(BQ) Part 1 book NMS surgery presentation of content: Principles of surgical physiology, essentials of general surgery, medical risk factors in surgical patients, principles of thoracic surgery, arterial disease, venous and lymphatic disease, stomach and duodenum, pancreas,...
NMS Na tio na l Me d ic a l Se rie s fo r Ind e p e nd e nt Stud y Surgery NMS Na tio na l Me d ic a l Se rie s fo r Ind e p e nd e nt Stud y Surgery Sixth Ed itio n Bruce E Jarrell, MD Professor Department of Surgery University of Maryland School of Medicine Baltimore, Maryland Stephen M Kavic, MD Associate Professor Department of Surgery Program Director Residency in General Surgery University of Maryland School of Medicine Baltimore, Maryland Acquisitions Editor: ari Broderick Product Development Editor: Amy Weintraub Editorial Assistant: Joshua Haf ner Marketing Manager: Joy Fisher-Williams Production Project Manager: Priscilla Crater Design Coordinator: erry Mallon Manufacturing Coordinator: Margie Orzech Prepress Vendor: Absolute Service, Inc Sixth Edition Copyright © 2016 Wolters Kluwer Copyright © 2008, © 2000, Lippincott Williams & Wilkins, a Wolters Kluwer business All rights reserved T is book is protected by copyright No part o this book may be reproduced or transmitted in any orm or by any means, including as photocopies or scanned-in or other electronic copies, or utilized by any in ormation storage and retrieval system without written permission rom the copyright owner, except or brie quotations embodied in critical articles and reviews Materials appearing in this book prepared by individuals as part o their o cial duties as U.S government employees are not covered by the abovementioned copyright o request permission, please contact Wolters Kluwer at wo Commerce Square, 2001 Market Street, Philadelphia, PA 19103, via email at permissions@lww.com, or via our website at lww.com (products and services) 987654321 Printed in China Library of Congress Cataloging-in-Publication Data NMS surgery / [edited by] Bruce E Jarrell, Stephen M Kavic — Sixth edition p ; cm — (National medical series or independent study) National medical series surgery Includes index ISBN 978-1-60831-584-0 I Jarrell, Bruce E., editor II Kavic, Stephen M (Stephen Michael), editor III itle: National medical series surgery IV Series: National medical series or independent study [DNLM: Surgical Procedures, Operative—Examination Questions Surgical Procedures, Operative—Outlines General Surgery— methods—Examination Questions General Surgery—methods—Outlines WO 18.2] RD37.2 617.0076—dc23 2015010501 T is work is provided “as is,” and the publisher disclaims any and all warranties, express or implied, including any warranties as to accuracy, comprehensiveness, or currency o the content o this work T is work is no substitute or individual patient assessment based on healthcare pro essionals’ examination o each patient and consideration o , among other things, age, weight, gender, current or prior medical conditions, medication history, laboratory data, and other actors unique to the patient T e publisher does not provide medical advice or guidance and this work is merely a re erence tool Healthcare pro essionals, and not the publisher, are solely responsible or the use o this work including all medical judgments and or any resulting diagnosis and treatments Given continuous, rapid advances in medical science and health in ormation, independent pro essional veri cation o medical diagnoses, indications, appropriate pharmaceutical selections and dosages, and treatment options should be made and healthcare pro essionals should consult a variety o sources When prescribing medication, healthcare pro essionals are advised to consult the product in ormation sheet (the manu acturer’s package insert) accompanying each drug to veri y, among other things, conditions o use, warnings, and side ef ects and identi y any changes in dosage schedule or contraindications, particularly i the medication to be administered is new, in requently used, or has a narrow therapeutic range o the maximum extent permitted under applicable law, no responsibility is assumed by the publisher or any injury and/or damage to persons or property, as a matter o products liability, negligence law or otherwise, or rom any re erence to or use by any person o this work LWW.com We b o th tha nk the m a ny m e nto rs who ve a d vis e d us e a c h thro ug ho ut o ur c a re e rs We a re fo re ve r ind e b te d to the m I wis h to tha nk m y wife , Le s lie , a nd m y wo nd e rful c hild re n fo r a ll o f the ir s up p o rt d uring m y c a re e r, a nd fo r the ir und e rs ta nd ing d uring the writing o f the m a ny e d itio ns o f NMS Surg e ry – BEJ De d ic a te d to m y lo ving wife J e nnife r a nd to m y lo ve ly d a ug hte r Em ily – SMK Foreword It is with tremendous pride that I introduce the sixth edition o NMS Surgery T is work has occupied a central role in the education o a generation o medical students T e outline ormat makes it a superb re erence or those learning the basics o surgery and a thorough review or those who already practice our art T e current edition has special signi cance to the University o Maryland School o Medicine Dr Jarrell is the Chie Academic and Research O cer and Dean o the Graduate School He also was my predecessor as Chair o the Department o Surgery Dr Kavic is the current program director o our surgery residency T e chapter authors and contributors include a virtual directory o our trainees and aculty It is a privilege to be associated with these esteemed educators I am continually impressed by the talents o the surgeons and surgeons-in-training at the University o Maryland T at quality is re ected in the ollowing chapters I know that you will enjoy this edition as much as I take pride in it Ste p h e n T Ba rtle tt, MD P e te r An g e los Dis tin g u is h e d P rofe s s or of Su rg e ry Ch a ir De p a rtm e n t of Su rg e ry Su rg e on -in -Ch ie f Un ive rs ity of Ma ryla n d Me d ic a l Sys te m Un ive rs ity of Ma ryla n d Sc h ool of Me d ic in e Ba ltim ore , Ma ryla n d vi Pre ace Welcome to the sixth edition o NMS Surgery T is book aims to build on the legacy o the previous ve editions We have retained much o the organization and ormat rom the last versions At the same time, we have strived to make this volume more readable It is an increasing challenge to limit content in the ace o rapidly expanding surgical knowledge As in previous editions, the text is not meant to be all-inclusive but rather serves as an introduction or the student o surgery All o the chapters have been thoroughly reviewed, rewritten, and updated to re ect the current state o the art in surgery T ere are dramatic dif erences in the ormat o this volume Perhaps most importantly, each section now begins with “Chapter Cuts and Caveats,” which are some o the most important principles worthy o the reader’s attention Within each chapter, we have added “Quick Cuts,” which are highlights that have been brought out separately rom the text In addition, we have added a new section at the end, “Grade A Cuts,” which are pairings that highlight associations in surgical thinking For the tremendous work put into this edition, we are indebted to the authors T eir high-quality and requently punctual contributions have made our jobs as editors pleasant We are also grate ul to the editorial team at Wolters Kluwer or their guidance and support throughout the process T e sixth edition o NMS Surgery is written primarily or students and residents in general surgery, but practicing surgeons as well as physicians in other specialties will no doubt nd it a use ul re erence We hope that all readers will nd that the book represents a declaration o the state o surgical art in 2015 —Bru c e E J a rre ll, MD —Ste p h e n M Ka vic , MD vii Contributors Willia m R Ale x, MD, FACS Cardiothoracic Surgery Riverside, Cali ornia H Ric rd Ale xa nd e r, MD, FACS Pro essor o Surgery University o Maryland School o Medicine Baltimore, Maryland And re a C Ba ffo rd , MD, FACS Assistant Pro essor o Surgery University o Maryland School o Medicine Baltimore, Maryland Em ily Be lla va nc e , MD, FACS Assistant Pro essor o Surgery University o Maryland School o Medicine Baltimore, Maryland Hug o Bo na tti, MD, FACS General and Minimally Invasive Surgery Easton, Maryland Che rif Bo utro s , MB, CHB, MSc , FACS Associate Pro essor o Surgery University o Maryland School o Medicine Baltimore, Maryland J o na tha n Bro m b e rg , MB, P hD, FACS Pro essor o Surgery Chie Division o ransplantation University o Maryland School o Medicine Baltimore, Maryland Bra nd o n Bruns , MD, FACS Assistant Pro essor o Surgery University o Maryland School o Medicine Baltimore, Maryland La ura S Buc na n, MD, FACS Assistant Pro essor o Surgery University o Maryland School o Medicine Baltimore, Maryland viii Whitne y Burro ws , MD, FACS Assistant Pro essor o Surgery University o Maryland School o Medicine Baltimore, Maryland Clint D Ca p p ie llo , MD Resident in Surgery University o Maryland Medical Center Baltimore, Maryland Ke nne th M Cra nd a ll, MD Resident in Neurosurgery University o Maryland Medical Center Baltimore, Maryland Ro b e rt S Cra wfo rd , MD, FACS Assistant Pro essor o Surgery University o Maryland School o Medicine Baltimore, Maryland P e te r E Da rwin, MD Pro essor o Medicine University o Maryland School o Medicine Baltimore, Maryland J o s e J Dia z, MD, FACS Pro essor o Surgery Chie Division o Acute Care Surgery University o Maryland School o Medicine Baltimore, Maryland Ga rim a Do s i, MD Fellow in Vascular Surgery University o Maryland Medical Center Baltimore, Maryland Ric rd N Ed ie , MD, FACS Cardiothoracic Surgery Philadelphia, Pennsylvania Ste ve n Fe ig e nb e rg , MD Pro essor o Radiation Oncology University o Maryland School o Medicine Baltimore, Maryland Chapter 17 T yroid, P r thyroid, Adren l Gl nds, nd T y us 281 Tyrosine Tyros ine hydroxyla s e Dopa Dopa de ca rboxyla s e Mona mine oxida s e , ca te chol-O-me thyltra ns fe s e Homovanillic acid—HVA Dopamine Dopa mine -βhydroxyla s e Norepinephrine P he nyle tha nola mine N-me thyltra ns fe s e Mona mine oxida s e , ca te chol-O -me thyltra ns fe s e Vanillylmandelic acid—VMA Epinephrine Fig ure 17-5: Schematic or catecholamine production HVA, homovanillic acid (From Philip PA, David PG Principles and Practice of Pediatric Oncology, 6th ed Baltimore: Lippincott Williams & Wilkins ; 2010.) Ad re no c o rtic a l Ins u f c ie nc y (Ad d is o n Dis e a s e ) I P rim a ry d is e a s e : results in di inished or bsent unction o the dren l cortex c used by utoi une dise se, bil ter l dren l tuberculosis, dren l ung l in ections, or bil ter l dren l he orrh ge (second ry to eningococc l septice i , postp rtu , or in p tients on ntico gul nt ther py) II Se c o nd a ry d is e a s e : trophy o the dren l cortex second ry to decre sed pituit ry AC H (Cushing syndro e) c used by AC H suppression by corticosteroid drugs or pri ry pituit ry p thology (Cushing dise se) A Clinical presentation: Sy pto s include norexi , l ise, weight loss, poor toler nce o stress, hypoglyce i , hypotension, nd occ sion lly, skin hyperpig ent tion B Aldosterone de ciency: results in volu e depletion, hypon tre i , hyperk le i , zote i , nd cidosis C Perioperative steroid replacement: H ndled on n individu l b sis nd depends on the length o steroid ther py, the dos ge t ken, nd the gnitude o the pl nned procedure In gener l, the t rget is 100–150 g o hydrocortisone IV d ily or 2–3 d ys; preoper tive or l dos ge is resu ed er d ys Hyp e rc o rtis o lis m Quic k Cut Patients with Addis on dis eas e cannot tolerate the s tres s o s urgery without receiving corticos teroid s upport Quic k Cut Cus hing s yndrome is mos t commonly caus ed by s uppres s ion o the cortex s econdary to exogenous corticos teroids Quic k Cut A patient who has taken s teroids regularly or any period during the pas t year is as s umed to have inadequate adrenal res erve I Cus hing s ynd ro m e : results ro chronic lly incre sed cortisol levels A AC H dependent Pituitary source (Cushing disease): ccounts or 70% o the c ses o endogenous Cushing syndro e nd is ore co on in iddle- ged wo en Overproduction o AC H by the pituit ry results in bil ter l dren l hyperpl si ; ost co e ro pituit ry deno Ectopic Cushing syndrome: ccounts or 15% o the c ses nd is ore co on in older en a Cause: AC H is produced by n extr - dren l, extr pituit ry neopl s , ost co only s ll cell c rcino o the lung but c n lso occur with bronchi l c rcinoids, thy o s, nd p ncre s or liver tu ors b AC H level: quite high ( 500 ng/ L) B AC H independent: Adren l Cushing syndro e ccounts or 15% o the c ses Cause: Excess cortisol produced utono ously by the dren l cortex due to n deno , c rcino , or bil ter l nodul r dyspl si nd ectopic cortisol-producing tu ors; re ining 282 Chapter 17 Adren l Gl nd Ta b le 17-4: Ad re na l Ca us e s o Hyp e rte ns io n Na m e (s ) Ho rm o ne (s ) Ad re na l So urc e Dia g no s tic Te s t(s ) Aldosteronoma (Conn syndrome) Aldosterone Cortex: zona glomerulosa Aldo/PRA Aldo suppression AVS Cortisol-producing adenoma (Cushing syndrome) Cortisol Cortex: zona asciculata mg dexamethasone suppression test ACTH Pheochromocytoma Epinephrine Norepinephrine Medulla PFMs 24-hr catecholamines Aldo, serum aldosterone; PRA, plasma renin activity; aldo suppression, aldosterone response to salt loading; AVS, adrenal vein sampling or aldosterone/cortisol; ACTH, adrenocorticotropic hormone; PFMs, plasma ractionated metanephrines drenocortic l tissue trophies, nd AC H levels re low bec use o suppression by the excess cortisol Clinical presentation: Most co on ni est tions re listed in ble 17-4 Diagnosis: Nor l diurnal rhythm o cortisol secretion is usu lly lost Cushing syndro e is st te o cortisol excess Quic k Cut a Best test or hypercortisolism: elev ted ( g/ No one tes t is dL) orning s ple er suppressing dose o g conclus ive or Cus hing dex eth sone the night be ore s yndrome: Laboratory tes t res ults and clinical b Alternative test: Me sure s liv ry cortisol t idnight, pres entation mus t be which is nor lly less th n 1.6 ng/ L cons idered together to make c Elevated ( 55 g/day) 24-hour urinary ree cortisol: an accurate diagnos is reli ble index o hypercortisolis due to the incre sed ren l cle r nce o un et bolized cortisol Pathogenesis: Pl s AC H level gives good indic tion i Cushing syndro e is AC Hdependent or independent a Extremely low values: ound with dren l Cushing syndro e due to the suppressive e ects o cortisol b Very high levels: occur with ectopic Cushing syndro e due to the utono ous AC H production c Normal values: Present in 50% o pituit ry Cushing syndro e d Intermediate range: Di erenti ting ectopic ro pituit ry Cushing syndro e c n be di cult (1) High-dose dexamethasone suppression test: P tient is given dex eth sone, g/d y or d ys, nd the urine is collected or e sure ent o 17-hydroxycorticosteroids In pituit ry dise se, the 17-hydroxycorticosteroid levels will usu lly decre se to less th n 50% o nor l, where s they will show no suppression in the ectopic syndro e (2) Also help ul: Corticotropin-rele sing hor one (CRH) sti ul tion, jugul r nd peripher l AC H levels, nd pl s lipotrophic hor one y lso be help ul Localization o pituitary and ectopic tumors: MRI nd C re the best tests to identi y s ll pituit ry deno s; chest f l usu lly shows n ectopic neopl s ; however, C or MRI y be necess ry Localization o adrenal tumors: C or MRI c n correctly identi y gre ter th n 90%, including deno s less th n c in di eter, c rcino s, nd bil ter l hyperpl si (1) Radioisotope scanning: R diocholesterol n logue NP-59 (iodo ethylnorcholesterol) c n loc lize unctioning drenocortic l tu ors T is test is r rely used (2) Adrenal vein sampling or cortisol: c n lso loc lize the source o cortisol production Curative therapy or pituitary Cushing syndrome a rans-sphenoidal resection o the tumor: procedure o choice b Pituitary irradiation rom an external source: H s been e ective in up to 80% o children However, the cure r te is only bout 15%–20% or dults Chapter 17 T yroid, P r thyroid, Adren l Gl nds, nd T y us 283 c Bilateral total adrenalectomy: now reserved or c ses in which no pituit ry deno is ound, r di tion h s iled, or when the p tient c nnot toler te the prolonged r di tion process (1) Advantage: i edi te nd co plete control o the Quic k Cut cushingoid st te Bilateral (2) Disadvantages: Incre sed orbidity nd ort lity In adrenalectomy produces a permanent Addis onian s tate 15% o the c ses, n AC H-secreting pituit ry tu or develops (Nelson syndrome) Ectopic Cushing syndrome treatment: Directed to the underlying neopl s Re ov l o the tu or is cur tive; however, bec use o the di use n ture o s ll cell lung c ncers, o en only p lli tive ther py c n be o ered Adrenal Cushing syndrome treatment: tot l dren lecto y Quic k Cut vi l p roscopic or open techniques Laparos copic a Approach: tr ns bdo in lly or retroperitone lly vi the adrenalectomy has become nk or b ck the pre erred approach or b Large or aggressive lesions: Open dren lecto y vi adrenal les ions les s than cm n nterior or nk ppro ch is dvis ble or signif c nt in s ize heterogeneity, nod l involve ent, nd loc l so tissue or v scul r inv sion c Partial resection: I ll o the lign nt tissue c nnot be re oved, p lli tive ther py is i proved 10 Palliative chemotherapy: o ered to those patients who have unresectable malignancies and to those undergoing radiation treatments a Remission: obt ined in 60% o the c ses, but rel pse is r pid er drug cess tion b Agents: y include bro ocriptine nd cyprohept dine or itot ne P rim a ry Hyp e ld o s te ro nis m (Co nn Synd ro m e ) I Ove rvie w: excess secretion o ldosterone by the dren l s result o unil ter l deno (85%), bil ter l deno s (5%), nd hyperpl si (10%) II Typ e s : In pri ry hyper ldosteronis , pl s renin levels re nor l or low In the second ry or , the ldosterone incre se is second ry to incre sed pl s renin ro decre se in pressure on the ren l juxt glo erul r cells Co on c uses include ren l rtery stenosis, lign nt hypertension, nd ede tous st tes, such s congestive he rt ilure, cirrhosis, nd the nephrotic syndro e III Sig ns a nd s ym p to m s : Incre sed ldosterone le ds to hypertension, polyuri nd polydipsi , nd he d ches Quic k Cut It is important to distinguish hyperaldosteronism due to an adenoma rom that due to hyperplasia because surgical excision is curative or most cases o adenoma but not hyperplasia uscle we kness, tigue, IV Dia gnos is : Spironol ctone should be discontinued weeks be ore testing, s it c n ect l bor tory v lues A Plasma electrolytes: Seru pot ssiu v lue less th n 3.5 Eq/L nd urin ry pot ssiu excretion gre ter th n 30 Eq/d y supports di gnosis o pri ry hyper ldosteronis B Screening: Concurrent seru ldosterone nd pl s renin Pl s ldosterone concentr tion (PAC) o gre ter th n 20 ng/dL nd PAC/pl s renin ctivity (PRA) r tio o gre ter th n 30 re di gnostic or ldosterono with 90% sensitivity C Con rmation: Achieved by de onstr ting in ppropri te ldosterone secretion with s lt lo ding, which involves 24-hour urine collection or sodiu nd ldosterone er d ys o high-sodiu diet IV s line in usion test or c ptopril ch llenge test is lso reli ble ethod but re not usu lly required V Lo c a liza tio n o a d e no m a s A High-resolution adrenal C : best test or loc liz tion o n dren l tu or B Adrenal vein sampling (to lateralize the source o aldosterone production): use ul in p tients when there is no dren l Quic k Cut bnor lity on C but technic lly di cult CT will detect echnique: Percut neous tr ns e or l c nnul tion o an aldos terone-producing both dren l veins is per or ed nd IV AC H (50 g/hr) adenoma in 90% o cas es is d inistered Si ult neous dren l vein blood s ples 284 Chapter 17 Adren l Gl nd or ldosterone nd cortisol re t ken be ore nd er AC H injection, nd their r tios re deter ined Results: PAC is rkedly higher ( t le st our- old) on the side o n deno , where s there is little or no le –right gr dient present in c ses o bil ter l dren l hyperpl si VI Surg ic a l tre a tm e nt: For p tients who h ve pri ry hyper ldosteronis due to n drenocortic l deno , the tre t ent o choice is l p roscopic dren lecto y; it is i port nt to restore pot ssiu levels be ore surgery VII Me d ic a l m a na g e m e nt with s p iro no la c to ne : Direct Quic k Cut nt gonis to ldosterone t the kidney tubule gr du lly le ds to Favorable response reduction in blood pressure nd return to nor l pot ssiu to spironolactone in potassium levels; used in pri ry hyper ldosteronis c used by dren l and blood pressure may predict success ul improvement a ter hyperpl si nd in the preoper tive restor tion o nor l seru adrenalectomy pot ssiu levels in p tients who h ve deno s P he o c hro m o c yto m a I Ove rvie w: Function lly ctive tu ors th t develop ro the neur l crest–derived chro n tissue; “10% rule” h s been pplied to these tu ors ( ble 17-5) A Characteristics: produce excess ounts o c techol ines; c n occur s p rt o MEN2 nd re usu lly bil ter l in these p tients B Malignancy: ost o these tu ors (90%) re benign; higher incidence o lign ncy with extr dren l tu ors Determination: Histologic ex in tion is not n ccur te e ns; presence o et st ses or direct inv sion by the tu or deter ines lign ncy Cause: Pheochro ocyto s y be due to ut tions in succin te dehydrogen se genes II Lo c a tio n: Ninety percent re ound in the dren l edull ; 10% o these re bil ter l O the extr dren l tu ors, ost re ound in the org ns o Zuckerk ndl, the extr - dren l p r g ngli , the urin ry bl dder, nd the edi stinu III Sig ns a nd s ym p to m s : Hypertension results ro the excess Quic k Cut production o c techol ines Other f ndings include tt cks o Hypertens ion he d ches, swe ting, p lpit tions, tre or, nervousness, weight loss, rom pheochromocytoma is intermittent in hal o cas es tigue, bdo in l or chest p ins, polydipsi nd polyuri , nd convulsions IV Dia g no s is : Urin ry levels o et nephrine nd VMA re the ost reli ble di gnostic screening tests A Fractionated plasma and urinary catecholamine levels: c n incre se the ccur cy o the di gnosis to virtu lly 100% B Provocative tests: Using hist ine, tyr ine, or gluc gon is potenti lly h z rdous nd seldo used V Loc a liza tion o the tum or: C nd MRI h ve e erged s the ost ccur te loc lizing tools Scintigr phy with 131I-l beled m-iodobenzylgu nidine (MIBG), which structur lly rese bles norepinephrine, h s been help ul or s ll extr - dren l tu ors Selective venous s pling is r rely necess ry VI P re p a tio n o r s urg e ry: should include drenergic block de Quic k Cut with both lph - nd bet -blockers Alpha blockade A Adrenergic blockade: help ul to provide preoper tive control should precede beta blockade o hypertension, reduce the risk o dr tic swings in blood in pheochromocytoma patients pressure during surgery, nd provide v sodil tion, llowing restor tion o nor l blood volu e Alpha blockade: Achieved f rst; phenoxybenz ine ther py is begun weeks be ore surgery, st rting with 40 g/d y nd djusting the dose until hypertension is controlled Ta b le 17-5: Te n-P e rc e nt Rule o P he o c hro m o c yto m a 10% Malignant 10% Multiple 10% Bilateral 10% Familial 10% Extra-adrenal 10% Children Chapter 17 T yroid, P r thyroid, Adren l Gl nds, nd T y us 285 Beta blockade: Propr nolol is st rted bout d ys be ore surgery, to control t chyc rdi , t dose o 40 g/d y B Newer drug regimens to manage hypertension: selective lph -1- drenergic nt gonists (ter zosin nd dox zosin) nd c lciu ch nnel blockers (ni edipine nd nic rdipine) VII Op e tio n : P tient should be onitored with n rteri l nd centr l venous pressure line bec use o the potenti l or wide blood pressure ch nges; l p roscopic, tr ns bdo in l ppro ch is pre erred A Sporadic pheochromocytomas: Adren lecto y is the procedure o choice B Malignant pheochromocytomas: Surgic l excision o the tu or Che other py or ther peutic MIBG 131I c n be used or extensive et st tic dise se C Component o MEN: Bil ter l cortex dren lecto y should be considered Ad re na l Cys ts a nd Othe r Ad re na l Tum o rs I Inc id e nta l a d re na l m a s s e s : Discovered t utopsy in up to 9% o p tients With the growing use o C nd MRI sc nning, n incre sed nu ber o these “incident lo s” re being discovered during li e A Routine screening: includes tests or pheochro ocyto Quic k Cut (pl s r ction ted et nephrines), hypercortisolis Incidental adrenal (low-dose overnight dex eth sone suppression test), nd mas s es mus t be as s es s ed hyper ldosteronis (PAC/PRA r tio) or hormonal production and B Imaging: Ch r cteristics on C indic te the likelihood o malignancy lign ncy u ors gre ter th n c re t risk o c ncer nd should be re oved C Routine biopsy: should be voided unless p tient h s prior risk o lign ncy (e.g., lung or el no ) nd pheochro ocyto h s been ruled out bioche ic lly II Ad re no c o rtic a l c a rc ino m a : r re (incidence o 0.5–2 c ses per illion per ye r), very ggressive ( ost p tients present with dv nced dise se), nd produce requent hor one overproduction syndro es (hypercortisolis , hyper ldosteronis , or viriliz tion) A Diagnosis: Contr st-enh nced C o the bdo en nd chest is i port nt to di gnose loc l tu or inv sion nd et st tic Quic k Cut lesions s well s to conf r unctioning contr l ter l kidney Comp le te re s e c tion o loc a lly c onf ne d tumor B reatment: Surgic l resection is the inst y or ll st ges; is the only c nc e or however, dist nt or loc l spre d is evident in 65% o c ses t c ure rom a d re noc ortic a l present tion c a rc inoma C Prognosis: Poor; edi n surviv l ollowing di gnosis or ll p tients is 18 onths III Ad re na l c ys ts : o c c ur in re q ue ntly A ypes: Most re either endotheli l cysts (ly ph ngio tous or ngio tous) or pseudocysts, resulting ro he orrh ge into nor l dren l tissue or into n dren l neopl s R rely re they retention cysts or cystic deno s B Symptoms: L rge cyst c n present s p lp ble ss nd c n c use dull ching or GI sy pto s due to pressure C Diagnosis: C nd MRI re the best ethods v il ble D reatment: Bec use neopl s c nnot be excluded, these cysts should be surgic lly excised TUMORS OF THE ENDOCRINE PANCREAS P a tho p hys io lo g y I P a nc re a tic ne uro e nd o c rine tum o rs (P NETs ): Arise ro nor l neuroendocrine cells loc ted in the p ncre tic islets Most re spor dic, but they c n be heredit ry II Cla s s if c a tio n: ccording to their clinic l virulence A Well-di erentiated tumors: h ve slow-growing, indolent course B Poorly di erentiated tumors: high gr de nd y be very ggressive III Func tio na lity: Most PNE s re non unction l; however, they y secrete v riety o hor ones including insulin, g strin, gluc gon, nd v so ctive intestin l peptide (VIP) 286 Chapter 17 u ors o the Endocrine P ncre s Ins ulino m a I P a tho g e ne s is : u or origin ting ro the bet cells o the p ncre tic islets th t rele ses bnor lly high ounts o insulin Most re solit ry nd benign deno s; 10% re lign nt with the potenti l to et st size II Clinic a l p re s e nta tio n: Hypoglyce i y result in biz rre beh vior, unconscious episodes, p lpit tions, nervousness, nd other sy pto s o sy p thetic disch rge III Dia g no s is : H ve the p tient st or 72 hours then e sure the insulin nd glucose levels Me sure ent o elev ted insulin C peptide nd sul onylure in blood is used to identi y i trogenic insulin nd or l hypoglyce ic overdose, respectively Quic k Cut The Whipple triad includes (1) epis odes o neuroglycopenic s ymptoms (abnormal mentation, anxiety, irritability, diaphores is , tremulous nes s , atigue) precipitated by as ting; (2) hypoglycemia during the epis odes , us ually with blood glucos e levels les s than 60 mg/dL; and (3) relie o s ymptoms by glucos e adminis tration IV Surg ic a l tre a tm e nt: b sed on preoper tive loc liz tion o the tu or, which y be detect ble on C or MRI Quic k Cut A Endoscopic US: best i ging test bec use ost re less th n 1.5 c An increas ed ins ulin B Percutaneous catheterization (o the portal vein with serial level in the pres ence o a insulin measurements): c n loc lize the tu or to the he d, low glucos e level (ins ulin-tobody, nd t il glucos e ratio greater than C Surgical resection: Only cur tive tre t ent; options re 0.25) e ectively conf rms an ins ulinoma enucle tion or dist l p ncre tecto y D Surgical exploration (o the entire pancreas): O en needed to identi y s ll r diogr phic lly occult insulino Intr oper tive US is help ul E Extent: When islet cell hyperpl si is present, n 80%–90% subtot l p ncre tecto y will usu lly control the sy pto s; i the tu or is not loc lized, blind p ncre tecto y should be voided Ga s trino m a (Zo lling e r-Ellis o n Synd ro m e ) I P a tho g e ne s is : Neuroendocrine tu ors th t rise ro enteroendocrine cells o the p ncre s or intestine (duodenu ); ost g strino s re spor dic but y be co ponent o MEN type I nd c n precede develop ent o hyperp r thyroidis II Clinic a l p re s e nta tio n: Sy pto s result ro oversecretion o g strin, the consequence o which is peptic ulcer tion due to high g stric cid secretion, which y c use bdo in l p in, GI bleeding, per or tion, or g stric outlet obstruction III Inhib itio n o p a nc re a tic e nzym e s : Le ds to di rrhe , ste torrhe , nd incre sed intestin l Pro ound dehydr tion nd lnutrition y be present otility IV Dia g no s is : M de b sed on the presence o the clinic l syndro e, not whether the tu or st ins or g strin Zollinger-Ellison syndro e (ZES) should be considered in ny p tient with n ulcer th t is re r ctory to intensive tre t ent with nt cids, H 2-receptor blockers, or proton pu p inhibitors (PPIs); recurrent peptic ulcer er st nd rd surgic l procedure or peptic ulcer dise se; nd peptic ulcer dise se nd re r ctory di rrhe A Laboratory ndings: provide the di gnosis Fasting gastrin level: Gre ter th n 1,000 pg/ L (nor l 110 pg/ L) in the presence o g stric cid (g stric pH 2.0) kes di gnosis o ZES Secretin stimulation testing: M y cl ri y the c use o odest elev tions in g strin In ZES, n in usion o secretin will incre se g strin by 120–200 pg/ L or ore B Localization o the tumor: Endoscopic US is the best test to identi y s ll g strino s in the duoden l w ll nd p ncre s C scan and MRI: best cross-section l i ging studies Somatostatin receptor scintigraphy (SRS) with 111-indium-penetreotide with SPEC : less ccur te th n C but used to deter ine et st tic sites V Tre a tm e nt: ust control g stric hyper cidity nd should re ove ll gross tu or A Gastrinoma triangle: def ned by the junction o the cystic nd co on bile ducts superiorly, the junction o the second nd Quic k Cut Mos t (80% ) gas trinomas are located in the “g a s trino m a tria ng le ” Chapter 17 T yroid, P r thyroid, Adren l Gl nds, nd T y us 287 third portions o the duodenu in eriorly, nd the junction o the neck nd body o the p ncre s edi lly B Standard surgical approach: Re ove ll identif ble tu ors ( requently ulti oc l), tr nsillu in te the duodenu , open the duodenu nd resect sub ucos l tu ors, nd re ove surrounding ly ph nodes C ZES in the setting o MEN1: Unco only cured surgic lly; edic l ther py is pre erred until tu ors re ch c in size D otal gastrectomy: Previously the cl ssic tre t ent or re r ctory ZES It should be per or ed r rely VI P ro g no s is : Good i the GI hyper cidity c n be controlled; lthough two thirds o the c us tive tu ors re lign nt, they re very slow growing VIP o m a (P a nc re a tic Cho le ) I Ove rvie w: syndro e o severe secretory di rrhe (low [ 50 OS /kg] os otic g p in stool s ple) due to hypersecretion o VIP II Clinic a l p re s e nta tio n: lso known s WDHA syndrome bec use Quic k Cut o w tery di rrhe ; hypok le i (with result nt pro ound uscul r I you s ee WDHA, we kness due to the high pot ssiu content in the stool), nd think VIPoma achlorhydri III P a tho g e ne s is : PNE is solit ry in 80% o c ses nd is usu lly loc lized to the body or t il o the p ncre s; 50% re lign nt IV Dia g no s is : Most VIPo s re gre ter th n c in size t di gnosis nd y be e ectively loc lized with C sc nning V Tre a tm e nt: So tost tin receptor n logues (octreotide or l nreotide) re usu lly very e ective in controlling the secretory di rrhe Surgic l excision o the pri ry tu or is indic ted or loc lized dise se VI P ro g no s is : st ge dependent nd o en poor due to the presence o et st sis t the ti e o di gnosis Gluc a g o no m a s I Ove rvie w: r re PNE s th t produce gluc gono syndro e II Clinic a l pre s e nta tion: Sy pto s o gluc gono syndro e include necrolytic igr tory erythe (NME) (erythe tous pl ques Quic k Cut I you s ee necrolytic on the ce, perineu , nd extre ities), weight loss, di betes ellitus, migratory erythema, think ne i , di rrhe , venous thro bosis, nd neuropsychi tric sy pto s glucagonoma III P a thog e ne s is : Due to tu ors o the p ncre tic lph islet cells; ost gluc gono s origin te in the p ncre tic t il nd re lign nt IV Dia g no s is : de by e sure ent o seru gluc gon, which usu lly exceeds 500–1,000 pg/ L A C or MRI: used to loc lize the tu or B Endoscopic US: use ul to guide biopsy nd ev lu te or suspicious denop thy V Tre a tm e nt: Surgic l resection o ll loc lized gross dise se, including liver et st ses As ost p tients (50%–100%) h ve et st tic dise se, tre t ent is ost o en p lli tive For p tients with widespre d dise se, octreotide y be help ul MULTIP LE ENDOCRINE NEOP LASIA Typ e s (Thre e ) I MEN typ e (Ta b le 17-6): r re utoso l in nt condition predisposing ected individu ls to tu ors o the p r thyroid gl nds, nterior pituit ry gl nd, nd enterop ncre tic endocrine cells A Clinical de nition: two or ore pri ry MEN1 tu or types or the occurrence o single tu or type in e ber o MEN1 kindred B Cause: Inherit nce o ut ted enin gene on chro oso e 11q13 T e ex ct unction o this gene is unknown but it is thought to ct s tu or suppressor Quic k Cut C Hyperparathyroidism: present in 90% or ore o the p tients MEN1 is the three usu lly be ore ge 50 ye rs, with ost h ving ultiple P’s : p arathyroid dis eas e, p r thyroid gl nd deno s p ituitary adenomas , and D Pituitary adenomas: present in 20%–60% o c ses nd re p ancreatic tumors usu lly l ctotroph deno s 288 Chapter 17 Multiple Endocrine Neopl si Ta b le 17-6: Multip le End o c rine Ne o p la s ia Synd ro m e s Synd ro m e Clinic a l P re s e nta tio n Tre a tm e nt Ge ne /Te s t MEN1 Primary hyperparathyroidism Prolactinoma Gastrinoma/insulinoma/PPoma/VIPoma/glucagonoma/ non unctional enteropancreatic tumor Parathyroidectomy Bromocriptine Pancreatectomy/duodenal tumor enucleation Menin MEN2A Medullary thyroid cancer Pheochromocytoma Primary hyperparathyroidism Thyroidectomy/CLND Adrenalectomy Parathyroidectomy RET MEN2B Medullary thyroid cancer Pheochromocytoma Ganglioneuroma Thyroidectomy/CLND Adrenalectomy None RET MEN, multiple endocrine neoplasia; PP, pancreatic polypeptide; VIP, vasoactive intestinal peptide; CLND, central cervical lymph node dissection; RET, rearranged during trans ection tyrosine kinase proto-oncogene E Enteropancreatic tumors (PNE ): present in up to 80% o p tients Common: G strino is the ost co on sy pto tic tu or nd occurs in up to 60% o MEN1 p tients I ge-detected non unctioning tu ors o the p ncre s re s co on Less common: Sy pto tic insulino , gluc gono , nd VIPo c n occur Metastasis: Both unctioning nd non unctioning PNE y et st size to liver; lign nt PNE is the ost co on c use o MEN1-specif c ort lity II MEN typ e 2A: Co prises M C, pheochro ocyto , nd, in MEN 2A, p r thyroid hyperpl si MEN2 syndro es re c used by ut tion o the RE gene, which ps to 10q11.2 nd cts s in nt oncogene A Medullary thyroid carcinoma: Occurs in ll p tients; it is usu lly ulti oc l nd preceded by non lign nt hyperpl si o the C cells Seru c lcitonin levels re incre sed B Pheochromocytomas: 40% o p tients; o en bil ter l, synchronous, nd usu lly benign; o en presenting l ter th n edull ry thyroid c ncer C Parathyroid hyperplasia (with consequent hyperparathyroidism): develops in 60% o p tients with MEN 2A nd is o en ilder th n the p r thyroid hyperpl si o MEN III MEN typ e 2B: s in MEN 2A, edull ry thyroid c rcino nd pheochro ocyto A Features: r noid body h bitus nd the develop ent o ultiple neuro tous ucos l nodules B Presentation: f rst or second dec de o li e with uch ore ggressive course Dia g no s is I MEN1: Hyperp r thyroidis in MEN1 is typic lly sy pto tic nd is usu lly detected by l bor tory bnor lities (c lciu nd P H) A Clinical presentation: P tients with MEN1 y present with sy pto s o peptic ulcer tion rel ted to the p ncre tic g strino or with sy pto s rel ted to the pituit ry tu or B Genetic testing: Mut tions in enin testing is v il ble to ny index p tient with clinic l MEN1 nd ll f rst-degree rel tives o known MEN1 ut tion c rriers C Asymptomatic biochemical screening: involves c lciu nd y include other ore det iled hor on l screening or g strin, sting glucose, insulin, insulinlike growth ctor 1, prol ctin, nd chro ogr nin A II MEN2A: Should be suspected in ny p tient with M C; p tient should be re erred or genetic counseling nd testing or ut tion o the RE proto-oncogene Finding o n incre sed seru c lcitonin level le ds to the di gnosis A Hyperparathyroidism: usu lly detected by incre ses in the seru c lciu nd P H levels B Pheochromocytomas or adrenal medullary hyperplasia: y be sy pto tic but should be detect ble by bioche ic l screening III MEN2B: Most p tients re di gnosed in e rly childhood; bec use it is ggressive, e rly di gnosis is i port nt so th t e ective tre t ent c n begin pro ptly Di gnosis is si il r to th t or MEN2A T e e rly ppe r nce o ucos l neuro s nd the r noid body h bitus ids di gnosis Chapter 17 T yroid, P r thyroid, Adren l Gl nds, nd T y us 289 Tre a tm e nt I MEN1: PHP in MEN1 involves ll gl nds, usu lly sy etric lly Surgery is not cur tive but p r thyroidecto y y reduce circul ting P H Choice o ppro ch is l rgely b sed on surgeon tr ining nd experience A Subtotal (3.5 gland) parathyroidectomy: Le ving nor l-sized well-v scul rized re n nt o p r thyroid tissue is co on ppro ch B otal parathyroidectomy (with nondominant orearm Quic k Cut parathyroid autotransplantation): y lso be per or ed Autotrans plantation C PNE s (located within the pancreas and duodenum): o parathyroid tis s ue to the Resection is per or ed to reduce thre t o lign nt spre d nd orearm has the advantage o to reduce hor on l sy pto s Resection o non unctioning avoiding repeat neck s urgery PNE s should be considered with gre ter th n c lesion i recurrence develops ZES: enucle tion o the tu or loc ted in the g strino tri ngle Insulinoma: enucle tion o tu ors in the p ncre tic he d nd y include dist l subtot l p ncre tecto y D Pituitary tumors: Usu lly tre ted edic lly with bro ocriptine r ns-sphenoid l hypophysecto y y be needed in so e c ses II MEN2A: P tients with known MEN2A– ssoci ted RE ut tions should receive prophyl ctic tot l thyroidecto y, which is cur tive A Pheochromocytoma or adrenal medullary hyperplasia: Adren lecto y (pre er ble l p roscopic) be ore thyroidecto y bec use these hor one-producing disorders c n le d to hypertensive crises during thyroidecto y B Hyperparathyroidism: c n be tre ted t the ti e o tot l thyroidecto y by either subtot l p r thyroidecto y or tot l p r thyroidecto y nd ore r p r thyroid utotr nspl nt tion III MEN2B: re ted si il rly with MEN2A Bec use MEN2B ssu es such n ggressive course, pro pt nd e ective tre t ent is i port nt THYMUS GLAND Ove rvie w I Intro d uc tio n: T y us is the origin o v riety o tu ors nd is signif c ntly involved in the develop ent o cellul r i unity A Cortex: consists pri rily o ly phocytes, which ppe r to igr te to the edull nd then e igr te ro the thy us B wo limbs: extend into the neck nd re o en ssoci ted with the in erior p r thyroid gl nds II Func tio ns : Essenti l or the develop ent o cellul r i unity, which controls del yed hypersensitivity re ctions nd tr nspl nt rejection Histologic bnor lities in the thy us (e.g., ly phoid hyperpl si or thy ic tu ors) re requently ound in ssoci tion with utoi une dise ses Mya s the nia Gra vis Quic k Cut I Ove rvie w: Autoi une dise se o neuro uscul r tr ns ission Myas thenia th t c uses skelet l uscle we kness T e ost co on sy pto s gravis exacerbations may re ptosis, double vision, dys rthri , dysph gi , n s l speech, nd be precipitated by upper we kness o the r s nd legs res piratory in ections II No rm a l ne uro m us c ula r tra ns m is s io n: Acetylcholine (ACh) is produced t the nerve ter in l o the yoneur l junction nd binds to receptor sites on the uscle endpl tes, which triggers uscle contr ction III Ne uro m us c ula r tra ns m is s io n in m ya s the nia g vis : ACh receptor ntibodies develop, resulting in reduced uscle contr ction IV Tre a tm e nt: Sust ined i prove ent is chieved with edic tion in only 33% o p tients without surgery nd in 85% o p tients er thy ecto y A Medical treatment: P tients respond to drugs th t sti ul te the neuro uscul r junction, such s neostig ine nd pyridostig ine B Surgical treatment: Re ov l o the thy o is dvised, lthough the e ect on the y stheni is unpredict ble However, even in p tients without thy ic tu ors, thy ecto y ppe rs to be the tre t ent o choice 290 Study Questions or P rt V Study Questions or P rt V Directions: Each o the numbered items in this section is ollowed by several possible answers Select the ONE lettered answer that is BEST in each case A 40-ye r-old n h s subtot l thyroidecto y per or ed or Gr ves dise se Sever l hours l ter, he co pl ins o di culty bre thing On ex in tion, he h s stridor nd rkedly swollen, tense neck wound Wh t should be one o the f rst steps in the n ge ent o this p tient? A B C D E Intub te with n endotr che l tube Per or tr cheosto y Control the bleeding site in the oper ting roo Open the wound to ev cu te the he to Aspir te the he to A 50-ye r-old hypertensive n h s def nitive bioche ic l evidence o pheochro ocyto C sc n nd MRI not reve l ny bnor lities, nd MIBG sc nning is not re dily v il ble Wh t should be the next step in the n ge ent o this p tient? A B C D E Abdo in l explor tion Continued clinic l observ tion Medi stinoscopy Selective venous s pling Medi stin l explor tion A 55-ye r-old wo n with progressive but episodic uscle we kness is di gnosed s h ving y stheni gr vis Her chest r diogr ph is nor l nd reve ls no evidence o edi stin l ss or tu or Wh t is the ost def nitive tre t ent th t c n be o ered to this p tient? A B C D E Prednisone Neostig ine T y ecto y Pl s pheresis Atropine A f rst-degree rel tive o Which screening A B C D E p tient ound to h ve dv nced M C is re erred or urther ev lu tion e sure is the choice or detection o edull ry thyroid p thology? C re ul physic l ex in tion Seru c lcitonin level Sti ul ted seru c lcitonin level (c lciu G strin level C rcinoe bryonic ntigen (CEA) level I nd pent g strin) f rst-degree rel tive o p tient with MEN2A syndro e is ound to h ve edull ry p thology requiring surgic l explor tion o the thyroid gl nd, wh t should the preoper tive screening include? A B C D E Seru cortisol level F sting glucose nd insulin C sc n o the he d Urin ry ldosterone nd renin Urin ry VMA nd et nephrines Study Questions or P rt V A 60-ye r-old e le p tient h s workup or episodic sy pto s o p lpit tions, nervousness, nd biz rre beh vior, ll o which tend to occur during sting st tes Bioche ic lly, she is di gnosed s h ving n insulino Wh t is the best choice or loc lizing this tu or? A B C D E C sc n MRI Selective rteriogr phy Percut neous c theteriz tion o the port l vein with selective venous s Surgic l explor tion nd intr oper tive ultr sound pling A 55-ye r-old e le p tient is ev lu ted or new onset o di betes ellitus Her edic l history is l rgely unre rk ble Her physic l ex in tion is unreve ling except or the presence o n erythe tous skin r sh Her urther ev lu tion should include n investig tion o the possibility o which o the ollowing? A B C D E Insulino Gluc gono G strino C rcinoid tu or P ncre tic choler A 23-ye r-old wo n h s p lp ble ss in her le bre st Ultr sound suggests cystic lesion On spir tion, so e d rk uid is retrieved, but the ss does not co pletely resolve Wh t is the next step in tre t ent? A B C D E Observ tion NSAIDs Repe t spir tion Excision Si ple stecto y T e best tre t ent or duct l c rcino A B C D E in situ is: Observ tion nd re ssur nce Lu pecto y P rti l stecto y with r di tion Si ple stecto y Modif ed r dic l stecto y 10 T e nerve th t supplies the l tissi us dorsi uscle nd y be injured during xill ry node dissection is: A B C D E Long thor cic T or codors l C7 Intercost l br chi l L ter l br chi l 11 A 30-ye r-old n h s hypertension nd h s co pl ints o we kness nd tigue His physic l ex in tion is unre rk ble Routine l bs re nor l except or pot ssiu o 3.0 Eq/L He undergoes bdo in l C sc n, which de onstr tes 3-c le dren l ss T e best next step in n ge ent is: A B C D E MRI o the bdo en with i ging Urin ry cortisol onitoring Bet block de Adrenocorticotropic hor one sti ul tion L p roscopic dren lecto y 291 292 Study Questions or P rt V 12 A 56-ye r-old wo n is undergoing surgery or le -sided p r thyroid deno T e initi l p r thyroid level is 100 pg/ L A er incision, the in erior gl nd is identif ed nd re oved, nd the p r thyroid level t 10 inutes lls to 90 pg/ L T e next step is: A B C D E Closure o incision Monitoring o 20- inute P H levels Explor tion o the le superior gl nd ot l p r thyroidecto y with utotr nspl nt tion St t c lciu e sure ent 13 Which o the ollowing is not ssoci ted with n incre sed incidence o inv sive duct l c rcino o the bre st? A B C D E Sclerosing denosis Lobul r c rcino in situ Atypic l duct l hyperpl si Epitheli l hyperpl si P pillo tosis Que s tio ns 14–17 Match the correct treatment with each inf ammatory or in ectious process o the breast 14 M stitis A Surgic l dr in ge 15 Abscess B Excision o sinus tr ct 16 Chronic sub reol r bscess C Antibiotics 17 Mondor dise se D NSAIDs 18 T e pre erred tre t ent or new-onset pubert l gyneco A B C D E R dic l excision with l tissi us dorsi Observ tion Suction lipecto y oxi en ther py Si ple stecto y sti is: p Que s tio ns 19–20 A 65-ye r-old wo n with no other signif c nt p st edic l history presents with l rge ss in the right bre st T e ss e sures pproxi tely c in di eter nd ppe rs to be f xed to the chest w ll In ddition, bulky denop thy is present in the right xill ry region T e p tient st tes th t the ss h s been enl rging or the l st sever l ye rs 19 Following A B C D E ogr phy, wh t should be the next step in this p tient’s ev lu tion? FNA Incision l or core biopsy Excision l biopsy Modif ed r dic l stecto y R dic l stecto y Study Questions or P rt V T e di gnosis or this p tient is inv sive duct l c rcino A ogr reve ls no other lesions in the right bre st nd no bnor lities in the le bre st A chest r diogr ph, bone sc n, nd liver unction tests re nor l 20 Wh t should the next step in the A B C D E n ge ent o this p tient involve? Neo djuv nt che other py R di tion ther py to the bre st nd xill R dic l stecto y Modif ed r dic l stecto y Si ple stecto y 293 294 Answers nd Expl n tions Answers nd Expl n tions The a ns we r is D (Ch pter 17, T yroid Dys unction Requiring Surgery, Gr ves Dise se, V E 2) Postoper tive bleeding er thyroidecto y c n c use irw y co pro ise due to tr che l co pression T e f rst step should be to open the wound to ev cu te the he to , ollowed by return to the oper ting roo to control the bleeding site Atte pts to per or either endotr che l intub tion or tr cheosto y y be di cult until the extern l co pression o the he to is relieved The a ns we r is D (Ch pter 17, Adren l Gl nd, Pheochro ocyto , V) Although 90% o pheochro ocyto s re loc ted in the dren l gl nds, they c n occur in ny tissue th t is derived ro neuroectoder When C sc n nd MRI not identi y tu or, MIBG sc nning c n be help ul; however, this is not lw ys v il ble Selective e sure ents o c techol ines dr wn t v rious levels ro the ven c v nd its jor br nches should be obt ined be ore surgic l explor tion The a ns we r is C (Ch pter17, T y us Gl nd, My stheni Gr vis, IV B) My stheni gr vis is n utoi une dise se o neuro uscul r tr ns ission th t c uses skelet l uscle we kness P r sy p tho i etic drugs h ve been ound to i prove uscle strength in these p tients Prednisone h s lso been used with so e success bec use o the utoi une n ture o this dise se Pl s pheresis y be e ective in prep ring the p tient preoper tively T e tre t ent o choice or ll or s o y stheni , except purely ocul r, ppe rs to be thy ecto y An incre sed percent ge o p tients h ve per nent re ission T e response to edic tion is i proved in p tients who not chieve co plete re ission The a ns we r is B (Ch pter 17, T yroid Dys unction Requiring Surgery, T yroid Neopl s s, VI A 2) All f rst-degree rel tives o p tients with M C should be screened or this disorder bec use it c n occur in ili l p ttern Physic l ex in tion o the thyroid gl nd should be per or ed or the detection o ny nodules An incre sed seru c lcitonin or n incre sed sti ul ted seru c lcitonin test will lso indic te underlying edull ry p thology, either hyperpl si or c rcino T e sti ul ted tests will detect dise se t n e rlier, ore cur ble st ge Incre sed g strin levels re ssoci ted with ZES nd re not p rt o this MEN type syndro e CEA is elev ted in so e GI lign ncies The a ns we r is E (Ch pter17, Adren l Gl nd, Pheochro ocyto , IV) M C y present s spor dic or ili l or ssoci ted with MEN type 2A or 2B Both re ssoci ted with pheochro ocyto s I pheochro ocyto is present, it should be di gnosed nd tre ted f rst to void the orbidity o cervic l explor tion in p tient with untre ted pheochro ocyto Urin ry VMA nd et nephrines should be ev lu ted preoper tively The a ns we r is E (Ch pter 17, u ors o the Endocrine P ncre s, Insulino , IV) T e p tient h s h d def nitive bioche ic l di gnosis o insulino T ese tu ors c n be present nywhere in the p ncre s Bec use they re usu lly s ll in size, rteriogr phy, C , nd MRI re less sensitive th n they would be or l rger tu ors With c re ul surgic l explor tion nd intr oper tive ultr sound, pproxi tely 90% o these tu ors c n be loc lized t the ti e o surgery The a ns we r is B (Ch pter 17, u ors o the Endocrine P ncre s, Gluc gono s, II) Gluc gonproducing tu ors o the p ncre s secrete gluc gon in l rge ounts P tients tend to present with new onset o di betes ellitus (hyperglyce i ) A ected individu ls lso ch r cteristic lly h ve igr tory erythe tous skin r sh The a ns we r is D (Ch pter 16, Bre st Ev lu tion, Biopsy, II) Cysts th t not resolve co pletely on spir tion should be tre ted s solid lesions nd excised NSAIDs re use ul in Mondor dise se (thro bophlebitis) Repe t spir tion y be tte pted, but cyst th t recurs once is likely to recur g in M stecto y is too r dic l ther py Answers nd Expl n tions The a ns we r is C (Ch pter 16, M lign nt Dise ses, Noninv sive Bre st C ncer, I A) Excision with cle r rgins is the st nd rd o c re, nd r di tion reduces the risk o recurrence by 50% 10 The a ns we r is B (Ch pter 16, M lign nt Dise ses, re t ent, I B c) T e thor codors l supplies the l tissi us T e long thor cic supplies serr tus nterior, nd injury produces winged sc pul 11 The a ns we r is E (Ch pter 17, Adren l Gl nd, Pri ry Hyper ldosteronis [Conn Syndro e], VI) T e p tient h s signs nd sy pto s o ldosterono nd i ging consistent with le dren l deno Further cross-section l i ging is unnecess ry Cortisol testing is used or suspicion o dren l hyper unction or insu ciency but is not needed in this c se Bet block de y be use ul or n ge ent o hypertension but would not be indic ted in the initi l n ge ent o pheochro ocyto In pr ctice, n ldosterone level could be checked preoper tively to conf r the di gnosis, then l p roscopic le dren lecto y 12 The a ns we r is C (Ch pter 17, P r thyroid Gl nds, Hyperp r thyroidis , I F 1) T e p tient h s evidence o loss o p r thyroid tissue but not su cient response to consider the bnor l gl nd re oved It is expected th t there is both 50% reduction in the P H level s well s ove ent into the nor l r nge Given th t this did not occur, explor tion o the re ining gl nds is the next step ot l p r thyroidecto y is overly ggressive, nd onitoring o ddition l l b v lues o P H or c lciu does not lter the decision- king process 13 The a ns we r is A (Ch pter 16, Benign Bre st Dise se, Benign Lesions, III G) Epitheli l hyperpl si , typic l duct l hyperpl si , nd p pillo tosis re proli er tive lesions o the bre st th t c rry n incre sed risk o inv sive duct l c rcino o the bre st P pillo tosis is si ply description o the p ttern the cells ssu e (p pill ry) Lobul r c rcino in situ o the bre st c rries n incre sed risk bil ter lly or n inv sive bre st c ncer, which c n be duct l or lobul r Sclerosing denosis is proli er tion o the cini th t ppe r to inv de, but it is not lign nt or pre lign nt lesion 14–17 The a ns we rs a re 14-C, 15-A, 16-B, a nd 17-D (Ch pter 16, Benign Bre st Dise se, In ectious nd In tory Bre st Dise ses, I–IV) Cellulitis o the bre st ( stitis) requires tre t ent with ntibiotics to cover Staphylococcus nd Streptococcus in ection An cute bscess requires surgic l dr in ge A chronic recurrent bscess requires excision o the sinus tr ct to void recurrence Mondor dise se is phlebitis o the superf ci l veins, nd lthough sel -li ited, tre t ent with NSAIDs c n llevi te the disco ort 18 The a ns we r is B (Ch pter 16, M lign nt Dise ses, Speci l C ses, II A 3) Gyneco sti represents bre st growth in le due to hor on l i b l nce T e initi l present tion during puberty should be observed, s the lesion o en regresses Sy pto tic c ses c n be tre ted edic lly with t oxi en or surgic lly with suction lipecto y More ggressive surgery such s stecto y is not w rr nted 19–20 The a ns we rs a re 19-B (Ch pter 16, Bre st Ev lu tion, Biopsy, I B) nd 20-A (Ch pter 16, Inv sive Bre st C ncer, re t ent, V B) Although FNA c n be per or ed, it y not be conclusive to w rr nt urther tre t ent A core-needle biopsy c n e sily be per or ed on o this size Excision is in ppropri te in sses l rger th n c Def nitive surgic l ther py should not be per or ed until er neo djuv nt che other py is given ss 295 ... 10 –20 12 0 14 0 Nil 0 .1 2 L 10 0 12 0 10 –20 11 0 10 –20 Ileum 1 3 L 80 14 0 5 10 60–90 30–50 Colon 0 .1 2 L 10 0 12 0 10 –30 90 30–50 Gallbladder 0.5 1 L 14 0 10 0 25 Pancreas 0.5 1 L 14 0 Stomach Duodenum 30... 16 1 Che rif Bo utro s , Erne s t L Ro s a to , a nd Fra nc is E Ro s a to J r Stomach 16 1 Peptic Ulcer Disease 16 3 Gastric Cancer 16 8 Postgastrectomy Syndromes 17 0 Benign Stomach Lesions 17 1... 11 8 Lower Extremity Arterial Occlusive Disease 11 8 Aortoiliac Occlusive Disease (AIOD) 12 0 Femoropopliteal Occlusive Disease 12 2 In rageniculate ibial Disease 12 3 Acute Arterial Insu ciency 12 4