The prognostic influence of the proliferative discordance in metastatic pancreatic neuroendocrine carcinoma revealed by peptide receptor radionuclide therapy: Case report and review of literature

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 tumor...

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Bibliographic Details
Published in:Medicine (Baltimore) 2017-02, Vol.96 (6), p.e6062
Main Authors: Montanier, Nathanaëlle, Joubert-Zakeyh, Juliette, Pétorin, Caroline, Montoriol, Pierre François, Maqdasy, Salwan, Kelly, Antony
Format: Article
Language:English
Subjects:
Carcinoma, Neuroendocrine - drug therapy
Carcinoma, Neuroendocrine - pathology
Clinical Case Report
Female
Humans
Liver Neoplasms - secondary
Middle Aged
Neoplasm Grading
Neoplasm Metastasis
Octreotide - analogs & derivatives
Octreotide - therapeutic use
Organometallic Compounds - therapeutic use
Pancreatic Neoplasms - drug therapy
Pancreatic Neoplasms - pathology
Prognosis
Receptors, Somatostatin - biosynthesis
Citations: Items that this one cites
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Summary: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. We describe a case of a progressive G3 pNEC with huge liver metastases successfully treated with PRRT (Lu DOTATATE). 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. 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.
ISSN:0025-7974
1536-5964
DOI:10.1097/MD.0000000000006062