PS02.175: CLINICAL CHARACTERISTICS AND TREATMENT OF NEUROENDOCRINE CARCINOMA OF THE ESOPHAGUS

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 171-172
Author(s):  
Masaru Morita ◽  
Masaki Kagawa ◽  
Yu Nakaji ◽  
Masahiko Sugiyama ◽  
Daisuke Yoshida ◽  
...  

Abstract Background Neuroendocrine carcinoma (NEC) of the esophagus is a rare and highly aggressive disease; however, an appropriate treatment strategy remains to be established, especially for surgical resection. The objective of this study is to clarify the clinical characteristics of NEC of the esophagus and to determine the optimal surgical strategy. Methods Nineteen patients who were immunohistochemically diagnosed with NEC of the esophagus from 1998 to 2017 were included in this study. The clinical features and therapeutic outcomes were examined. Results 1. Clinical features Sixteen of 19 patients showed protruding or localized type with or without ulceration. Only five patients were negative for both lymph node and organ metastasis and eight cases were positive for metastasis to distant organs and/or distant lymph nodes. 2. Surgical treatment Five patients underwent esophagectomy. Four cases were classified as cStage I (cT1bN0M0); the other case was classified as cStage III (cT3N2M0). The preoperative diagnoses, based on the examination of biopsy specimens, were NEC (n = 2), SCC (n = 1), adenocarcinoma (n = 1), and carcinoma (n = 1). Subtotal and distal esophagostomy were performed in 3 and 2 patients, respectively. Salvage esophagectomy for recurrent disease was performed after definitive chemotherapy in one patient. Anastomotic leakage, which was conservatively healed, developed in one patient. Two patients with histologically-positive node metastasis died at 8 and 13 months, respectively, while another 3 patients (pT1bN0, n = 2; pT2N0, patient, n = 1) are currently alive without any recurrence, at 12, 45 and 72 months after esophagectomy, respectively. 3. Non-surgical treatment Systemic chemotherapy was performed as the main treatment for 13 patients with advanced NEC. The regimens included cisplatin combined with irinotecan or etoposide (n = 10), cisplatin plus 5-fluorouracil (n = 2) and UFT plus irinotecan (n = 1). The MST was 13 months (range, 6–17 months). Conclusion The possibility of NEC should be kept in mind when we encounter protruded tumors of a distinctive shape. Consistent with previous reports, the prognosis of NEC of the esophagus is dismal, irrespective of the administration of systemic chemotherapy. However, surgical resection is considered to be a treatment option for cStage I/II NEC without node metastasis, as a long-term survival after esophagectomy was achieved by some patients. Disclosure All authors have declared no conflicts of interest.

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 102-102
Author(s):  
Marco Antonio Guimaraes Filho ◽  
Flávio Sabino ◽  
Daniel Fernandes ◽  
Carlos Eduardo Pinto ◽  
Luis Felipe Pinto ◽  
...  

Abstract Background Esophageal cancer is the 8th most common cancer in the world. It is an lethal disease, responsible for almost 400.000 deaths by year. Surgical resection is considered the gold standard in esophageal cancer treatment, with a global 15–40% cure rate. In this study, the results of esophageal cancer surgical treatment at Brazilian National Cancer Institute, Abdominal-pelvic Surgical Section, is analyzed. Methods The medical records of 215 patients with esophageal cancer, treated with surgical resection (esophagectomy), between January 1999 and December 2015, were retrospectively studied. The endpoints analyzed in the study were: hospitalization time, operative complications and mortality, and overall survival. Results Esophageal cancer was predominant in male patients; median age was 58 years (27–78). Primary tumor location varied between 7,5 - 41 cm (median 32cm) and tumor extension 1 - 16cm (median 5cm). Median surgical time was 330 minutes (120–720); transhiatal esophagectomy with gastric tube reconstruction was the most used surgical approach. Tumors histopathological types were equaly distributed. ICU (Intensive Care Unit) stay median time was 5 days (1–87) and median hospitalization time was 15 days (5–166). Most common surgical complications were anastomotic leakage (25,5%) and pneumonia (20%), with a surgical morbidity rate of 61,8%. Surgical mortality rate was 12%, with 61% of these cases occuring in the 30 days after surgery. Median 2-year overall survival was 44,3 months. Conclusion Besides the high surgical morbidity, esophagectomy for esophageal cancer remains the standard treatment for patients with ressectable tumors and without clinical contraindications for surgery. Reduction of surgical mortality depends on rigorous patients selection, surgical team expertise and adequate perioperative and postoperative care. Disclosure All authors have declared no conflicts of interest.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3895-3895
Author(s):  
Tingxun Lu ◽  
Yi Miao ◽  
Jia-Zhu Wu ◽  
Qi-Xing Gong ◽  
Jin-Hua Liang ◽  
...  

Abstract Introduction: Based on combination of the three markers (CD10, Bcl6 and MUM1), Hans algorithm could divide DLBCL into two groups (GCB and non-GCB subtype). Although MUM1 is used as a post GC marker, cases with coexpression of CD10 and MUM1 (CD10+ MUM1+, double positive or DP), which was classified as GCB subtype according to Hans algorithm, do exsit. However, the differences of clinical characteristics and prognosis between DP and other GCB (GCB excluding DP) is unknown. Additionally, DLBCL without any positive staining of these three markers (CD10¨C Bcl6¨C MUM1¨C, triple negative or TN) were also noted. These cases, based on Hans algorithm, are classified as non-GCB subtype. However, little was known about the difference between TN cases with GCB or other non-GCB (non-GCB excluding TN). Patients and methods: Immunohistochemistry was appiled in this study with the antibodies as follows. CD10 (clone 56C6, Dako), CD20 (clone L26, Abcam), Bcl6 (clone LN22, Dako), and MUM1 (clone MUM1p, Dako), FOXP1 (clone JC12, Abcam), GCET1 (clone RAM341, Abcam), LMO2 (clone 1A9-1, Santa Cruz), Myc (clone Y69; Abcam, cut-off: 40%) and Bcl2 (clone 124; Dako, cut-off: 50%). Fluorescence in situ hybridization (FISH) was carried out according to manufacturer¡¯s instructions on with the following probes: MYC dual-color, break apart translocation probe (Vysis LSI) and IGH/BCL2 dual-color, and dual fusion translocation probe (Vysis LSI). For cases with MYC translocation, BCL6 dual-color break apart rearrangement probe (Vysis LSI) wasfurtheranalyzed. A total of 601 cases were included in the study. Among these, 306 cases were treated with rituximab based chemoimmunotherapy and well follow up. The median follow up time was 24.3 months (1 to 115.4 months).We analyzed the clinical features of different groups: GCB vs. non-GCB; DP vs. other GCB (GCB*) or non-GCB; TN vs. other non-GCB (non-GCB*) or GCB. Besides, we further analyzed the survival differences among above groups in patients who were treated with rituximab based chemoimmunotherapy and well followed up. Results: The incidence of the DP patients was 13.3% (80/601, 95%CI: 10.7%-16.3%). The DP phenotype was more likely to occur in elderly patients (P =0.0432) and associated with poor PS status (P =0.0192) than GCB* phenotype. Additionally, although not statistically significant, the DP group showed higher incidence of double hit lymphoma (DHL) than GCB* patients (10.7% vs 2.9%, P =0.139). However, the DP group showed more frequent incidence of double expression lymphoma (DEL, Myc and Bcl2 coexpression) (P =0.044), MYC rearrangement (P =0.016) and DHL (P =0.029) than non-GCB group. The incidence of the TN patients was 8.8% (53/601, 95%CI: 6.7%-11.4%). There was no difference in any of clinical characteristics between the TN and GCB group. However, advanced Ann Arbor stage (III or IV) (P =0.0442), better PS status (P =0.0321), high-risk IPI (P =0.0042) Myc expression as well as BCL2 rearrangement were significantly more common in the non-GCB* group than the TN group. The DP group showed similar overall survival (OS) (median OS: both not reached, P =0.3650) and progression-free survival (PFS) (median PFS: 47.0 vs. 32.7 months, P =0.0878) with the non-GCB group while the TN group showed similar OS (median OS: both not reached, P =0.9278) and PFS (median PFS: both not reached, P =0.9420) with the GCB group (Fig 1-2). Conclusion: Cases in the DP and TE group might hinder the intrinsic power of Hans algorithm in other studies. More detailed classification of DLBCL based on Hans algorithm may help to identify patients with distinct clinical characteristics and prognosis, thereby improving the stratification of patients for risk-adjusted therapies. Disclosures No relevant conflicts of interest to declare.


2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 95-96
Author(s):  
Han-Yu Deng ◽  
Yidan Lin

Abstract Background Esophageal neuroendocrine carcinoma (NEC) is a rare malignant tumor, and it is rarely reported in large cohort studies. Therefore, the prognostic factors of esophageal NEC were not well established. In this study, we tried to analyse a large cohort of esophageal NEC patients aiming to investigate its possible prognostic factors. Methods Patients who underwent esophagectomy for esophageal NEC in our department from January 2007 to June 2015 were identified and analyzed. Both univariate and multivariate analyses were applied to identify potential prognostic factors. Results A total of 72 patients were identified for analysis. After a median follow-up time of 54.8 months, the median survival time of those patients was 21.5 months. One-year, three year, and five-year survival rates were for all those patients were 75.0%, 33.4%, and 28.4% respectively. In the univariate analysis, TNM stage (P = 0.001), lymph node metastasis (P = 0.008), and adjuvant therapy (P = 0.001) were found to significantly influence the overall survival time of those patients; while in multivariate analysis, only TNM stage (P = 0.018) and adjuvant therapy (P = 0.002) were found to be independent prognostic factors. Conclusion Esophageal NEC was extremely rare with poor prognosis. TNM stage and adjuvant therapy are the independent prognostic factors of esophageal NEC. Disclosure All authors have declared no conflicts of interest.


2019 ◽  
Vol 37 (7_suppl) ◽  
pp. 531-531
Author(s):  
Lucas W. Dean ◽  
Nathan Colin Wong ◽  
Shawn Dason ◽  
Sumit Isharwal ◽  
Mark Donoghue ◽  
...  

531 Background: The incidence of secondary somatic malignancy (SSM) arising from teratoma is increased in 2 settings; late relapse and primary mediastinal nonseminomatous germ cell tumors (PM-NSGCT). Here, we report the clinical features and outcomes of patients with SSM in the setting of PM-NSGCT. Methods: Between 1985 and 2018, 29 patients with PM-NSGCT and SSM who had sufficient clinical follow-up to evaluate outcome were identified. Clinical and pathologic parameters were reviewed. The Kaplan-Meier method was used to estimate overall survival (OS) from time of SSM diagnosis and the log rank test to compare estimates. Results: Median age was 28 years (range 18-59) and all patients were male. Most presented with local symptoms (n=24, 83%), elevated tumor markers (n=26, 90%), and disease isolated to the mediastinum (n=25, 86%). A total of 39 SSM histologies were present in the 29 cases, with 8 (28%) having 2 (n=6) or 3 (n=2) SSM histologies; 25 (86%) also had viable non-teratomatous GCT in the mediastinal mass. Sarcoma was found in all 29 cases including rhabdomyosarcoma (n=15), angiosarcoma (n=6), sarcoma NOS (n=5), spindle cell (n=4), PNET (n=3), and other (n=3). Non-sarcoma histologies (n=1 each) included AML, SCC, and neuroblastoma. Most patients received GCT-directed chemotherapy followed by an attempt at surgical resection (90%). With a median follow-up of 2 years for survivors, median OS was 1.8 years (95% CI 0-3.9 years), with 18 patients succumbing to disease. Complete surgical resection was achieved in 23 men (79%) and was associated with superior OS (3.1 vs. 0.3 years, p=0.005). At relapse or progression, 11 received SSM histology-directed and 7 GCT-directed chemotherapy with no difference in OS (1.3 vs. 1.2 years, p=0.993). 7 patients developed SSM in the form of leukemia, a finding associated with significantly inferior OS (0.3 vs. 3.0 years, p=0.009). Conclusions: Sarcoma is the predominant SSM histology associated with PM-NSGCT and portends a poor prognosis even with initially localized disease. Complete resection following chemotherapy is critical to achieving long-term survival whereas SSM in the form of leukemia portends especially poor outcome.


2016 ◽  
Vol 34 (4_suppl) ◽  
pp. 84-84
Author(s):  
Joseph C Poen ◽  
Joseph W Poen ◽  
David S. Gullion

84 Background: A subset of patients with local and regionally confined carcinoma of the esophagus (ES) or esophagogastric junction (EGJ) will not benefit from esophagectomy or esophagogastrectomy. Examples include medical inoperability and confined disease that is technically unresectable (T4 and/or N2-3). With that in mind we undertook to optimize the non-operative component of ES and EGJ cancer treatment, with surgery reserved for selected cases (medically fit, limited stage, suspicion of residual or recurrent disease). Methods: Eligible patients had AJCC stage T2-4,N0-3,M0 squamous or adenocarcinoma of the esophagus or esophagogastric junction. Medically inoperable patients were included if fit for ECF and chemoradiation (CRT) and regional disease could be encompassed within extended radiation fields. E (50mg/m2), C (60mg/m2) were given days 1,22 with F (200mg/m2) days 1-42 by protracted infusion. Subsequent CRT was C (100mg/m2) days 49,70 and F (200mg/m2) days 49-91 with 54 Gy in 30 fractions over 6 weeks. Complete responders by PET and biopsy received 2 additional cycles of ECF. Results: Between 2003 and 2015, 40 patients with non-metastatic squamous (n = 13) and adenocarcinoma (n = 27) signed informed consent. Most had clinical stage T3-4 (78%), positive regional lymph nodes (58%) and/or were felt to be inoperable due to medical comorbidities or advanced disease (50%). Dose reductions were allowed for grade II-III toxicity during ECF (13%) and CRT (33%). There was one treatment-related death (3%). At a median follow-up of 5 years, 10 patients (25%) have undergone esophagogastrectomy for persistent or recurrent disease. Median overall survival (OS) is 46 months. Survival at 1,3,5 and 7 years is 82%, 53%, 44% and 35% respectively. Conclusions: In local/regionally confined ES and EGJ cancer, long term survival with acceptable toxicity can be achieved with neoadjuvant ECF and CRT followed by selective surgical resection/salvage. This strategy appears to be appropriate for medically inoperable and technically unresectable cases, and can be considered for operable cases as well.


2015 ◽  
Vol 16 (13) ◽  
pp. 5433-5438 ◽  
Author(s):  
Shigenori Kadowaki ◽  
Azusa Komori ◽  
Daisuke Takahari ◽  
Takashi Ura ◽  
Seiji Ito ◽  
...  

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