Adrenocortical carcinoma

Slide 1

Adrenocortical carcinoma

Dr. Ravi Roshan KhadkaPhase B residentDepartment of UrologyBSMMU

Patient ProfileName: Md .SakhawatAge : 28 yearsGender: MaleOccupation: day labor in MalaysiaMarital status : unmarriedFathers name : Md Mujim sheikhPermanent add: Dhaka Date of admission : 02/09/15

Chief complaintsPain at left upper abdomen------1 yearLoss of appetite and nausea ---- 1 yearOccasional fever --------- 1 year

3

History of present illnessAccording to patients statement he was apparently well 1 year back and then he gradually developed pain at left upper abdomen which was dull aching in nature & mild to moderate in intensity. Pain radiates to back which used to aggravated by exertion and food intake and relieved by medication. Pain was episodic in nature and associated with nausea and loss of appetite and weight loss of 21 kg in 1 year. He also complaints of occasional low grade fever which was relived by antipyretic.

History of present illnessHe gave no history of coughing out of blood, blood mixed urine, passage of stone in urine, cloudy urine or bone pain.With these complaints he visited general physician and treated symptomatically and 1 months back after returning to bangladesh he underwent thorough investigations and later referred to department of urology for further evaluation and management.

Past medical and surgical historyNothing contributory

Family historyNothing contributory

Personal historyEducation level : SSCOccupation: day labor in Malaysia Smoking/ Tobacco: non smokerAlcohol : non alcoholicSocioeconomic status : lower middle class

Drug historyAnalgesic, antipyretic and antiemetic and antibiotics for symptomatic relief.

General physical examinationsAppearance: anxious and illookingBuilt: average Weight: 54 kgheight: 1.65 mBMI=19.8Anaemia: moderately pallorJaundice: AbsentOedema: AbsentLymph nodes: Accessible lymph nodes are not palpableClubbing : AbsentKoilonychia : AbsentCyanosis : absent

Abdomen ExaminationInspection:Scaphoid shape with slight bulge at left hypochondriac regionUmbilicus centrally placed and inverted Flanks are not fullNo visible peristalsis and engorged veinNo visible cough impulse

Abdomen ExaminationPalpation Temperature ; not raisedMass at left hypochondriac regionThere is a Hard, mild tender, irregular mass at left hypochondriac , epigastric and lumbar region measuring about 8 cm from left costal margin along MCL. Margins are irregular and upper limit of margin couldnt be traced.Insinuation is not possible and notch is absent.Mass moves with respiration.BallotableHead rising test; Mass didnt bulge Knee elbow test ; Mass doesnt fall forwardNo other organomegaly

Abdomen ExaminationPercussionDull with band of resonanceTympanytic all over abdomenAuscultationNo bruitBowel sound ; presentD/R/E ; NormalGenital exam : normal

Systemic examination

CVSRespiratory systemNervous system

NAD

Salient featureMd. Sakhawat 28 years old , non diabetic, normotensive, nonsmoker, male , hailing from dhaka presented with gradual onset of episodic dull aching left hypochondriac pain for 1 year. Pain was mild to moderate in intensity that radiates to back and aggravates by exertion and food intake and relieved by medication. He also complaints of nausea , vomiting and significant weight loss (21 kg in 1 year) for same duration. He is also experiencing occasional low grade fever.

Salient featureHe gave no h/o hemoptysis, hemturia, graveluria, pyuria or bone pain. With these complaints he visited general physician and treated symptomatically and 1 months back after returning to Bangladesh he underwent thorough investigations and later referred to department of urology for further evaluation and management.

Salient featureOn physical examination, vitals are stable, moderately pallor, All accessible lymph nods are not palpable. Jaundice and koilonychia are absent. Abdominal examination revealed; Hard, mild tender, irregular mass at left hypochondriac ,epigastric and lumbar region measuring about 8 cm from left costal margin along MCL. Margins of mass are irregular whose upper limit couldnt be traced and notch is not felt. Insinuation is not possible. Mass moves with respiration and its ballotable .It is dull on percussion with band of resonance and bruit is absent.

Salient featureNo other organomegaly, ascites is absent and hernial orifices are intact.Genitalia, CVS, Respiratory and CNS reveled no abnormalities

Provisional diagnosisLeft Renal massLeft Retroperitoneal extra renal massSplenomegaly

INVESTIGATIONS

CBC

Urine R/M/E

+

USG W/ALtRt

HORMONE ANALYSIS

CT SCAN

CECT

31

After one pint of blood transfusion

OTHER INVESTIGATIONS

INVESTIGATIONS

Hb ElectrophorosisINVESTIGATIONSSerum ferritin = 459 ng/ml (20-300 ng/ml) Montoux test = negative

CLINICAL DIAGNOSISLeft adrenal massLeft Renal mass

ProcedureLeft sided Radical NephrectomyDate:15/09/15

37

PositionUnder G/A patient kept in supine position with bridge beneath left flank and painting and draping done.Incisionmodified chevron / hockey stick (extended left sub costal).Intervening layers are incised along the line of incision

Short chevron, hockey stick, extended left subcostal inision.38

Abdomen cavity reached and findings notedLarge ,hard irregular mass seen subcostally which was pushing pancreas and spleen upward.Mass was adherent to transverse mesocolon and diaphragmMultiple dilated vessels were seen over mass.Operability assessed.

Findings:

39

Dissection was started laterally by dissecting posterior peritoneum along left colon and carried out proximally.Adherent transverse mesocolon was clamped, cut and ligated to free it from mass and hence colon was reflected medially.

Dilated vessels over mass were clamped, cut and ligated which made the mass slightly free.Blunt and sharp dissection carried out along the lower border of mass as there was still confusion regarding origin.As the upper pole of kidney was reached ,a plane was found between mass and kidney hence dissection was continued but after a while plane obliterated hence Nephrectomy was planned, thinking of mass arising from upper pole of kidney

Dissection was carried out laterally and posteriorly

Superiorly a plane was found between panaceas and the mass and hence dissection carried out along the plane .

Medial dissectionRenal hilum was approached and dissection was carried out to identify renal vessels separately.First renal artery and then renal vein were clamped, cut and ligated separately by 1/0 silk.Ureter was identified , clamped, cut and ligated.

While dissecting medially ,another large vessel found reaching kidney which was clamped, cut and ligated (main renal artery).Finally blunt dissection carried laterally and posteriorly to remove specimen

After removing specimen,hot mop was placed and then observed for any bleeding.After ensuring haemostatis 16 fr drain kept in situ Abdomen was closed in layer by 1/0 vicryl and skin closed by stapler

Post operative Follow up

Follow up ----1st PODSubjectivePain abdomen and one episode of fever at evening (101 F)ObjectiveBP: 100/70 mmHg , pulse: 84/minT: 98.4deg F , R/R = 16/minLung; B/L expansion normal, Vesicular breath sound and no added sound,Heart : NADP/A : Mild tenderness, soft, Bowel sound absent and dressing dry.Drain : 270 ml ,reddishUrine Output : 2800ml, clearPlan: I/V fluids and antibiotics, analgesic, Chest physio and limb exercise.

Follow up ----2nd PODSubjectivePain abdomen and one episode of fever at evening (100.4 F)ObjectiveBP: 110/70 mmHg , pulse: 80/minT: 98.6deg F , R/R = 16/minLung; B/L expansion normal, Vesicular breath sound and no added sound,Heart : NADP/A : Mild tenderness, soft, Bowel sound- absent and dressing dry.Drain : 40-50 ml ,blackishUrine Output : 1900ml, clearPlan: I/V fluids and antibiotics, analgesic ensured, Chest physio and limb exercise.

Follow up ----3rd PODSubjectivePain abdomen an one episode of fever at evening (100.6 F)ObjectiveBP: 100/60 mmHg , pulse: 86/minT: 98.2deg F , R/R = 14/minLung; B/L expansion normal, Vesicular breath sound and no added sound,Heart : NADP/A : Mild tenderness, soft, Bowel sound- present and dressing dry.Drain : 20 ml ,blackishUrine Output : 1900ml, clearPlan: sips of water, I/V fluids and antibiotics, analgesic ensured, Chest physio and limb exercise,

Follow up 4th PODSlight painBP: 100/60 mmHg, Pulse = 82/min,Swelling and mild tenderness at cannula siteHeart= NADLung =NADP/A = Soft, mild tender, BS + , Dressing dryDrain : NilPlan: Oral, catheter off, i/v cannula changedCBC, S. Creatinine and CXR-P/A sent

Follow up 5th PODSlight painBP: 110/70 mmHg, Pulse = 86/min,Heart= NADLung =NADP/A = Soft, mild tender, BS + , wound healthyDrain : NilPlan: oral , drain off, check dressing . 6th PODNo fresh complaintBP: 100/60 mmHg, Pulse = 80/min,Heart= NADLung =NADP/A = Soft, mild tender, BS + , dressing dryPlan: Anxiolytics

57

Follow up 7th PODNo fresh complaintBP: 100/70 mmHg, Pulse = 82/min,Heart= NADLung =NADP/A = Soft, mild tender, BS + , dressing dry 8th PODNo fresh complaintBP: 100/60 mmHg, Pulse = 80/min,Heart= NADLung =NADP/A = Soft, mild tender, BS + , dressing dryPlan : Alternate stitch off

58

Final diagnosisAdrenocortical carcinomaStage III (T3N0M0)

Adrenocortical carcinoma

Short review

Introduction and EpidemiologyRare malignancy with an incidence of 0.5 to 2 per million.Bimodal age distribution that peaks in children in the first decade of life and adults in the fourth to fifth decdes of life.Slight female predominance of 1.5 to 2.1Majority of ACCs are sporadic and unilateral loss of TP53 function and increased IGF expression represent late events in the tumorigenesis of sporadic ACC

Clinical CharacteristicsIncidental detection.Tumor-related symptoms Local symptoms: abdominal fullness, back pain, nausea and vomiting.Systemic / metastatic futures: Anorexia, anaemia, asthenia and weight loss and features of metastasis.Hormone secreting ACC (50% to 79% of adult and 90% of pediatric ACCs)

Clinical Characteristics

most common hormone secreted by adrenal cortical carcinomais cortisol

Investigations

Imaging- CT scan/ MRISize Relative indicator of malignancy, with 4% to 5% of tumors less than 4 cm, 10% of tumors larger than 4 cm, and 25% of tumors greater than 6cm.Common radiographic characteristic of ACC on CT imaging include the presence of irregular borders, irregular enhancement, calcifications Necrotic areas with cystic degeneration.Evaluation of spread/metsIVC, Adjacent organs, Liver ,lungs, Bones

In cases of suspicion for venous tumor thrombus,MRI can be an essential tool in detecting the presence of atumor clot and delineating its extent.

66

Role of biopsyFor diagnosing adrenal cortical carcinomas, percutaneous needle biopsy is generally not performed prior to surgical excisiondue to a clinically unacceptable risk of needle-tract seeding (Fassnacht et al, 2004; Schteingart et al, 2005). The primary indication for needle biopsy is in cases of unresectable, locally advanced, or metastatic disease, to confirm the diagnosis prior to systemic medical therapy.

67

Pathologic Evaluation.

Stage I and II tumors are confined to the adrenal gland and are distinguished by a size cutoff of 5 cm. Stage III disease includes tumor extension into adjacent adipose tissue or having regional lymph node involvement. Stage IV disease includes tumors invading adjacent organs and the presence of distant metastatic disease.

ManagementUnfortunately, the majority of patients with adrenal cortical carcinomas present with advanced disease those who do have localized disease are at a high risk of local recurrence and metastatic progression. Treatment of ACC often includes multimodal therapy directed by a team of surgeons, medical oncologists, endocrinologists, and radiation oncologists. Despite aggressive surgical resection, adrenal carcinoma is associated with a high rate (60% to 80%) of recurrent disease

SurgeryComplete surgical excision is essential in the management of adrenal cortical carcinomas En bloc resection of surrounding organs involved with locally advanced disease should be performed whenever possible. Cases of venous tumor thrombus involving the IVC may require vascular bypass techniques, IVC replacement, and/or IVC interruption. In cases of metastatic adrenal carcinoma, Cytoreductive removal of the primary tumor and debulking metastatectomy should be considered if greater than 90% of the disease burden can be removed. Although debulking surgery may not improve survival, it may alleviate tumor-related side effects and facilitate additional therapies . Local or distant disease recurrences, following initial resection, should be considered for surgical excision and have been associated with improved survival in retrospective series.

ApproachSmaller radiographically organ- confined disease can be approached laparoscopically, but should be performed with caution due to the risk of tumor spillage

71

RADIATIONCurrently there is a limited role for radiation for therapy in the treatment of primary adrenal cortical carcinomas;Radiation therapy remains the treatment of choice in Management of bone and CNS metastasisTo decrease local recurrence rates following complete tumor resection, (with reported local recurrence rates of 14% and 79%, with and without adjuvant radiation therapy, respectively )Unfortunately, a significant improvement in disease-free or overall survival was not observed.

MEDICAL THERAPY Mitotane is most commonly used chemotherapeutic agent in the treatment of adrenal cortical carcinoma. Benefit in the adjuvant setting following surgical resection and in patients with metastatic diseaseA significant increase in recurrence-free survival and overall survival was noted in patients receiving mitotane compared with controls

Follow upFollow-up should include CT examination of the chest, abdomen, and pelvis every 3 months for the first 2 years. In patients with evidence of functional tumors, measurement of the initially elevated hormones postoperatively may help to reveal early disease recurrence despite negative radiographic studies. After a disease-free interval of 2 years, surveillance should continue, but the frequency of imaging may decrease.

PrognosisOverall 5-year survival in adrenal cortical carcinomas is poor, ranging from 20% to 47%.