ICL 12.4: Pancreatic Cancer and Pre-Malignant Conditions of Pancreas Flashcards

1
Q

how common is pancreas cancer?

A

9th in women

10th in men

however, it’s the 4th most lethal cancer in both genders which isn’t good…and it’s projected to be 2nd in the next decade

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2
Q

why does pancreatic cancer have a high mortality?

A

the incidence matches the mortality so not a lot of people survive…

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3
Q

what is the race association with pancreatic cancer?

A

predominance in african americans

multifactorial: health care access, PCP, earlier diagnosis, genetics

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4
Q

what is the mean cancer diagnosis age?

A

70

but it can happen in younger people too

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5
Q

what are the risk factors for pancreas cancer?

A
  1. tobacco*
  2. alcohol
  3. obesity
  4. DM and type I*
  5. non-O blood type
  6. H. pylori
  7. chronic pancreatitis*
  8. IPMN*
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6
Q

which genes are associated with pancreatic cancer?

A
  1. KRAS (80-95%)
  2. SMAD4 (55%)
  3. TP53 (50%)
  4. TP16
  5. BRACA2

pancreatic adenocarcinoma type has an average of sixty-three genetic aberrations at time of diagnosis…

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7
Q

how long does it take pancreatic cancer to develop?

A

~17 year latency from initial somatic mutation to acquisition of metastatic potential

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8
Q

what is the location of pancreatic cancer lesions?

A

75% occur in the head or neck region

15-20% occur in the body

5-10% in the tail

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9
Q

what’s the clinical presentation of a pancreatic cancer in the head of the pancreas?

A

the tumor is more likely to be near the bile duct so it can cause:
1. painless jaundice –> you’re obstructing the bile duct and it’ll back up into the liver and it’ll deposit all over your body and you get jaundice

  1. dark urine and light stool
  2. nausea
  3. unintended weight loss
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10
Q

what’s the clinical presentation of a pancreatic cancer in the body of the pancreas?

A

the tumor is more likely to be near the celiac nerve so tumors in this location can cause:

  1. back pain (pancreas is a retroperitoneal organ so pain will be referred to the back)
  2. upper abdomen pain
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11
Q

what’s the clinical presentation of a pancreatic cancer in the tail of the pancreas?

A

the tumor presses on the stomach and small intestines

if the tumor is toward the middle of the pancreas it can also be near the celiac nerve

it can cause:
1. lower abdomen pain

  1. back pain
  2. nausea
  3. loss of appetite
  4. unintended weight loss
  5. pain after eating
  6. indigestion
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12
Q

what clinical considerations should you keep in mind with pancreatic cancer?

A
  1. always be suspicious of painless jaundice, especially when accompanied by weight loss or risk factors (tobacco, diabetes, pancreatitis, etc.)
  2. nausea and vomiting may be the result of duodenal obstruction
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13
Q

what is the detection challenge with pancreatic cancer?

A

pancreatic adenocarcinomas remain small for most of their development

symptoms often do not result until the tumor becomes large (causing mass effect) or spreads to adjacent organs

conventional imaging (CT and MRI) has poor sensitivity for small (< 1 cm) lesions…you can’t even see them on imagining before they hit 1 cm

biomarker CA 19-9 correlates with tumor size but is neither sensitive nor specific for small tumors

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14
Q

how do you workup a suspected pancreatic cancer?

A

pancreatic cancer is a tissue diagnosis –suspicious masses should be biopsied, typically by endoscopic ultrasound

histology helps guide therapy

dvaluate locoregional lymphadenopathy

all patients with locoregional or metastatic disease will receive (neoadjuvant) chemotherapy; surgery isn’t really an option at that point

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15
Q

how do you image the pancreas?

A
  1. ultrasonography: transabdominal vs endoscopic
  2. triphasic CT scan with IV contrast (for spread and invasion and to see which blood vessels are associated with it)
  3. endoscopic retrograde cholangiopancreatography (ERCP)
  4. MRI / MRCP for high-resolution anatomical details
  5. PET/CT to detect distant metastases
  6. diagnostic laparoscopy
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16
Q

based on the location of the pancreatic tumor, what surgery do you do?

A

uncinate/head/neck lesion = pancreaticoduodenectomy = Whipple –> way harder; involves the liver, bile duct, duodenum, pancreas and stomach

body/tail = distal pancreatectomy and splenectomy

17
Q

what is the pathology of pancreatic cancer?

A

cancers arise from both the exocrine and endocrine portions of glands

  1. exocrine cancers are way more common (95%) = acinar and duct tissue
    ex. pancreatic ductal adenocarcinoma or acinar cell carcinoma
  2. endocrine = involves islets of Langerhans and causes hormone secretion
    ex. neuroendocrine tumors
18
Q

what is the prognosis for pancreatic adenocarcinoma?

A

5 year survival is <10%

5 year survival is 20% after pancreatic resection –> to maximize survival make sure you do node resection, small tumors less than 3 cm, negative resection margins, well differentiated tumors

19
Q

how do you treat pancreatic adenocarincoma?

A
  1. inherently resistant to chemotherapeutic regimens
  2. 5-FU and gemcitabine have been the most active agents
  3. radiation therapy alone has little impact, often combined with radiosensitizing agents 5-FU or gemcitabine.

most high-volume centers advocate for neoadjuvant chemoradiation for all potentially operable disease or for patients with locally advanced disease – upon finishing this therapy patient is restaged and if disease still appears resectable, resection is undertaken

20
Q

what is the 20-20 rule with pancreatic adenocarcinoma?

A

20% of patients present with resectable pancreas cancer

of those that get a resection, they have a 20% 5 year survival

21
Q

what is a local advanced pancreatic tumor?

A

direction invasion of surrounding visceral organs and vessels

this would include things like mets to the liver, peritoneum or lung

treat with chemotherapy/chemoradiotherapy

22
Q

when would you stage someone with pancreatic cancer?

A

staging laparoscopy for patients at high risk for occult metastatic disease

you would stage them if:

  1. tumors in the pancreatic body or tail
  2. large or anatomically extensive
  3. high CA 19-9 level
  4. equivocal CT findings of metastasis
  5. clinical findings suggestive of advanced disease (e.g., marked weight loss, ascites)
23
Q

what are pancreatic neuroendocrine tumors?

A

tumors involving the islets of langerhans!

alpha cells = glucagon

beta cells = insulin

delta cells = somatostatin

G cells = gastrin

24
Q

how do you image pancreatic neuroendocrine tumors?

A

gallium-68 DOTATATE scan

fancy PET scan where they give patients octreotide linked to radioactive isotope which binds to somatostatin receptors on neuroendocrine tumor

if you biopsy and find a NET you can assess for metastasis with this scan

25
Q

what is the biomarker for a pancreatic neuroendocrine tumor?

A

chromogranin A

26
Q

what are the symptoms of pancreatic neuroendocrine tumors?

A
  1. diarrhea

2. cramping

27
Q

what is a glucagonoma?

A

rare tumor of alpha cells – malignant potential

treat with surgical resection

presents as weight loss and the D’s:
1. dermatitis (necrolytic migratory erythema)

  1. diabetes (hyperglycemia due to constantlyhigh glucagon levels)
  2. diarrhea
  3. DVT
  4. depression
28
Q

what is an insulinoma?

A

most common pancreatic neuroendocrine tumor; 90% are benign –> treat with surgical resection

insulin-secreting tumor of B-cells

hypoglycemic symptoms = hunger, nervous, sweating, lethargy, confusion, coma

Whipple’s triad:
1. hypoglycemia (glucose < 55)

  1. symptoms of hypoglycemia
  2. correction of symptoms with glucose administration
29
Q

what are the lab results in a patient with an insulinoma?

A
  1. hypoglycemia
  2. elevated insulin
  3. elevated C-peptide
30
Q

what is a somatostatinoma?

A

rare tumor of delta cells, usually in the head of the pancreas –> malignant!!

treat with resection

symptoms are due to inhibition of secretion by somatostatin = gallstones, steatorrhea, weight loss

31
Q

what is Zollinger-Ellison syndrome?

A

aka gastrinomas = tumors secreting gastrin that form in the pancreas or duodenum (“gastronome triangle”) –> this leads to excess acid production

elevated serum gastrin level (>1000) is the most sensitive and specific diagnostic factor

presents as:
1. peptic ulcer disease

  1. diarrhea
  2. steatorrhea
  3. B12 malabsorption
32
Q

how do you treat Zollinger-ellison syndrome?

A

total gastrectomy is not necessary for managing patients with ZES because effective medical therapy (proton pump inhibitors) for the acid hypersecretion can prevent ulcer disease

surgical removal of the gastrinoma could eliminate the source of excessive gastrin release and present the chance for cure - this is frequently not accomplished

33
Q

what is IPMN?

A

intraductal papillary mutinous neoplasm

they are cysts lined by columnar epithelium which secrete mucus

they have malignant potential!!!

suggestive indications: high cyst CEA, high serum CA 19-9, large size

34
Q

what is a mutinous cystic neoplasm?

A

very female predominant 20:1

they are thick cyst wall (“eggshell calcification”) with thick viscous fluid (“ovarian-like stroma”) and septations

elevated cyst fluid CEA

malignant potential, resection recommended

associated with KRAS mutation in 20% of low-grade, 33% of intermediate-grade, and 90% of high-grade MCNs

35
Q

what are serous cyst adenoma?

A

benign lesions; tend to be left alone unless they get too big

they are csyst lined with flat epithelium, contains serous fluid (glycogen rich cells*)

they typically occupy body and tail of pancreas

calcified central scar