Clinical outcomes of locally advanced esophageal neuroendocrine carcinoma treated with chemoradiotherapy

Abstract

Background: Neuroendocrine carcinoma (NEC) arising from the esophagus (EsoNEC) is extreme rare, accounting for approximately 1% of esophageal cancer.Even for localized NEC, multidisciplinary approach including chemotherapy is recommended in treatment guidelines because of its high rates of systemic recurrence.However, it is controversial whether adding surgery or radiotherapy is appropriate local treatment for EsoNEC. There have been few reports regarding the clinical outcomes of definitive chemoradiotherapy (dCRT) for EsoNEC. The purpose of this study was to clarify the survival outcome of patients with locally advanced EsoNEC treated with dCRT. Methods: Clinical outcomes, feasibility, and prognostic factors of patients with locally advanced EsoNEC treated with radiotherapy (60 Gy/30 fraction) in combination with platinum plus etoposide (CE-RT) or cisplatin plus 5-fluorouracil (CF-RT) at the National Cancer Center Hospital from 2001 to 2017 were retrospectively analyzed. Results: A total of 22 patients were identified as the subjects of this study. The overall response rate and clinical complete remission rate in all patients were 86.4% and 77.3%, respectively. The median progression-free survival and median survival time in all patients were 12.7 and 37.5 months, associated with a 5-year survival rate of 45.4%. Patients treated with CE-RT experienced more hematological adverse events, especially in neutropenia (≥grade 3) and febrile neutropenia(≥grade 3), but achieved more long-term progression-free survival than with CF-RT. Conclusions: Definitive chemoradiotherapy can be considered as an important treatment option for locally advanced esophageal neuroendocrine carcinoma. K E Y W O R D S chemoradiotherapy, esophageal neuroendocrine carcinoma, etoposide, multidisciplinary treatment, platinum 596 | HONMA et Al.

Clinical characteristics, prognostic factors, and survival trends in esophageal neuroendocrine carcinomas: A population‐based study

“…Our study showed that both surgery and chemotherapy could significantly increase cancer‐specific survival of ENEC patients. According to previous studies, many centers recommend that chemotherapy should be the cornerstone of the treatment of ENEC 8,23 . A study from the Memorial Sloan‐Kettering Cancer Center suggested that for small cell ENEC patients treated with induction chemotherapy followed by consolidative chemoradiation can achieve long‐term survival, while the contribution of surgery was unclear 24 .…”

“…According to previous studies, many centers recommend that chemotherapy should be the cornerstone of the treatment of ENEC. 8 , 23 A study from the Memorial Sloan‐Kettering Cancer Center suggested that for small cell ENEC patients treated with induction chemotherapy followed by consolidative chemoradiation can achieve long‐term survival, while the contribution of surgery was unclear. 24 A recent nationwide study from Japan revealed that there was no significant difference in survival between the operative or non‐operative groups of stage I and II ENEC patients, and for stage III and IV ENEC, chemoradiotherapy led to significantly better prognosis.…”

Incidence, treatment, and survival analysis in esophageal neuroendocrine carcinoma population

“…Chemotherapy, such as irinotecan and platinum, is also among the first-line use. By now, multidisciplinary treatment, including adjuvant chemoradiotherapy, serves as an appropriate option, although ineffectiveness has been reported in large-cell ENEC concerning clinical outcomes ( 28 , 29 ). Besides, specific inhibition of molecules like CDK4/6 by using monoclonal antibody seems to be a promising strategy indicated by some cases ( 30 ).…”

Prognostic value of pretreatment contrast-enhanced computed tomography in esophageal neuroendocrine carcinoma: A multi-center follow-up study

“…Currently, the same AJCC guidelines for esophageal squamous cell carcinoma /adenocarcinoma are used for diagnosis and treatment[ 20 ]. Previous studies have demonstrated that surgery can extend the survival of esophageal NEC patients[ 21 ]. However, recent studies have shown that surgery may benefit only patients with early stage disease[ 6 , 13 ].…”