e15616 Background: Primary gastro-entero-pancreatic poorly differentiated neuroendocrine carcinomas (GEP-PDNECAs) are highly aggressive neoplasms with a very poor prognosis. This study was conducted to evaluate the immuno-morphological spectrum of GEP-PDNECA, and patient survival with systemic platinum and etoposide therapy. Methods: Under an IRB-approved protocol, clinico-pathologic data were collected on 68 adult patients with GEP-PDNECA who had undergone biopsy / resection at MCC or outside institution. Data sources: Pathology archives, consultation files, tumor registry and social security index. All available slides were reviewed and tumors were histologically sub-typed. Subsequently, clinico-pathologic data and patient survival were analyzed. Results: Patients: 41 M/27 F. Age: 25–76 yrs (mean 42 yrs). Sites: Colo-rectum 39, pancreas 19, small intestine (SI) 4, stomach 3, colon/SI/pancreas 3. 63 of 68 (93%) patients presented with lymph node/distant metastases. Of 68 tumors 37 (54%) were classified as small cell carcinoma (SCCA), 16 (24%) large cell carcinoma (LCCA), 5 (7%) mixed small and large cell (MSLCCA) and 10 (15%) poorly differentiated carcinoma with neuroendocrine features (PDCA-NEF). Tumors were positive for chromogranin in 38/65 (55%), synaptophysin in 62/67 (92%), and CD56 in 17/21 (81%) cases. One marker was positive in 22/68 (32%), 2 in 40/68 (59%) and all 3 were positive in 9/68 (13%) cases. Fifty eight of 68 (85%) patients were treated with platinum and etoposide. Overall survival at 1, 3 and 5 years was 85%, 40% and 24% respectively. Patient survival was independent of age (r= -0.1022), sex (r= -0.909) and histologic subtype (r= - 0.1028) (p= 0.128) but was related to distant metastases (r=0.306; p=0.0383). Conclusions: Diagnosis of GEP-PDNECA can be based on histo-morphologic features and expression of neuroendocrine markers. Synaptophysin was the most sensitive marker; however, a panel of 2 or 3 neuroendocrine markers (Syn, Cg and CD56) may be more useful to avoid under-diagnosis of GEP-PDNECA, especially in the metastatic setting. Although survival of GEP-PDNECA patients following platinum and etoposide in our series was relatively favorable, there is need for novel therapies to improve patient survival. No significant financial relationships to disclose.
Prolonged survival with erlotinib followed by afatinib in a caucasian smoker with metastatic, poorly differentiated large cell carcinoma of the lung: a case report
