Pancreatic neuroendocrine tumors (pnets)-case conference

1. Pancreatic neuroendocrine
tumors (PNETs)
Dr Priyanka Vishwakarma
10th April 2014

2. • 2% to 3% of all pancreatic tumors
• 4th–6th decades of life
• No gender predilection
• The overall prognosis and long-term
survival for PNET patients are far better
than for patients with ductal pancreatic
cancer
• Furthermore, 19% of all pancreatic lesions
incidentally detected by computed
tomography (CT) are PNETs

3. By definition, PNETs express
neuroendocrine markers—for example,
synaptophysin , neuron-specific enolase
(NSE) , and/or chromogranin A (CgA) —
namely, proteins associated with the
secretory apparatus of endocrine cells.
PETs often have been referred to as islet
cell tumors; however, that term is no
longer acceptable because of evidence
that they do not arise from the islets of
Langerhans, but rather from ductal
pluripotent stem cells

4. Functional VS Non Functional
NF –PNET: hormones may be biologically
inactive, of insufficient quantity to cause
symptoms, or of a type that does not lead
to a clinical syndrome (eg, pancreatic
polypeptide)
Insulinoma is the most common syndromic
ICT

5. Summary of PNET characteristics [15, 122].

6. Majority well differentiated and slow growing
Malignant –
Gastrinoma,
Somatostatinoma
,Glucogonoma
MEN 1-associated with gastrinoma

7. PNETs may also be associated with genetic
syndromes such as
• MEN-1-multifocal duodenal and/or pancreatic
lesions- (young onset, two organ manifestations,
family history) should lead to tests
• von Hippel-Lindau (VHL) disease-The majority
(91%) show true cysts, whereas the minority
(12%) develop PNETs
And Rarely with
• neurofibromatosis 1 (NF-1), and tuberous
sclerosis (TSC)

8. • The American Joint Committee on Cancer
has published the 7th edition of the AJCC
Cancer Staging Manual, which for the first
time incorporates pancreatic
neuroendocrine tumors in the same
staging system as pancreatic exocrine
tumors

9. • Primary Tumor (T)
• TX Primary tumor cannot be assessed.
• T0 No evidence of primary tumor.
• Tis Carcinoma in situ.
• T1 Tumor limited to the pancreas, ≤2 cm in greatest dimension.
• T2 Tumor limited to the pancreas, >2 cm in greatest dimension.
• T3 Tumor extends beyond the pancreas but without involvement of
the celiac axis or the superior mesenteric artery.
• T4 Tumor involves the celiac axis or the superior mesenteric
artery (unresectable primary tumor).
• Regional Lymph Nodes (N)
• NX Regional lymph nodes cannot be assessed.
• N0 No regional lymph node metastasis.
• N1 Regional lymph node metastasis.
• Distant Metastasis (M)
• M0 No distant metastasis.
• M1 Distant metastasis

11. Imaging
• PETs that are smaller than 0.5 cm are defined
as microadenomas
• well circumscribed
• displace, rather than invade, adjacent structures
• Blush-Early enhancing lesion in in arterial phase
• Small-homogenous:
• Large-hetrogenous-necrotic
Functional PNETS-Small in size

12. NF PNETS
• Large
• Calcifications
• venous tumor thrombus
Atypical findings
Can be cystic with peripheral enhancement
Can be exophytic,
Can enhance on venous phase
Metastasis
• Liver Metastasis-Nodes-enhancing
• Bone mets-sclerotic
• Lung Mets

13. pancreatic ductal obstruction and dilatation
have not been considered typical features
of neuroendocrine tumors, except in those
cases with very large masses that obstruct
the duct secondary to mass effect.

15. Follow up on Imaging
• RECIST 1.1 criterion for evaluating response to
treatment.(Response Evaluation Criteria in Solid
Tumors)
• >1 cm
• Not for necrotic,confluent lesions.not for
ascites,pleural fluid,bone mets,peritoneal or
leptomeningeal disease.
• maximum of five total (and two per organ,
maximum)
• short axis of >15 mm are considered
measurable

16. RECIST 1.1
• CR (complete response) = disappearance
of all target lesions
• PR (partial response) = 30% decrease in
the sum of the longest diameter of target
lesions
• PD (progressive disease) = 20% increase
in the sum of the longest diameter of
target lesions
• SD (stable disease) = small changes that
do not meet above criteria

17. • PR(WHO)====PR (Recist)
• PD Rescist will require greater increase in
volume than WHO PD
• EORTC instrument

18. References
1)http://www.ajronline.org/doi/full/10.2214/AJR.12.8627
2)Pancreatic Endocrine Tumors : Radiologic Clinicopathologic
Correlation, RadioGraphics 2010 30:6, 1445-1464
2)The Oncologist May 2009 vol. 14no. 5 456-467
3)AJCC ,7Th edition
4) Multi–Detector Row CT of Pancreatic Islet Cell Tumors ,
RadioGraphics 2006