Tumors of the Exocrine Pancreas

Dr.CSBR.Prasad, M.D.

Ductal Adenocarcinoma Most common neoplasm of the pancreas 5th leading cause of cancer death in the US

28,000 new cases (and nearly as many deaths) per year

Most common between 60 & 80 years of age

Slight male predominance (1.6:1)

Ductal Adenocarcinoma – Risk Factors

Cigarette smoking High fat diets Chronic pancreatitis Diabetes mellitus Chemical (carcinogen) exposure

Ductal Adenocarcinoma – Genetic Risk Factors

Hereditary breast and ovarian cancer BRCA2 families

Hereditary pancreatitis (50X) Peutz-Jeghers Syndrome HNPCC

Ductal Adenocarcinoma- Clinical Features Painless jaundice Abdominal pain Weight loss Migratory thrombophlebitis

TROUSSEAU SIGN

Ductal Adenocarcinoma- Molecular Genetics K-RAS point mutations

(present in >90% of pancreatic cancers) Tumor suppressor gene mutations

P53 (present in 60-80%) p16

HER/2-neu overexpression (present in ~70%)

Ductal Adenocarcinoma- Gross Pathology

Most occur in the head of the pancreas (60%) 15% in the body 5% in the tail 20% diffuse

Poorly circumscribed, grey-white, firm masses

Can infiltrate outside the pancreas into other organs

Ductal Adenocarcinoma- Histopathology Arise from ductal (glandular) epithelium Typically moderately to poorly differentiated

adenocarcinoma Perineural and angiolymphatic invasion common Almost always associated with chronic

pancreatitis Histologic variants

Adenosquamous Carcinoma Anaplastic Carcinoma Undifferentiated carcinoma with osteoclast-like giant

cells

Ductal Adenocarcinoma - Gross

Moderately differentiated

Ductal Adenocarcinoma - Micro

Moderately differentiated

Ductal Adenocarcinoma - Micro

Ductal Adenocarcinoma - MicroPoorly differentiated

Ductal Adenocarcinoma - MicroPerineural invasion

NERV

E

Ductal Adenocarcinoma- Treatment Surgical resection

Pancreaticoduodenectomy (Whipple procedure) for tumors in the head

Distal pancreatectomy for tumors in the body and tail Prognosis

3 months without treatment 10-20 months with Whipple

Medical Combined 5-FU-based chemotherapy and radiation

therapy Palliative (supportive) therapy

Sister Mary Joseph’s nodules TROUSSEAU SIGN

TROUSSEAU SIGN

Other Exocrine Tumors * Intraductal Papillary Mucinous Neoplasm

(IPMN) Mucinous Cystic Neoplasm Microcystic Adenoma Solid-pseudopapillary Neoplasm Acinar Cell Carcinoma Pancreatoblastoma* Not covered in Robbins

Intraductal Papillary Mucinous Neoplasm (IPMN) Mucinous neoplasm that arises in and

causes cystic dilatation of the main (large) pancreatic ducts

Can progress to invasive adenocarcinoma

Intraductal papillary mucinous neoplasm - IPMN

Intraductal papillary mucinous neoplasm - IPMN

IPMN

Mucinous Cystic Neoplasm Form unilocular or multilocular cysts Often within the tail of the pancreas No connection to the ductal system Usually occurs in women Can progress to invasive adenocarcinoma

Mucinous Cystic Neoplasm

Acinar Cell Carcinoma

Tumor of the pancreatic acinar cells Not ductal-derived

Normal

Pancreatoblastoma Really Really Really Really Really Rare Occurs in children (<10y)

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