Clinical=20 features & investigations (excluding radiological aspect ) of = Tumours of=20 Pancreas & related Recent advances

Dilip=20 P. Amonkar, Department of Surgery, Goa Medical College,=20 Goa

=20 Tumours of pancreas are mainly divided into 2=20 subtypes.

Endocrine Pancreas (from islet = of=20 Langerhans)

Exocrine = Pancreas ( Non isler=20 tumours)

Endocrine=20 pancreas tumours are divided as per the type of cell & accordingly = shall be=20 the clinical features.

Some of=20 the tumours secrete more than 1 hormone at different stages of tumour=20 progression.

CELL &n= bsp; &nb= sp;=20 CONTENTS MAJOR &n= bsp; =20 MINOR

1.A=20 cell &n= bsp; &nb= sp; =20 Glucagon &n= bsp; =20 TRH, CCK Endorphins, PYY, Pancreastin

2.B=20 cell &n= bsp; &nb= sp; =20 Insulin =20 &n= bsp; TRH,CGRP,Amylin,Pancreastin,=20 Prolactin

3.D=20 cell &n= bsp; &nb= sp; =20 Somatostatin &n= bsp; =20 Metaencephalon

4.D2=20 cell &n= bsp; &nb= sp; =20 VIP

5.EC &n= bsp; &nb= sp; =20 Substance P &n= bsp; =20 Serotonin

6.G=20 cell &n= bsp; &nb= sp; =20 Gastrin &n= bsp; &nb= sp; =20 ACTH related peptides

7.PP &n= bsp; &nb= sp; =20 Pacreatic &n= bsp; =20 Metaencephalan, PHI

&n= bsp; &nb= sp; &nbs= p; =20 Polypeptides =20

The=20 above mentioned chart shows various cells of pancreas & hormones = secreted by=20 them.

Now let=20 us discuss each major cell with syndrome = separately.

(1) = ; =20 Insulinoma

Insulinoma usually arise from = body &=20 tail of pancreas from B cells.

Clinical=20 features: are due to insulin & catecholamine=20 release.

1.=20 Whipples Triad = Symptoms=20 of Hypoglycemia, Blood Glucose levels of 40 mgs%, relief=20 of

symptoms after IV=20 Glucose.

2.=20 Fatigue., weakness, tremors, sweating.

3.=20 Autonomic Hyperactivity

Mental confusion,=20 irritability, delirium,blurring of vision, stupor, convulsion,=20 coma.

10% of insulinomas are = malignant, 10% are=20 associated with MEN I Syndrome.

Investigations;

During=20 attacks of Hypoglycemia, Serum insulin levels are > 5u/=20 ml.=20
Selective=20 Porto-Venous Sampling for insulin levels in pancreatic venous = tributaries. It=20 has

Accuracy=20 of 75%.

Selective=20 Intra-arterial injection of Calcium in Gastroduodenal, Splenic, Rt, = Hepatic=20 arteries &

Obtain=20 samples for radioimmunoassay of insulin from Rt.Hepatic vein. Here Ca = stimulates=20 release

Of=20 insulin.

(2) = ; =20 Glucagonamas

=20

=20 Glucagonamas arise from A cells. All are malignant & arise = from body=20 & tail of pancreas.

= Clinical=20 features:

Skin=20 Rash (Necrolytic Migratory Erythema ) & unrelenting=20 dermatitis.=20
Diabetes=20 mellitus (mild).=20
Anaemia,=20 weight loss , increase in Glucose levels.

Investigation:

1.Serum=20 levels of Glucagon = 200-2000pg/ml is=20 diagnostic.

(3) = ; =20 Gastrinomas=20 (Zollinger- Ellison Syndrome)

= Gastrinoma=20 is second most common islet cell tumours. It is most symptomatic& = malignant=20 endocrine

Tumour=20 of pancreas. Half of Gastrinomas arise from = duodenum.

Clinical=20 Features:

Presents=20 as virulent peptic ulcer or Gastro esophageal reflux=20 disease.=20
Absence=20 of H. Pylori & no response to H. Pylori = treatment.=20
Present=20 with secretory diarrhoea which is halted by Naso Gastric=20 Aspiration.=20
25%=20 associated with MEN I Syndrome.=20
Pain,severe=20 peptic ulcer symptoms, bleeding & perforation.=20
1/3=20 present as severe Gastro esophageal reflux disease.=20
25%=20 present as MEN I Syndrome. Here it presents as hyperparathyroidism, = Pituitary=20 tumours.

Investigations.

1.Serum=20 levels of Gastrin > 1000 pg / ml.

2.=20 Basal acid output > 15 mEq /hr with Gastric juice pH <=20 2.

3.IV=20 Secretin 2 cu / kg, Serum Gastrin levels > 200 pg / = ml.

4.SRS=20 Somatostatin Receptor Scintigram

Inject Radiolabelled = octreotide 6 mci=20 & body images are taken at 4 & 24 hrs. It will localize=20 gastrinoma

It is most sensitive for = primary &=20 metastatic gastrinomas.

5.Selective=20 porto-venous sampling of gastrin.

6.Direct=20 Endoscopy & Transillumation used for localization of duodenal=20 gastrinomas.

(4) = VIPO=20 mas (Verner = Morrison=20 Syndrome)

It secretes = VIP.

Clinical=20 Features:

1.Watery=20 Diarrhoea (3-5 litres / day) = causes=20 hypovolemia, hypokalemia, Acidosis.

2.=20 Achlorhydria / Hypercalcemia / Hyperglycemia

Present=20 as flushing with rash.

Investigation:

Serum=20 levels of VIP > 225 pg / ml.

(4) = ; =20 Somatostatinomas

It=20 arises in body & tail of pancreas . Also in ectopic sites like upper = small=20 bowel & duodenum.

Clinical=20 Features:

1.Presest=20 as Steatorrhoea , diabetes mellitus, hypochlorhydria , Gall=20 stones.

Investigation:

Serum=20 Levels of somatostatin 100-400 pg / ml.

Exocrine=20 Pancreatic Tumors

Are=20 mainly classified as Benign & Malignant . =

(A)=20 Benign Exocrine Pancreatic Tumours

Various=20 sub types are;

1. =20 Serous=20 Cystadenomas

2. =20 Mucinous=20 Tumours

3. =20 Intradutal=20 papillary mucinous tumours

4. =20 SPEN ( Solid Papillary Epithelial = Neoplasia )=20

Clinical=20 Features;

1.=20 Asymptomatic.

2.=20 Symptoms due to compression on =20 adjacent structures

Jaundice , = Intestinal or=20 Gasric Outlet Obstruction.

Investigation:=20

FNAC=20 : for nature of cystic fluid . To =20 study for glycogen & mucin content & presence of=20 malignant

Cells.

CEA=20 levels of cystic fluid .

(B)Malignant=20 Tumours of Pancreas =20

1.90%=20 are adenocarcinomas. = Various sub=20 types are Colloid Ca , Signet ring Ca ,Adeno = Squamous

Ca ,=20 Anaplastic Ca .

2.=20 Panreatoblastomas

3.=20 Leiomyosarcoma , Liposarcoma ,Lymphymas , = Plasmacytomas.

Clinical=20 features:

1.Painless=20 Progressive Obstructive Jaundice.

2.Pain=20 without jaundice in patients with tumours of body & tail of = pancreas.

3.Anaemia=20 , cachexia of malignancy .

4.Anorexia,=20 nausea, vomiting

Signs:

1.Enlarged=20 gall bladder

=20 2 = Hepatomegaly,Splenomegaly=20 (due to splenic vein thrombosis )

&n= bsp; =20 3. Icterus,Anaemia,

&n= bsp; =20 4. Enlarged lt. Supra-clavicular lymph = nodes.

5.=20 Ascites Nodules in umbilicus.

6.=20 Peripheral=20 edema , Thrombophlebitis (Trousseau sign ) .

Investigation

&n= bsp; =20 :

&n= bsp;=20 1.Liver=20 Funtion Tests

&n= bsp;=20 In Ca Head = Pancreas ^=20 bilirubin , ^ Serum Alk. Phosphatase

&n= bsp;=20 2. Tumour Marker of Ca Pancreas are CEA , CA 19-9 levels=20 .

&n= bsp;=20 3. Protein products of over expressed gene , MIC-I , = synuclein-gamma=20 mesothelian, osteopentin.

= 4.=20 Detection of aberrantly methylated genes in serum.

=20 5.Proteomics, Mass spectrometry band direct analysis of unknown = proteins=20 in serum

&n= bsp;=20 6.. Endoscopy for Ampullary Tumours .

&n= bsp;=20 7. Biopsy = Transduodenal=20 Biopsy of Ca Head Pancreas .

8. Laparoscopy diagnosis=20 .