title: Targeted Therapies Provide Treatment Options for Poorly Differentiated Pancreatic Neuroendocrine Carcinomas.
abstract: Poorly differentiated pancreatic neuroendocrine carcinoma (PD pNECs) is a rare disease that has a poor prognosis and is treated with systemic chemotherapy as the standard of care. We present 6 cases of chemo-naïve patients diagnosed with PD pNECs who refused systemic chemotherapy and received targeted therapies with sunitinib (37.5 mg/day, 5 patients) or the mammalian target of rapamycin (mTOR) inhibitor everolimus (10 mg/day, 1 patient) as the first-line treatment. We evaluated the drugs' toxicities and survival. The median age of the patients was 55 years (4 males, 2 females, functioning tumor in 1 of 6 patients). The median of the Ki67 index was 45% (range 20-80). Targeted therapies were combined with somatostatin analogues in 4 of 6 patients (30 mg Sandostatine LAR monthly). Toxicities (acute and late) were manageable and no toxicities necessitated cessation of treatment. All patients had progression-free survival during the 15-month treatment and an overall survival of more than 2 years after diagnosis. Even though this is a small cohort of selected patients, we conclude that sunitinib or everolimus are both feasible and safe and have encouraging results of efficacy as first-line therapies for PD pNEC.
authors.affiliation: Department of Medical Oncology, Paoli-Calmettes Institute, Marseille, France.
authors.forename: Marine
authors.initials: M
authors.lastname: Gilabert
authors.forename: Young Soo
authors.initials: YS
authors.lastname: Rho
authors.forename: Petr
authors.initials: P
authors.lastname: Kavan
mesh_terms: D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use; D018278:Carcinoma, Neuroendocrine / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D002454:Cell Differentiation / Q000187:drug effects; D000068338:Everolimus / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007211:Indoles / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D058990:Molecular Targeted Therapy; D010190:Pancreatic Neoplasms / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D011758:Pyrroles / Q000009:adverse effects / Q000627:therapeutic use*; D000077210:Sunitinib; D058570:TOR Serine-Threonine Kinases / Q000037:antagonists & inhibitors
chemical_list: D000970:Antineoplastic Agents; D007211:Indoles; D011758:Pyrroles; D000068338:Everolimus; C546842:MTOR protein, human; D058570:TOR Serine-Threonine Kinases; D000077210:Sunitinib