Small Cell Carcinoma of the Esophagus

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Abstract

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Abstract

Background and Purpose:

Small cell carcinoma of the esophagus (SCEC) is a rare subtype of esophageal cancer for which optimal treatment is unknown. We analyzed the impact of treatment factors on outcome in patients with nonmetastasized SCEC.

Methods:

Patients with a histologically confirmed SCEC without distant metastases were analyzed in a nationwide multicenter retrospective cohort. All patients received radiotherapy as part of curative treatment between January 2000 and December 2014. Details on treatment and outcome were retrieved from individual charts. Cox regression analysis was used to determine prognostic factors for survival.

Results:

Fifty-eight patients were analyzed. Median survival was 16 months (95% confidence interval, 11-21 mo). Infield recurrences occurred in 25%, distant metastases in 45%, and brain metastases in 12%. In total, 63% of patients developed a recurrence. Most recurrences (67%) occurred within 1 year. In univariable analyses an increased number of chemotherapy cycles (>3) and lower radiotherapy doses (<45 Gy) were associated with improved survival. T-stage, N-stage, treatment period, type of chemotherapy, prophylactic cranial irradiation, and age were not associated with survival. In multivariable analyses, only the number of chemotherapy cycles was associated with better survival (hazard ratio, 0.78; P=0.006).

Conclusions:

SCEC recurs frequently at distant sites after definitive chemoradiotherapy and usually within 1 year after curative treatment. With a dose of 45 to 50 Gy, infield recurrence rate was low. We found a relationship between number of received chemotherapy cycles and survival with best results obtained after at least 4 cycles of chemotherapy.

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Most cited references 19

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Prophylactic cranial irradiation for patients with small-cell lung cancer in complete remission. Prophylactic Cranial Irradiation Overview Collaborative Group.

R Stephens, A. Gregor, C Le Péchoux … (1999)

Prophylactic cranial irradiation reduces the incidence of brain metastasis in patients with small-cell lung cancer. Whether this treatment, when given to patients in complete remission, improves survival is not known. We performed a meta-analysis to determine whether prophylactic cranial irradiation prolongs survival. We analyzed individual data on 987 patients with small-cell lung cancer in complete remission who took part in seven trials that compared prophylactic cranial irradiation with no prophylactic cranial irradiation. The main end point was survival. The relative risk of death in the treatment group as compared with the control group was 0.84 (95 percent confidence interval, 0.73 to 0.97; P= 0.01), which corresponds to a 5.4 percent increase in the rate of survival at three years (15.3 percent in the control group vs. 20.7 percent in the treatment group). Prophylactic cranial irradiation also increased the rate of disease-free survival (relative risk of recurrence or death, 0.75; 95 percent confidence interval, 0.65 to 0.86; P<0.001) and decreased the cumulative incidence of brain metastasis (relative risk, 0.46; 95 percent confidence interval, 0.38 to 0.57; P<0.001). Larger doses of radiation led to greater decreases in the risk of brain metastasis, according to an analysis of four total doses (8 Gy, 24 to 25 Gy, 30 Gy, and 36 to 40 Gy) (P for trend=0.02), but the effect on survival did not differ significantly according to the dose. We also identified a trend (P=0.01) toward a decrease in the risk of brain metastasis with earlier administration of cranial irradiation after the initiation of induction chemotherapy. Prophylactic cranial irradiation improves both overall survival and disease-free survival among patients with small-cell lung cancer in complete remission.

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Small-cell carcinomas of the gastrointestinal tract: a review.

H. Tang, David Klimstra, D Kelsen … (2004)

To improve our understanding of the entity of small-cell carcinoma (SmCC) of the gastrointestinal (GI) tract. A MEDLINE search was done, using the terms "small cell carcinoma" or "oat cell carcinoma" combined with "gastrointestinal" or with any of the GI sites, for the period 1970 to 2003. The 138 eligible reports identified in this way were reviewed for clinical data. To date, approximately 544 cases of GI SmCC have been reported. The disease represents 0.1% to 1% of all GI malignancies, with the esophagus being the most common primary site. A majority of patients present with overt distant metastases. Systemic symptoms are common; ectopic hormonal secretion may occur. By light microscopy, GI SmCCs are essentially indistinguishable from primary pulmonary SmCC. The presence of non-SmCC components is common. Data from molecular analysis of the disease has identified some similarities to pulmonary SmCC. Chemotherapy represents the main treatment option, with modest impact on survival. In locoregional disease, the literature suggests that treatment be initiated using chemoradiotherapy and then, if metastatic disease is still excluded, surgical resection be considered. The disease is highly aggressive, and survival is in the range of several weeks for untreated patients and of 6 to 12 months for those receiving therapy. SmCC of the GI tract is a rare and lethal disease. Although there are many similarities to pulmonary SmCC, some differences between the two entities are suggested. While chemotherapy can achieve significant palliation, surgery may have a potential impact on long-term survival of patients with locoregional disease.

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Primary small cell carcinoma of the esophagus: a review of the literature with emphasis on therapy and prognosis.

A Biete, B Farrus, F Casas … (1997)

Few studies of patients with esophageal small cell carcinoma (SCC) have been conducted. Choice of treatment remains controversial. The authors analyzed data on 199 evaluable esophageal SCC patients, selected from among 230 patients found in the literature, and a data extraction form that recorded 11 features was completed. To allow for the evaluation of prognostic factors that influenced survival, the patients were grouped according to limited stage (LS), which was defined as disease confined to the esophagus, or extensive stage (ES), which was defined as disease that had spread beyond locoregional boundaries. Univariate and multivariate analyses were performed. Treatment was categorized as either local or local with systemic; for the ES cases, the categories were defined as treatment versus no treatment. The tumor site was described in 178 cases (89%). Mean tumor size was 6.1. Pure SCC was found in 137 cases (68.8%), whereas 62 cases (31.2%) showed mixed SCC; 93 (46.7%) were LS, whereas 95 (47.7%) were ES. In 11 cases (5.5%), the stage was not determined. There was a significant difference in survival between patients with LS and those with ES (P 60 years, the median survival was 6 months), tumor size (for those with tumors 5 cm, the median survival was 4 months), and type of treatment (with local plus systemic treatment, the median survival was 20 months, whereas with local it was 5 months). In multivariate analysis, tumor size (P = 0.007) and type of treatment (P < 0.001) were shown to be independent predictive variables. Esophageal SCC is an aggressive type of tumor. This study shows that there are significant differences between LS and ES and that in LS, both tumor size and type of treatment are possible prognostic factors.

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Author and article information

Journal

Journal ID (nlm-ta): Am J Clin Oncol

Journal ID (iso-abbrev): Am. J. Clin. Oncol

Journal ID (publisher-id): COC

Title: American Journal of Clinical Oncology

Publisher: Lippincott Williams & Wilkins

ISSN (Print): 0277-3732

ISSN (Electronic): 1537-453X

Publication date (Print): June 2019

Publication date (Electronic): 22 April 2019

Volume: 42

Issue: 6

Pages: 534-538

Affiliations

[* ]Department of Radiation Oncology, Academic Medical Center Amsterdam

[∥ ]Department of Radiation Oncology, The Netherlands Cancer Institute

[## ]Department of Radiation Oncology, VU University Medical Center, Amsterdam

[† ]Radiotherapiegroep, Deventer

[‡ ]Department of Radiation Oncology, University Medical Center Groningen, University of Groningen, Groningen

[§ ]Instituut Verbeeten, Tilburg

[¶ ]Department of Medical Oncology, Leiden University Medical Centre, Leiden

[# ]Department of Radiotherapy, Erasmus MC Cancer Institute, Rotterdam

[** ]Department of Radiotherapy, Isala Kliniek, Zwolle

[†† ]Department of Radiotherapy, Radboud UMC, Nijmegen

[‡‡ ]Radiotherapy Institute Friesland, Leeuwarden

[§§ ]Department of Radiation Oncology, Haaglanden Medical Center, The Hague

[∥∥ ]Department of Radiotherapy, Catharina Hospital Eindhoven

[¶¶ ]Department of Radiotherapy, Noordwest Ziekenhuisgroep, Alkmaar

[*** ]MAASTRO Clinic Maastricht, GROW School for Oncology and Developmental Biology, Maastricht University Medical Centre, Maastricht

[††† ]Department of Radiotherapy, University Medical Center Utrecht, Utrecht, The Netherlands

Author notes

Reprints: Paul M. Jeene, MD, Department of Radiation Oncology, Academic Medical Center Amsterdam, Meibergdreef 9, B0-107, 1105 AZ Amsterdam, The Netherlands. E-mail: p.m.jeene@ 123456amc.uva.nl .

Article

Accession ID: 00007

DOI: 10.1097/COC.0000000000000546

PMC ID: 6554014

PubMed ID: 31021827

SO-VID: 3d5d588e-6dd4-4790-a80a-cf1b8fefd007

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0/

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Keywords: small cell carcinoma,esophagus,chemotherapy,radiotherapy,chemoradiotherapy,outcome,survival

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Keywords: small cell carcinoma, esophagus, chemotherapy, radiotherapy, chemoradiotherapy, outcome, survival

Small Cell Carcinoma of the Esophagus : A Nationwide Analysis of Treatment and Outcome at Patient Level in Locoregional Disease

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Abstract

Abstract

Background and Purpose:

Methods:

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Related collections

Cancer Immunotherapy

Most cited references 19

Prophylactic cranial irradiation for patients with small-cell lung cancer in complete remission. Prophylactic Cranial Irradiation Overview Collaborative Group.

Small-cell carcinomas of the gastrointestinal tract: a review.

Primary small cell carcinoma of the esophagus: a review of the literature with emphasis on therapy and prognosis.

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