Patient with a rare clinical syndrome related to a pNET. Sequencing treatment for progressive disease

Patient with a rare clinical syndrome related to a pNET. Sequencing treatment for progressive disease

by

Professor Kjell Öberg,

M.D., Ph.D.

Dept. of Endocrine Oncology, University Hospital, Uppsala, Sweden

A 58 year old male, who presented 10 years ago with abdominal pain. Initial evaluation:

CT-scan                5 cm tumor in the pancreatic tail, multiple focal lesions in the liver

FNAB     Well differentiated NET (G2)

SRS         Grade 3 uptake in the pNET and liver met.

Biochemistry     CgA 5.2 nmol/L (N<4)

                PP 195 pmol/L (N<100)

1. Further diagnostic work-up
2. Surgery
3. Liver embolization
4. Chemotherapy
5. Everolimus + SMS

• Primary surgery + liver resection was suggested but the patient refused.
• Chemotherapy streptozotocin + 5FU plus Octreotide LAR, 20 mg q4w

Disease progression after 1 year:

• PEG-Intron A 80 mcg/w was added
• Chemotherapy had to be discontinued after 2.5 years (impaired renal function)

Radiology 10/2005: Stable disease

• CgA 10.5 nmol/L
• PP 300 pmol/L

2006       Hypercalcemia occurred for the first time (s-Ca 2.95 mmol/L) (N 2.15-2.50)

                Ionazide Ca 1.43 (N 1.3); hypophosphatemia (p 0.4; 0.64 mmol/L (0.74-1.25); low PTH 0.27 pmol/L (1.10-6.90); PTHrP 84 pmol/L (N<2)

                Radiology: Tumor growth in the liver

                Octreotide increased to 30 mg q 3w

•                 CgA 45 nmol/L
•                 PP 425 pmol/L

2008       PEG-Intron A was discontinued (mental depression)