Case report. Kováčová Martina Comenius University in Bratislava Slovakia Faculty of medicine

Transcription

1 Case report Kováčová Martina Comenius University in Bratislava Slovakia Faculty of medicine

2 Past medical history 38 years old male patient No past medical or surgical history prior to presentation No medications No known allergies to medications Family history mother died of breast cancer (43 years); father died of lung cancer (48 years) Social history Non-smoker, occasional alcohol use

3 History of Present Illness Late 2005: Patient presented with weight loss, swelling weakness, frequent stools (10x/day) Physical examination - the patient was jaundiced, with yellow skin and sclera, abdominal exam was notable for a palpable gallbladder (Courvoisier sign), neither the liver nor spleen were palpably enlarged

4 Laboratory evaluation Serum electrolytes and creatinine were within normal limits Liver function tests were suggestive of obstructive jaundice (total bilirubin 93.4 μmol/l [0 25], conjugated bilirubin 76.9 μmol/l [0 5], alkaline phosphatase 7.91 μkat/l [ ], alanine aminotransferase 6.01 μkat/l [ ] and aspartate aminotransferase 5.23 μkat/l [ ])

5 Investigations Cross-sectional imaging revealed a pancreatic head tumor with multiple liver metastases Biopsy of liver metastasis demonstrated...??? (no adenocarcinoma)

6 Investigations Biopsy of liver metastasis demonstrated a welldifferentiated pancreatic neuroendocrine tumor (PNET), Ki67 index 2% What further investigations do we need??? 1. Biochemical evaluation...??? 2. Radiologic evalution...???

7 Biochemical evaluation Chromogranin A (CgA)121 ng/ml (< 100 ng/ml) Neuron-specific enolase (NSE) 14.9 ug/l ( ug/l) TSH, cortisol, metanephrine and normetanephrine, gastrin, insulin, ACTH, prolactin, calcitonin, PTH - within normal limits Calcitonin pg/ml (normal <10 pg/ml)

8 Radiologic evaluation Head/neck CT: no thyroid pathology Octreotide Scan (intravenous injection of radiolabeled somatostatin analogs, used for the localozation of the primary and distant metastases): a high density of somatostatin receptors in the head of pancreas and metastases in liver; no signal in thyroid PET-CT with 18 FDG: no pathology

9 Consultation Surgical consultation: surgical removal was not indicated (surgery should be performed only if 90% of the tumor mass can be successfully removed). Biopsy of liver metastasis demonstrated a welldifferentiated pancreatic neuroendocrine tumor (PNET), Ki67 index 2%. Conclusion: Inoperable, well-differentiated, slow-growing, calcitonin-secreting pancreatic neuroendocrine tumor

10 Follow-up Somatostatin analogues are effective in the control of symptoms in functioning pancreatic neuroendocrine tumors that express somatostatin receptors 2006 started treatment with octreotide Clinical response: Stool decreased to 2x/day Biochemical response: Calcitonin decreased to 612 pg/ml (from pg/ml) Radiologic response: no change in tumor size

11 Follow-up (continued) 2008 given 2x PRRT 90 Yttrium: peptide receptor radionuclide therapy used in inoperable metastatic disease in tumors with high somatostatin receptor expression 2009 CT abdomen/pelvis demonstrated reduction of pancreatic tumor mass (30% reduction) stent was placed in the ductus hepatocholedochus

12 Partial remission of biochemical markers and tumor volume 2006: 2011: Calcitonin pg/ml CgA 121 ng/ml CEA 6.62 ug/l NSE 14.9 ug/l CT: pancreatic head tumor 54x50 mm with liver metastases Calcitonin pg/ml CgA 101 ng/ml CEA 4.72 ug/l NSE ug/l CT: pancreatic head tumor 37x35 mm with liver metastases

13 Today Now without complaints, stool frequency has further decreased to 1x/day Therapy continues with somatostatin analogs: octreotid (Sandostatinom LAR) 20mg 1x/month 2011: successful ascent of Kilimanjaro (the highest mountain in Africa) 2012: successful ascent of Aconcagua (the highest mountain in the Americas)

14 Consultation Calcitonin is a small peptide hormone typically secreted by thyroid C-cells. As such, calcitonin is considered a marker for medullary thyroid cancer, but a number of physiologic and pathologic conditions have been associated with hypercalcitonemia. PNETs that secrete calcitonin are exceedingly rare, but in a patient with a pancreatic mass, should be considered in the differential diagnosis

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16 Thank you for your attention