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    ADRENAL AND EXTRA-ADRENAL

    HORMONE SECRETING TUMOURS

    A SEVEN YEARS EXPERIENCE AT RGH

    Dr.Nadir Mehmood

    Assistant ProfessorSurgery

    Rawalpindi Medical College

    Rawalpindi

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    INTRODUCTION

    Small in size but powerful in influence , the Adrenal glands and theirhormones are necessary to sustain life and maintain homeostasis.

    Adrenal glands were overlooked by early anatomist and were firstaccurately described in 1563 by Bartholomeos Eustachius.

    More than 300 years passed before there was some understandingof the functions of these glands.

    Adrenal and extra adrenal tumors are rare and often present laterwhen physiological derangement is well established .

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    INTRODUCTION

    Despite the rarity of such tumors there have beensignificant advances in the understanding andmanagement of these tumors in the recent years.

    Disorders of Adrenals and the related structure maypresent in a variety of ways depending on the effects ofhormones they produce.

    Even such tumors may produce mass effect or be an

    incidental finding in the course of investigating some

    other problem. So such findings do not always warrant treatment.

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    PHYSIOLOGY

    Hyperfunction of adrenal gland is divided into threecategories depending upon the main hormone ofsecretion

    1: Hypercotisolism

    Cushing syndrome ( primary adrenal pathology

    Cushings disease (secondary to raised ACTH)

    2: Hyperaldosteronsim

    Conns syndrome ( primary adrenal pathology)

    Secondary hyperaldosteronism ( chronic renalfailure

    3: Hypercathecholaminemia

    Adrenal glands tumors

    Extra adrenal chromoffin tissue tumors

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    PHSYIOLOGY

    As these adrenal hormones regulate a wide range ofphysiological functions,it is not surprising that hyperplastic orneoplastic conditions of adrenal and extra-adrenal tissues cancause excessive secretion of various hormones resulting in avariety of clinical syndrome

    The presentation and diagnosis of adrenal and extra-adrenaltumors secreting hormones is complex and the discriminatingfactors for each are necessary to minimize the time and costin diagnosis.

    Because of the rarity of such tumors, most of the studies in

    the literature are retrospective in nature.

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    PURPOSE OF THE STUDY

    To evaluate a broad range of features ,including

    Clinical Features

    Evolution of disease/syndrome

    Hormone secretion

    Imaging technique

    Problems during anesthesia

    Approaches to the exposure of the related

    structures Technique used

    Intra and postoperative morbidity and mortality

    Histopathological diagnosis

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    METHOD

    A detailed history, especially pertaining to headache,sweating,palpitation,flushing,mass or pain abdomen,uncontrolled hypertension, breathlessness, episodicweakness of limbs or tremors in hand, nausea,vomiting or diarrhea and weight loss/gain wasrecorded on data sheets.

    History of any medical treatment for the presentingsymptoms/complaints and their efficacy in relievingthe complaints was recorded.

    Previous medical and surgical history,family H/O anydisease like hypertension,diabetes or obesity or tumorof any site was recorded.

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    METHOD

    A detailed clinical examination of each patient includingweight and height, vital signs, skin, face, neck, hairdistribution, extremities and abdomen was performed.

    A record of blood pressure was analyzed, especiallyafter the abdominal examination/palpitation in patientswith pheochrmocytoma.

    Patients with visual complains and headaches weretested for visual acuity and in patients with severe

    uncontrolled hypertension, fundoscopy was alsoperformed.

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    INVESTIGATIONSRoutine investigations on all patients

    C.P. E.S.R

    R/E urine

    B.S.R

    Urea and creatinine

    Serum electrolyte Blood grouping

    E.C.G

    Chest X-ray P.A view

    Special tests were selectively performed including,

    L.F.Ts

    I.V.U

    Echocardiography

    Serum hormones level - cortisol, aldosterone, renin andurinary VMA

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    INVESTIGATIONS

    Routine imaging included

    - Ultrasound examination

    - C.T scan on all patients Results of all these investigations

    were listed in tabulated form and

    the findings of ultrasound, C.T scanand operative findings werecomparatively analyzed.

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    OPERATIONS A total 14 operations were performed in 13 patients. One patient with bilateral adrenal pheochromocytoma was operated

    twice , once at a time.

    2 patients were not operated, one with incidentaloma, and the otherwith right adrenal pheochromcytoma with multiple metastasis in theliver ( Pt.refused operation )

    Preoperative blood pressure was controlled using a combination ofdrugs which included

    - Diovan(Valsartan)

    - Prazocin

    - Nifidipine

    - Atenolol

    - Frusemide

    for patients with pheochromocytoma and Cushing syndrome and- Spinolactone for patients with Conns synd.

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    TECHNIQUE

    Antibiotic prophylaxis as 1 gm of 1st generationCephalosporin I/V was given to all the patientsfollowed by two doses postoperatively.

    General anesthesia by a senior anesthetist. Posterolateral approach through the bed of 12th rib

    in 11patients.

    Anterior midline in 2 patients with paraganglionoma

    and pheochromocytoma of organ Zukerkandle.

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    Peroperative tachyarrhthmia during themanipulation of pheochromocytoma wascontrolled by infusion of lignocaine, and rise inB.P was controlled by nitrocine infusion.

    Postoperative hypotension of varying degreeoccurred in almost all patients withpheochromocytoma, even the one with bilateraladrenal pheochromocytoma which wascontrolled by infusion of:

    0.9% N/Saline

    Gelafundin

    Blood transfusion

    Infusion of dopamine

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    Replacement of fluid ranged from 3.5 litres to 7 litres

    Average 5.25 litres as

    -N/saline 2000ml

    -Gelafundin 100ml

    -5% D/W 1000ml-2 units of blood.

    Coticosteriod replacement was given to all patientsafter surgery for Cushings syndrome and after secondoperation on patient with bilateral pheochromocytoma.

    The patients were followed up for an average of 2.5years, ranging from 12 months to 5 years

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    PRESENTATION /CLINICAL

    FEATURES

    PHEOCHROMOCYTOMA

    Recurrent episodes of severe headache,sweating palpitation and flushing ( face, handsand chest) 7

    Triad of headache, palpitation and sweating withmass in Rt.H.C and Rt.L.R 1

    Pain in Rt.L.R and Rt.H.C.associated with triad1

    PARAGANGIONOMA

    Mass in Rt.H.C 1

    Hypertension not controlled by ordinary dosageof antihypertensive drugs

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    PRESENTATION /CLINICAL

    FEATURES

    CONN SYNDROME

    Episodic weakness of limbs and tremors in the hand 3

    Adding headache,palpitation and breathlessness 1

    Other symptoms of nausea, vomiting and diarrhea 1

    CUSHING DISEASE

    Obesity,breathlessness on exertion, mood changes and easybruisibility. 2

    Off and on complain of backache, joint pain, increased appetite, weightgain, sweating, vertigo and headache

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    Age

    Most of the patients were young

    Age Range 15 - 45 years

    Average age 31.066 years

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    ON CLINICAL EXAMINATION

    Pulse rate Range 80-96/min

    Av 80/min

    B.P Systolic range 140-240mmhg

    Av 196

    Diastolic range 95-150mmHg

    Av 128.57mmHg

    R/R Range 18-28/min

    Av 20/ min

    Weight 125kg=2 with cushing Incident 130kg

    Ht 5 . 1, 5 . 2 and 5 . 4

    Range 46-96kg with pheo and conn Av 62.09kg

    Height 4 . 10 - 5 . 8Av 5 . 2

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    ABDOMINAL EXAMINATION

    Mass abdomen in3 cases - all pheo (20% of all cases )

    Visible bulge and palpable mass that was ballotable andmoved with respiration.

    After completing the abdominal examination patientstypically experienced episodes of headache, sweatingand flushing -- all pheo cases

    So medical students and junior residents were refrainedfrom examination of patients with suspectedpheochromocytoma.

    Striae distentie on abdomen, chest and limb in boththe cases of Cushings and incedendaloma.

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    INVESTIGATIONS

    H.b Range 9.6 - 15gm

    Av. 11.6533gm

    T.L.C Range 4400-8900

    Av. 6200

    E.S.R Range 10-90mn

    Av. 23.6mn

    Urine R/E Albumin +/++3 cases

    B.S.R

    S.Urea/ creatinine

    L.F.Ts Select

    H.B.V and C Screen all

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    INVESTIGATIONS

    S.Electrolytes

    Na Range N Av N

    K Range 1.3-2.6 Av 2(conns only)

    Cl Range N Av N

    Ca Range N Av N

    S.Renin Range 0.6-2.62

    Av 1.32666

    S.Aldosterone Range 35.9-130

    Av 91.8666

    S.Prolactin 789.2 (N-61-512.m.I.u/ml)

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    Three measurements of 24-hr urinary VMA

    in 10 patients of pheochromocytoma and

    incidentaloma

    Range 4 - 15.6mg / 24hr

    Average9.1972mg / 24hr

    Normal upto 8 mg / 24 hrNo relation with the size of the tumor

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    Tumor N Male Female Rt Lt B/L

    Total 15 5 10 7 5 1

    Adrenal 13 5 8 7 5 0

    Pheo 7 4 3 4 2 1

    Conn 3 1 2 2 1 0

    Cush 2 0 2 1 1 0

    Incid 1 0 1 0 1 0

    Pheo OrgZ 1 0 1 0 0 0

    Paragang 1 0 1 0 0 0

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    PREOP-PREPARATION Patients were admitted on an average of 5 days preoperatively B.P control with

    Cap Diovan ( Valsartan) 80mg OD

    Tab minipres ( prazocin ) 1 mg - 2mg T.D.S

    Cap Adalet ( nifidipine) 10 mg-20 mg T.D.S

    Tab Atenolol 50mg-100mg O.D/ B.D

    Tab Frusemide 20mg-40mg O.D or B.DTab Spinoloctone 100mg TDS

    Tab Voltaral( diclfenac) 50mg BD for pain

    Correction of iron deficiency with hematanics

    Fluids

    C.V.P was passed in Rt.Subclavian vein via infraclavicular route in O.T.

    preoperativly.

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    DURATION OF SURGERY

    Range 1.75 hrs - 5.5hrs

    Average 2.5 hours

    Postoperative stay

    Range 7 - 15 days Average 9.3 days

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    MORBIDITY

    No wound infection

    Chest infection in 1 patient

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    MORTALITY

    One patient with paraganglianoma

    suffered from cardiac arrestperoperatively.

    Died on 3rd postoperative day due toC.V.A. and respiratroy distress.

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    FOLLOW UP

    After two months of follow up all the

    patients were symptomatically improvedwithout any antihypertensive therapy.

    Serum electrolyte and other laboratory

    data became normal after the first week ofoperation.

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    S. No. U/S C.T Scan V.M.A Perop Finding

    1. 83 X 68mm 80 X60mm 4.7mg/ 24hr 100 X 90mm2. Normal 30 X 20mm 7.5mg/ 24hr 30 X 20mm3. 40 X 16mm 24 X 25mm 9.5mg/ 24hr Not operated4. Normal 6.0mg/ 24hr 5. 55 X 52 X 40mm 45 X 38 X 48mm 15.6mg/ 24hr 45 X 35 X 45mm6. 120 X 80 X 50mm 150 X 90X 60mm 13.2mg/ 24hr 150 X 90 X 60mm7. (Rt) 35 X 39mm

    (Lt) 36 X 24mm41.27 X 35.63

    36 X 24mm40 X 50mm30 X 40mm

    8. 65 X 80mm 58 X 75mm 9.5mg/ 24hr 55 X 75mm9. 30 X 38mm 35 X 40mm 11.2mg/ 24hr 35X 40mm10. 40 X 29mm 40 X 33mm 9.0mg/ 24hr 40 X 30mm11. Huge mass>150mm Huge mass>150mm 7.5mg/ 24hr Not operated12. 15 X 12mm 15 X 12mm 15 X 10mm13. 35 X 40mm 35 X 40mm 7.6mg/ 24hr 35 X 40mm14. 25 X 30mm 26 X 32mm 25 X 30mm15. 50 X 40mm 51 X 40mm 9.5mg/ 24hr 50 X 40mm

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    ConclusionYoung hypertensive patients should be

    investigated thoroughly.

    Pre-op preparation is essential to succesful

    management of such patients.

    Comparing theresult of U/S, C.T. and operativefindings, U/S had a sensitivity of 86.6% in

    diagnosing tumors compared with 100%

    sensitivity of C.T. scan.Urinary VMA excretion was not related to size of

    the tumor.

    Over-night dexamethasone test has good

    diagnostic yield in hypercathecholaminia.

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    Cortical sparing adrenalectomy is

    a good option in patients with

    bilateral tumors.

    Sequential operation is better

    choice to simultaneous surgery in

    patients with bilateral tumors.