The prognostic influence of the proliferative discordance in metastatic pancreatic neuroendocrine carcinoma revealed by peptide receptor radionuclide therapy: Case report and review of literature.
BACKGROUND
Pancreatic neuroendocrine tumors (pNET) are rare slowly growing tumors with a high metastatic potential. Peptide receptor radionuclide therapy (PRRT) with radiolabeled analogues has been developed as a new tool for the management of metastatic well-differentiated (grade 1 and 2) neuroendocrine tumors expressing somatostatin receptor (SSTR2). Chemotherapy is the mainstay in the management of grade 3 (G3) unresectable pancreatic neuroendocrine carcinoma (pNEC). To date, no study has evaluated the efficacy of PRRT in such tumors.


METHODS
We describe a case of a progressive G3 pNEC with huge liver metastases successfully treated with PRRT (Lu DOTATATE).


RESULTS
Complete remission was obtained for 3 years. Indeed, the mitotic index was low (as G2 tumors) but with a very high Ki-67 index (45%-70%). Such discordance between the proliferative markers should consider the use of PRRT before chemotherapy in unresectable metastatic G3 tumors expressing SSTR2.


CONCLUSIONS
This case supports the hypotheses highlighting the heterogeneity of G3 pNEC. The latter should be subdivided into 2 distinct categories: proliferation-discordant (well differentiated) and concordant (poorly differentiated) NEC. PRRT could be suggested for the former group before the conventional chemotherapy.
CHU Clermont-Ferrand, Service d'endocrinologie, diabétologie et maladies métaboliques CHU Clermont-Ferrand, Service d'anatomopathologie CHU Clermont-Ferrand, Service de chirurgie et oncologie digestive Centre de Lutte Contre le Cancer Jean Perrin, Service de radiologie, Clermont-Ferrand UMR CNRS 6293, INSERM U1103, Université Clermont-Auvergne, Génétique Reproduction et Développement, Aubiere Centre de Lutte Contre le Cancer Jean Perrin, Service de Médecine nucléaire, Clermont-Ferrand, France.
Nathanaëlle
N
Montanier
Juliette
J
Joubert-Zakeyh
Caroline
C
Pétorin
Pierre François
PF
Montoriol
Salwan
S
Maqdasy
Antony
A
Kelly
D018278:Carcinoma, Neuroendocrine / Q000188:drug therapy* / Q000473:pathology; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary; D008875:Middle Aged; D060787:Neoplasm Grading; D009362:Neoplasm Metastasis; D015282:Octreotide / Q000031:analogs & derivatives* / Q000627:therapeutic use; D009942:Organometallic Compounds / Q000627:therapeutic use*; D010190:Pancreatic Neoplasms / Q000188:drug therapy* / Q000473:pathology; D011379:Prognosis; D017481:Receptors, Somatostatin / Q000096:biosynthesis
D009942:Organometallic Compounds; D017481:Receptors, Somatostatin; C447941:lutetium Lu 177 dotatate; D015282:Octreotide