Multimodality management of a polyfunctional pancreatic endocrine carcinoma with markedly elevated serum vasoactive intestinal polypeptide and calcitonin levels.
We present an unusual case of a 52-year-old woman with severe, uncontrollable, refractory diarrhea attributable to pancreatic endocrine carcinoma (ECA) with markedly elevated serum vasoactive intestinal polypeptide (VIP) and calcitonin levels. After initial correction of fluid and electrolyte abnormalities, the patient was treated with high-dose octreotide. Shortly thereafter, due to the intractable nature of her diarrhea, she underwent cytoreductive hepatic surgery. The pancreatosplenectomy specimen showed a poorly differentiated ECA of the distal pancreas, immunoreactive for synaptophysin, CD56, and S100 protein, with morphologically similar hepatic and lymph node metastases. Postoperatively, her diarrhea improved, along with decline in serum VIP and calcitonin levels. Systemic chemotherapy with etoposide and cisplatin did not result in any radiographic and biochemical improvement. Having radiologically stable disease with depot-octreotide and short-acting octreotide (Sandostatin), she was subjected to peptide receptor radiotherapy with [177Lu-DOTA0,Tyr]octreotate (LuTate) that resulted in marked clinical and biochemical improvement, along with dramatic reduction in the number and size of hepatic metastases. In summary, this is a unique case of metastatic VIP- and calcitonin-secreting pancreatic ECA with dramatic sustained clinical, biochemical, and objective tumor response to peptide receptor radionuclide therapy.
Nasir
Aejaz
A
Department of Interdisciplinary Oncology, Neuroendocrine Cancer Research Group, H. Lee Moffitt Cancer Center and Research Institute, University of South Florida, Tampa, FL 33612, USA. aejaz.nasir@moffitt.org
Gardner
Nancy M
NM
Strosberg
Jonathan
J
Ahmad
Nazeel
N
Choi
Junsung
J
Malafa
Mokenge P
MP
Coppola
Domenico
D
Kwekkeboom
Dik J
DJ
Teunissen
Jaap J M
JJ
Kvols
Larry K
LK
D002116:Calcitonin / Q000097:blood*; D003131:Combined Modality Therapy; D003967:Diarrhea / Q000209:etiology / Q000628:therapy; D005260:Female; D006573:Heterocyclic Compounds, 1-Ring / Q000627:therapeutic use; D006801:Humans; D008187:Lutetium / Q000627:therapeutic use; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000097:blood* / Q000150:complications / Q000628:therapy*; D009384:Paraneoplastic Endocrine Syndromes / Q000097:blood* / Q000150:complications / Q000628:therapy*; D010456:Peptides, Cyclic / Q000627:therapeutic use; D019275:Radiopharmaceuticals / Q000627:therapeutic use; D014660:Vasoactive Intestinal Peptide / Q000097:blood*
C496695:177Lu-DOTA-lanreotide; D006573:Heterocyclic Compounds, 1-Ring; D010456:Peptides, Cyclic; D019275:Radiopharmaceuticals; D014660:Vasoactive Intestinal Peptide; D008187:Lutetium; D002116:Calcitonin