary mediastinal veins, 136f aldosteronism, 155 normal parathyroid glands, 143 Polycystic ovaries (PCO), 25t retrograde filling of vena thyroid veins, 134f Polycystic ovary syndrome, 21-22 right superior and middle thyroidal veins, 135f Polypeptide hormones, 76 thoracic veins, anterior-posterior position, 132f Poly tomography of sella, 51 venous drainage, 129-130 Postural stimulation test, 156-157 Parathyroid glands, location of, 128f Primary adrenal hyperplasia, 155 Parathyroid hormone (PTH), 167-169 Primary hyperparathyroidism, 126-127 in Ca absorption, 131 clinical workup, 168 hormone assay, 168-169 and parathyroid hormone, 167-168 laboratory techniques, 135 preoperativelocalization methods, 168 laboratory tests, 134-135 Primary reninism (renin secreting Ctumors), main actions of, 129, 131 169-170 secretion of, 129 PTRA. See Percutaneous transluminal renal Parathyroid hormone (PTH) excess. See Hyper• angioplasty parathyroidism Parathyroid venous sampling R complications, 143-144 Renal angiography, 169 intraarterial DSA, 142-143 Renal artery stenosis, arteriographic findings in, results of, 142 123t roadmapping of, 140-141 Renal hormones, 169-170 technical difficulties of sampling, 141-142 Renin, 36-38, 119 technique, 138-140 hormone assay, 170 Peptic ulcers, 166 normal metabolism of, 119-120 Percutaneous transluminal renal angioplasty in pathological states, 120 (PTRA), 120, 122 Renovascular hypertension, 119-124, 169 renal vein after, 123f, 124f arteriography in, 122 Peripheral compound F, 13 bilateral disease, 123 Pheochromocytoma, 39-42 causes of failure to identify, 123 arteriography of, 40f clinical findings in patients with, 12lt clinical workup, 39-42 detection of, 120-121 computed tomography of, 40 evaluation of, 121 diagnosing, 41 hypothetical relationship among body sodium invasive imaging of, 42 balance, blood pressure, and renin, 120f magnetic resonance imaging (MRI) in, 31, 40f incorrect sampling of renal vein renin, 123 in multiple endocrine adenomatosis type II, 144 intravenous digital subtraction angiography noninvasive imaging, 42 (IVDSA) in, 122 treating, 41-42 introduction, 119 Phlebography, 32 laboratory findings in patients with, 121t Index 183

normal renin metabolism, 119-120 Tolbutamide tolerance test, 81 renal vein renin (RVR) sampling and assay and, Transcatheter therapy, 144 122 Tumor blush, 91 renin in pathologic states, 120 Tumorous hyperandrogenemia, 23-24 Retrograde venography, 1 in hyperandrogenemia, 10 U parenchyma of right adrenal gland after, 17f Ulcerogenic syndrome, 75 Right adrenal gland Ultrasonography/ultrasound aldosteronoma of, 37f in adrenal glands, 28, 32 arteriography of pheochromocytoma of, 40f complications from, in hyperparathyroidism, 136 retrograde phlebography of, 33f in hyperandrogenemia, 4 Right adrenal vein, 5, 6f in hyperparathyroidism, 135-137 differentiation between hepatic veins and, 8t intraoperative, in hyperparathyroidism, 135-136 venouscontributionsto,8f in islet cell localization, 89-90 Right hepatic veins, 7f \ results of, in hyperparathyroidism, 137 Right ovarian vein, 8, 13f Ultrasound-guided fine needle aspiration, 136 draining into right renal vein, 14f Ultrasound-guided percutaneous treatment of parathyroid tumors, 144-145 S Urinary free cortisol excretion rate, 152 Saline infusion test, 157-158 Urinary 17-hydroxycorticosteroid, 52 Sarcoidosis, 133 Scintigraphy V in hyperandrogenemia, 4 Vasoactive intestinal peptide (VIP), 76 in hyperparathyroidism, 137 Venous plexuses, 56 Secondary aldosteronism, 38 Verner-Morrison syndrome, 84. See also VIP-oma Secondary hyperparathyroidism, 127-128 of the pancreas Selective bilateral catherization, 32 clinical features of, 86-87 Selective bilateral ovarian-adrenal catheterization, clinical presentation of, 86 24-25 in 48-year-old woman, 1Hf Sipple's syndrome, 39, 87,126 VIP-oma of the pancreas, 86-87 Skull films, 51 diagnostic workup, 86-87 Somatostatin, 83 histologic and architectural data, 86 Somatostatinoma, 83 Virilization. See Hirsutism and virilization Spironolactone, 36 Vitamin D intoxication, 133 Superior ophthalmic vein, 55 von Hippel-Lindau disease, 39 Superior petrosal sinus, location and description of, von Recklinghausen's disease, 39 56 Sylvian veins, 56 W Syndrome of familial multiple endocrine Watery diarrhea. See VIP-oma of the pancreas adenomatosis-type II (Sipple's syndrome), Werner's syndrome, 87,126 39,87,126 Whipple's triad, 109 Williams syndrome, 133 T Technetium-thallium scintigraphy (TIS), 137 Z Tertiary hyperparathyroidism, 127 Zollinger-Ellison syndrome, 78-79,108-109. See Thermography, in hyperparathyroidism, 138 also Gastrinoma Thyroid bed, major veins of, 129f clinical workup, 167 Thyrotoxicosis, 133 patient with, 106f