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This Concept Map, created with IHMC CmapTools, has information related to: Adrenal, Parathyroid, Adrenal Medulla PARAGANGLIOMAS Pheos outside of adrenal -mostly in TEENS, twenties, M=F -can also refer to only non-fcnal -anywhere there paragang tissue -Carotid body, jugulotympanic (chemodectomas) -more often multicentric -10% mets->death -10-40% malignant-more than ad MORPH-zellballen -all like pheo -more anaplastic->disseminate, Parathyroid Primary Hyperparathyroidism -often caught on routine labs Manifestations: -hypercalcemia: -seizures -bone erosion ('clasts) ->osteoporosis -trabecular ('blasts) ->osteitis fibrosa cystica -brown tumors (clasts, giant cells, hemorr debris) -osteitis fibrosa cystica -UT stones, calcinosis -metastatic calcification in: stomach, lungs, myocard -hypophosphatemia -GI disturbances-N/constip, gallstones, ulcers, panc'itis -Neuromusc-weakness, fatigue -Valve, vessel calcifications, MULTIPLE ENDOCRINE NEOPLASIA SYNDROMES (MENs) MEN I WERMER SYNDROME-3 Ps -11q13 muts in most fams Parathyroids-hyperPTism-most common -by 40s-50s -also adenomas Pancreas-islet cell tumors->ins, glucagon, gastrin, VIP, somatostatin -usually multiple Pituitary Glands-esp PRLoma -less prominent than others Duodenum-gastrinoma Carcinoid tumors Thyroid, AC CA CLINICAL-recurrent HYPOglycemia-ins'omas -recurrent peptic ulcers-gastrin- Zollinger-Ellison syn, Parathyroid SECONDARY HYPERPARATHYROIDISM Any condition->low Ca++ ->increased gland activity RENAL FAILURE-most common -dec'd PO4 excretion ->hyperPO4-emia->depress Ca Vit D def Inadequate Ca intake Renal Tubule Acidosis Fanconi's Syndrome MORPH-hyperplastic glands ->bone changes, metc. cal'tion -less severe than primary CLINICAL-dominated by renal -vasc involvement->ischemia =calciphylaxis Tertiary hyperPTism -activity becomes autonomous, excessive, MULTIPLE ENDOCRINE NEOPLASIA SYNDROMES (MENs) MEN II MEN IIB=MEN III -clinically like IIA -BUT W/ NEUROMAS, GANGLIONEUROMAS -skin, oral muc, resp tract, eyes, GI tract -BUT diff mut in RET -single AA change -> ALL MEN IIB, Adrenal Medulla PHEOCHROMOCYTOMA RULE OF 10s 10% in familial syndrome 10% extra-adrenal 10% of nonfam are BILATERAL 10% malignant 10% arise in childhood, MULTIPLE ENDOCRINE NEOPLASIA SYNDROMES (MENs) MEN II MEN IIA - SIPPLE SYNDROME Pheochromocytoma-40-50% -often bilateral, can be exra-adrenal Medullary CA-almost 100% -multifocal, C cell hyperplasia ->calcitonin, aggressive! Parathyroid hyperplasia-10-20% ->hyperCa+, stones Muts of RET protooncogene -Chrom 10 ->constitutively active Tyr Kinase rec. -fam screening->THYROIDECTOMY, Pineal Gland Pinealomas -most from emb GERM CELLS PINEOCYTOMAS -adults, slower growing -compress surrounding structures -don't invade -stain for GFAP if astrocytomas (glial) -retinal S-antigen if retinal diff'n -pineocytes - neuron-specific enolase -staining -PSEUDOROSETTES rimmed by rows of pineocytes-eo'philic cytoplasm -in lobular masses CLINICAL-7 yr course ->HA, visual defects, mental deterioration, dementia-like -excision difficult, MULTIPLE ENDOCRINE NEOPLASIA SYNDROMES (MENs) MEN II FAMILIAL MEDULLARY THYROID CA -variant of MEN IIA- RET mutation -BUT not other Sx, just med CA ~25% of med CA familial, Pineal Gland Pinealomas -most from emb GERM CELLS PINEOBLASTOMAS -young ppl ងyo -invade hypothal, midbrain, 3rd vent -large hyperchromatic nuclei, frequent mitoses -look like small blue cell-primitive embryonal tumor -poorly-formed rosettes of cells -spread via CSF, can compress aqueduct (Sylvius) ->hydrocephalus->death w/in 2 yrs -look and spread like medullablastoma, Parathyroid Primary Hyperparathyroidism -often caught on routine labs ADENOMA-75-80% ᡪs WOMEN -h/o irradiation 30-40 yr ago Sporadic: PRAD 1 -> cyclin D1 -chrom 11 inversion -10-20% of sporadic Familial: MEN 1 - lose supp gene on 11q13 -most of fam, some spor MEN2A - RET oncogene Fam Hypocalciuric hyperCa+ -CASR mutations MORPH-high Ca++->sequelae -little adiposity, usually solitary -chief cells predominate, Parathyroid Hypoparathyroidism CLINICAL -TETANY-irritability -tingling, spasm, seizures -Chvostek sign, Trousseau -emotional instability, psychosis, hallucinations -ocular dz - Ca++ of lens->cataracts -CV-conduction defect-prolong QT int -dental-if early in development, ADENOMA-75-80% ᡪs WOMEN -h/o irradiation 30-40 yr ago Sporadic: PRAD 1 -> cyclin D1 -chrom 11 inversion -10-20% of sporadic Familial: MEN 1 - lose supp gene on 11q13 -most of fam, some spor MEN2A - RET oncogene Fam Hypocalciuric hyperCa+ -CASR mutations MORPH-high Ca++->sequelae -little adiposity, usually solitary -chief cells predominate Monoclonal cells PRIMARY HYPERPLASIA-10-15% -diffuse or nodular MORPH- spor, or in MEN-I or IIA -usually all 4 glands -little fat -hyperplasia->diffuse, Parathyroid Primary Hyperparathyroidism -often caught on routine labs Nonparathyroid Dzs -PTHrP secretion by tumors -PTH levels low, Ca high -nonmetastatic tumors -poor prognosis -Osteolytic mets-cytokines released->TNF-a, IL-1, 6 ->'clast matures, 'blasts -most common clinically-sig hyperCa++, Adrenal Medulla NEUROBLASTOMA MOST COMMON extracranial solid tumor of childhood -originates in SNS or ad medulla -mostly sporadic, some familial, Parathyroid Primary Hyperparathyroidism -often caught on routine labs CARCINOMA -sim to adenoma -nodular or trabecular cords Dx-based on INVASION, METS only, Parathyroid Hypoparathyroidism Less common than hyper Major Causes: -Surgical removal (w/ thyroid) -Congenital absence -Primary (idiopathic atrophy) -auto-AB to Ca-sensing receptor -prevents PTH release -Familial- w/ chronic mucocutaneous candidiasis, primary adrenal insuff -presents as kid w/ candida ->then hypopara'ism, then ad, Adrenal Medulla PHEOCHROMOCYTOMA Chromaffin cells ->catecholamines, peptides ->surgically-treatable HTN 85% in ad med, some extra-ad -more malignancy in extra-ad 10% familial (AD) - MEN, NF-I (von Recklignhausan's), VHL, Sturge-Weber syndromes -YOUNG MALES -mostly bilateral 90% sporadic - 40-60yo FEMALES -mostly unilateral MORPH-large w/ hemorr, nec, cyst -efface cortex - encapsulated -ZELLBALLEN-spindly chromaffin -rare mitotic figures-doen's mean malig. -malig determined by METS: -LNs, liver, lung, bone, Parathyroid Primary Hyperparathyroidism -often caught on routine labs PRIMARY HYPERPLASIA-10-15% -diffuse or nodular MORPH- spor, or in MEN-I or IIA -usually all 4 glands -little fat -hyperplasia->diffuse, Adrenal Medulla PHEOCHROMOCYTOMA CLINICAL-HYPERTENSION!!-abrupt -tachycardia, palps, HA, swelling, tremor, apprehension -can be paroxysmal in ə/2 -can->CHF, pulm edema, MI, VFib, CVAs -"catechol cardiomyopathy" -dmg due to constriction of aa -can release ACTH, somatostatin Labs - metanephrine, VML Tx-excision after adrenergic blockers