Pancreatic%20Neuroendocrine%20Neoplasm%20(pNEN)%20case

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Pancreatic%20Neuroendocrine%20Neoplasm%20(pNEN)%20case

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Pancreatic Neuroendocrine Neoplasm (pNEN) case. Endocrinology Unit. Pathophysiology Department. Laikon General Hospital. Case Presentation: Dr. Chatzellis Eleftherios ... – PowerPoint PPT presentation

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Title: Pancreatic%20Neuroendocrine%20Neoplasm%20(pNEN)%20case

1
Pancreatic Neuroendocrine Neoplasm (pNEN) case

Case Presentation
Dr. Chatzellis Eleftherios MDIntern in
Endocrinology

2
Case History - 2001

58 year old male patient
2001 abdominal pain
CT scan 5cm tumor pancreatic tail
Multiple focal lesions in the liver
FNAB (liver lesion) ? Well Differentiated NEN
grade 2 (ki67 4)
CgA 5,2 nmol/l (lt4), other markers negative
No secretory symptoms (non-functioning pNEN)
SRS (Octreoscan) avid uptake (Krenning score
3) in both primary pNEN and liver metastases

3
Case History
HE stain
ki67
Chromogranin A
4
Case History 2001-2005

Sweden (Uppsala) ? Suggested surgery to reduce
tumor burden and local complications
Patient refused
Chemotherapy (Streptozotocin 5-FU)
Somatostatin analogues (SSAs)
1 year later (2002) DISEASE PROGRESSION (PD)
Addition of pegylated INF-a
Chemotherapy was discontinued after 2,5 years
(mild renal impairment)
2002-2005 STABLE DISEASE (SD)
CgA 10,5 nmol/l (lt4)

5
Case History 2006-2008

2006 Hypercalcemia occurred for the first time
Ca 11,8 mg/dl (8,5 - 10,1)
P 1,98 mg/dl (2,5 - 4,5)
PTH 2,54 pg/ml (10 - 65)
PTHrP 84 pmol/l (lt2)
CgA 45 nmol/l (lt4)
Imaging DISEASE PROGRESSION (PD)
Increased SSAs dosage peg-INFa
Normalization Ca
2006-2008 STABLE DISEASE (SD) - Biochemical
control

Humoral Malignancy-associated Hypercalcemia (PTHrP
- related)
6
Case History 2008-2009

2008 peg-INFa discontinued (depression)
2008 Hypercalcemia (12,9 mg/dl)
DISEASE PROGRESSION (PD)
2008-2009 177Lu-DOTATATE x5 cycles (25.6 GBq)
Increased SSAs dosage
Addition of pasireotide 1200µg bid
?Ca 10,7 mg/dl, CgA115 nmol/l, PTHrP140
pmol/l
2009 DISEASE PROGRESSION (PD)
biochemical relapse (Ca 11,8 mg/dl)

7
Case History 2009-2010

Temozolomide Capecitabine (CAP-TEM)
2009-2010 STABLE DISEASE (SD)
Biochemical control of Ca required additional
treatment
SOM230 and SSAs
Forced Diuresis
Prednisolone 40mg/d
Biphosphonates (i.v. zolendronate 4mg monthly)
Cinacalcet 90mg/d
SIDE-EFFECTS Proximal myopathy - muscle atrophy,
patient immobilization (wheel chair), severe
Diabetes Mellitus (100 IU insulin/d)

8
Case History 2010

Tumor burden reduction (cytoreduction) necessary
Biochemical control (Ca)
Reduce treatment side-effects
OPTIONS
Surgery Patient still denied
PRRT Already performed (GFR, marrow toxicity)
RF Ablation Not applicable due to large liver
lesions
Embolization (TAE/TACE)
Pre-embolization evaluation Portal vein
thrombosis
Selective approach (embolization of small
branches of hepatic artery)

9
Case History - TAE
Post-embolization
Pre-embolization
10
Post-embolization status
Case History - TAE

TMZ Capecitabine
SSAs
Pasireotide 1200mcg/d
Prednisolone 40 mg/d
Zolendronate 4mg/m
Insulin treatment

TMZ Capecitabine
SSAs at ½ dosage
X
Prednisolone 8 mg/d
X
X

Ca 10,2 mg/dl
CgA 50 nmol/l
PTHrP 38 pmol/l

11
Case History 2010

6 months after TAE
Ca 13,2 mg/dl
DISEASE PROGRESSION (PD)
Painful lump on left thigh

T2
T1
12
Case History 2010
Bone scintigraphy Tc 99m
13
Case History 2010

BONE METASTASES vs BENIGN LESION
Bone Biopsy ? Brown tumor (in the context of
prolonged PTHrP action on bone)
New liver lesion biopsy ? ki67 4, IHC () for
PTHrP

14
Case History 2010-2012

Temozolomide (200mg/m2 Days 1-5 q4w)
Bevacizumab (10 mg/kg q2w) Everolimus (10mg/d)
SSAs
Ca 8,8 mg/dl
Temozolomide and Bevacizumab D/C after 6 months
due to thrombocytopenia
2010-2012 STABLE DISEASE (SD)

15
Case History 2012-2014

2012 DISEASE PROGRESSION (PD)
Ca9,34 mg/dl, PTHrP 50 pmol/l
Sunitinib 37,5 mg/d SSAs
6 months later Ca 6,5 mg/dl !!!
2012-2014 STABLE DISEASE (SD)

April 2014 Patient deceased (hepatic
encephalopathy - malnutrition)
16
Pegylated INFa
Pasireotide
BEV-TEM
STZ 5FU
Everolimus
Sunitinib
PRRT
CAP-TEM
SSAs
Embolization

Ca
CgA
PTHrP
2006
2006
2009
2009
2009
2010
2012
2012
2014
2005
2001
2008
2010
2010
17
Case synopsis

WD pNEN grade 2 Stage IV (liver metastases)
Change of functional status during disease course
Rare PTHrP secretion (paraneoplastic syndrome)
Even more rare brown tumor due to PTHrP
Employment of several treatment agents/modalities
to achieve both tumoral and biochemical control
Importance of cytoreductive interventions and
novel molecular targeted therapies in controlling
secretory symptoms/syndrome
Long survival despite metastatic disease at
presentation (application of different
therapeutic modalities)