MUCINOUS CYSTIC NEOPLASM

OF PANCREAS

BENSY ISAAC
 INTRODUCTION
Definition:
A cyst forming epithelial neoplasm
Columnar mucin producing epithelium
No communication with duct system
Ovarian type subepithelial stroma

• Almost exclusively in women

 OLD CLASSIFICATION

• Noninvasive
MCN with low grade dysplasia
MCN with intermediate grade dysplasia
MCN with high grade dysplasia

• Invasive
MCN with associated invasive carcinoma
 WHO 2019
• MCN with low grade dysplasia
• MCN with high grade dysplasia
• MCN with associated invasive
carcinoma
 Epidemiology
• Rare
• 8% of surgically resected cystic lesions of
pancreas
• F:M 20:1
• Mean age 40-50yrs ( range 14-95 yrs)
• Invasive: 10yrs older
 Location
• Body and tail of pancreas (>95%)
 Clinical features
• <3cm – found incidentally
• Abdominal mass
• Compression to adjacent structures
• Rare: invasion to bile duct, stomach, colon,
peritoneal cavity, liver metastasis

• New onset DM
 Serum Markers
• Serum tumor and cystic fluid markers:
CEA & CA 19-9*

• Markers very high: invasive component

• Cyst fluid Amylase activity: low*
 WHEN TO SUSPECT
• Suspect: cystic lesion in body-tail of young
middle aged woman ( no h/o pancreatitis)

• Imaging: sharply demarcated lesion with one

or more thick walled large loculations
 Features s/o invasion

 Large size
 Irregular thickening of cyst wall
 Mural nodules
 Papillary excrescences
 Gross

• Single spherical mass

• Fibrous pseudocapsule of variable thickness
• Calcifications- occasionally

• Size: 2-35cm ( avg 6-10 cm)

 GROSS
C/S:
• Uniloculated or multiloculated
• Content: thick mucin or mixture of mucin &
haemorrhagic-necrotic material

• Internal surface- smooth & glistening

• High grade: papillary projections/ mural nodules
• Infiltrating CA: invade adjacent organs
GROSS
 Spread
• Regional peripancreatic lymphnodes
• Liver
 Microscopy
2 distinct components:
• Epithelial lining
• Underlying ovarian type stroma
 MICROSCOPY
• Lining: tall columnar mucin producing cells
PAS positive Diastase resistant

• Pyloric
• Gastric foveolar
• Small & large intestinal
• Squamous differentiation
MICROSCOPY
 MCN with low-grade dysplasia

• Minimal to mild architectural & cytological

atypia
• Slight increase in size of basally located nuclei
• Absent mitoses
 MCN with low-grade dysplasia
• Mild- moderate architectural and cytological
atypia
• Papillary projections or crypt like invaginations
• Cellular pseudostratification
• Crowding of enlarged nuclei
• Occasional mitoses
 MCN with High grade dysplasia

• s/f architectural & cytological atypia

• Papillae with irregular branching & budding
• Nuclear stratification
• Loss of polarity
• Pleomorphism, prominent nucleoli
• Frequent mitoses; atypical
 MCN with Invasive carcinoma

• Invasive component- focal

• Extensive histological examination needed
• Trapped non-neoplastic glands: stromal
desmoplasia

• Resemble: infiltrating ductal adeno carcinoma

 Rare variants
• Adenosquamous carcinoma
• Undifferentiated carcinoma
• Undifferentiated carcinoma with osteoclast
like giant cells
 MICROSCOPY
Stroma :
• Densely packed spindle shaped cells
• Sparse cytoplasm
• Round or elongated nuclei
 MICROSCOPY
• Variable degree of luteinization
• Frequency decrease as dysplasia increase
• Fibrotic & hypocellular
• Some foci- appearance of corpus albicans
• Stroma predominates- solid mass

• Adjoining pancreatic tissue-fibrous atrophy-

obstruction of main duct
 Ultrastructure
• Columnar epithelial cells on thin basement
membrane
• Mucin granules
• Well developed microvilli

• Ovarian type stroma: myofibroblastic

differentiation
 Molecular Pathology
• Activating mutation in codon 12 of KRAS gene

• Alterations in TP53, CDKN2A and SMAD4

tumor suppressor genes- invasive
 Immunohistochemistry
• CK 7,8,18 and 19
• CEA, EMA
• MUC5AC, DUPAN-2 and CA19-9

• Scattered goblet cells- MUC2

• Neuroendocrine cells- chromogranin positive
 IHC
• Increasing atypia- p53 postivity
• Non invasive- SMAD4(DPC4) postive,
MUC1 neg
• Invasive: loss of expression for SMAD4
MUC1 positive
CA 19-9 reactivity in atypical
epithelial cells
 IHC contd…
Stroma:

Smooth muscle actin(SMA)

PR (60-90%)
ER (30%)
PR
POSITIVITY

ER
POSITIVITY
 Differential diagnosis
• Other cystic neoplasms of pancreas

• Pseudocyst of pancreas
• Intraductal papillary mucinous neoplasm
 TYPES OF PANCREATIC CYST
Injury and Inflammation-Related Cysts
Neoplastic cysts
Congenital cysts
Miscellaneous cysts
INJURY & INFLAMMATION RELATED CYSTS

 Pseudocyst

 Paraduodenal wall cyst

 Infection- related cysts (parasitic cyst-
Echinococcus-etc.)
 NEOPLASTIC CYST

• A. Ductal lineage; Mucinous type

- IPMN
- MCN
- Cystic change in ordinary ductal adenoca &
other invasive Cas
- Retention cyst with dysplasia
 NEOPLASTIC CYST

• B. Ductal lineage; Serous type

- Serous cystadenoma – Microcystic & oligocystic variants

- vHL syndrome-associated pancreatic cysts
- Serous cystadenocarcinoma
• C. Ductal lineage; NOS

- Intraductal tubulopapillary neoplasm

 NEOPLASTIC CYST

• D. Endocrine lineage
- Cystic pancreatic NET
• E. Acinar Lineage
- Acinar cell cystadenoma
- Acinar cell cystadenocarcinoma
- Cystic/ Intraductal adenocarcinoma
 NEOPLASTIC CYST

• F. Endothelial lineage
- Lymphangioma
• G. Mesenchymal Lineage
- Schwannoma
• H. Undetermined lineage
- SPN
• I. Other
- Mature Cystic Teratoma
 CONGENITAL CYST

• Duplication Cyst
• Duodenal diverticula
• Associated with inherited polycystic diseases
• AV malformation
 MISCELLANEOUS CYST

• Lymphoepithelial cyst
• Squamoid cyst of pancreatic ducts
• Epidermoid cyst within intrapancreatic accessory spleen
• Cystic hamartoma
• Endometriotic Cyst
• Secondary tumours
Thank you