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Ann Thorac Surg 2002;74:1924-1927
© 2002 The Society of Thoracic Surgeons
a Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, India
b Department of Pathology, Tata Memorial Hospital, Mumbai, India
Accepted for publication July 10, 2002.
* Address reprint requests to Dr Pantvaidya, Department of Surgical Oncology, Tata Memorial Hospital, Mumbai 400012, India.
e-mail: docgouri{at}yahoo.com
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Abstract |
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METHODS: We performed a retrospective review of all patients with small cell carcinoma of the esophagus diagnosed at the Tata Memorial Hospital between 1985 and 2001. We retrieved and analyzed data regarding demographic details, diagnosis, staging, type of treatment, and overall survival.
RESULTS: Eighteen patients with a mean age of 62 years (range 48 to 80 years) diagnosed as having small cell carcinoma of the esophagus were analyzed. The group included 13 men and 5 women. Seven of the 18 patients (39%) presented with metastatic disease including 5 patients (28%) with liver metastases. Four patients were treated with surgery, with or without chemotherapy or radiotherapy. Three patients were treated with combination chemoradiotherapy, 2 patients with chemotherapy alone, and 5 patients with radiotherapy alone. Four patients with advanced disease and poor general condition were not offered any treatment. The overall median survival of our patients was 6 months. Patients treated with surgery and chemotherapy had a better overall survival.
CONCLUSIONS: Small cell carcinoma of the esophagus should be regarded as a systemic disease with a high distant failure rate. Treatment strategies hence must incorporate systemic chemotherapy along with radical surgery or radiotherapy as part of a multimodality approach.
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Introduction |
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TopAbstractIntroductionPatients and methodsResultsCommentReferences |
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Patients and methods |
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TopAbstractIntroductionPatients and methodsResultsCommentReferences |
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Diagnostic criteria for esophageal small cell carcinoma were defined as (a) definite growth in the esophagus on upper gastrointestinal endoscopy, (b) histopathologic evidence of small cell carcinoma on endoscopic esophageal biopsy, (c) normal lungs on computed tomography scan of the thorax, and (d) normal tracheobronchial tree on fiber optic bronchoscopy. The last two criteria were used to rule out secondary involvement of the esophagus from primary small cell lung cancer. Treatment was decided taking into consideration the stage of disease, the general condition and performance status of patients, the ability of patients to tolerate therapy, and the main symptom requiring palliation in patients with advanced disease.
The data were tabulated with special reference to symptoms, site, exact pathology, radiologic studies, treatment given, outcome, and follow-up. Patients who did not have follow-up after initial presentation were excluded from survival analysis. Data were analyzed using the SPSS-10 software (SPSS Inc, Chicago, IL) and survival analysis was carried out by Kaplan–Meier survival curves.
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Results |
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Of 2 patients treated with radical chemoradiotherapy, 1 was lost to follow-up and the other died after 10 months. One patient, treated with radical radiotherapy alone, developed a supraclavicular recurrence and died after 9 months. One patient treated with palliative chemoradiotherapy, because of a supraclavicular lymph node on presentation, died 6 months after treatment. Two patients treated with palliative chemotherapy alone died after 5 and 6 months, respectively. Four patients were treated with palliative radiotherapy alone, 2 of whom died at 1 and 7 months after treatment. Of the remaining 2, 1 developed liver metastases and died after 7 months and the other was lost to follow-up.
Four patients with advanced disease and poor general condition were not offered any treatment. Autopsies were not performed as a routine in all patients as they had confirmed diagnoses before death.
The median survival of our patients was 6 months. We compared the overall survival of patients who had received surgery (with or without neoadjuvant chemotherapy and radiotherapy) with those who underwent nonsurgical modalities of treatment (chemotherapy, radiotherapy, or a combination of both). All patients treated nonsurgically, ie, with chemotherapy or radiotherapy, were dead within 10 months of diagnosis. However, all patients treated with surgery were alive, 1 with distant metastases, after a mean follow-up of 14.3 months.
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Comment |
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Histologically, small cell carcinoma of the esophagus is indistinguishable from its pulmonary counterpart. They are both believed to originate from Kulchitsky’s or amine precursor uptake and decarboxylation cells of neuroectodermal origin. It is believed that a totipotent primitive cell served as the common precursor for all types of esophageal carcinomas including the small cell variety. Hence, an occasional small cell carcinoma with epidermoid differentiation or even frank squamous carcinoma may be seen [4]. Three of our cases had similar features, with 3 having foci of frank squamous carcinoma in association with small cell carcinoma.
The clinical presentation of our patients was similar to that reported in world literature with a male-to-female ratio of 2.6:1 and a mean age of 62 years. McFadden and coworkers [5] reported a 3:2 male preponderance and a mean age of 64 years. Dysphagia of varying degrees is the most common presenting complaint [6, 7].
Like small cell carcinoma of the lung, small cell carcinomas of the esophagus are aggressive tumors that generally carry a poor prognosis. The treatment of this type of esophageal cancer is difficult because of the advanced stage of the disease at presentation. The staging system for esophageal (and all other extrapulmonary) small cell carcinoma is similar to pulmonary small cell carcinoma. Limited disease is defined as tumor confined to the esophagus and local regional nodes that can be encompassed within a radiation portal. Tumors that have spread beyond one radiation portal are defined as extensive. Forty-four percent of patients presenting to us had extensive-stage disease, similar to the series by Law and colleagues (45%) [3]. Caldwell and associates [8] and Isolauri and colleagues [9] reported higher incidences of metastatic disease: 56% and 62.5%, respectively. Distant metastases occur most frequently to the liver, lungs, and bones. Five of our 18 patients (28%) had hepatic metastases.
The rarity of the disease with attendant inability to conduct prospective treatment trials have resulted in varied treatment options and lack of a fixed protocol, which was also noticed in the management of the patients in our series. Small cell carcinoma of the esophagus, like its pulmonary counterpart, appears to be a chemosensitive neoplasm. Radiation therapy and surgery may achieve local control in some patients, but systemic disease is often present at diagnosis, thereby making systemic failure the primary therapeutic challenge. The emerging consensus has been to use systemic chemotherapy as an important part of multimodality therapy [6]. A few anecdotal cases have been reported in which complete response to combination chemotherapy was observed [10–13]. The median survival reported in patients treated with combination chemotherapy is 16.7 months, in contrast to 2.2 months in patients who did not receive any form of chemotherapy [3]. However, a direct inference cannot be made from this study as some of these patients were also given radiation therapy and most patients who did not receive chemotherapy were in advanced stages of disease [3].
The role of surgery as part of multimodality treatment in patients with limited-stage disease remains controversial. However, the longest survivals have been reported in patients treated with esophagectomy combined with chemotherapy or radiotherapy [10]. Nishimaki and associates [11] reviewed 13 patients with small cell carcinoma of the esophagus, of whom 2 survived long term. Both these patients had locoregional disease and were treated with multimodality treatment incorporating surgery. One patient, who was treated with neoadjuvant cisplatin, radical esophagectomy, and postoperative radiotherapy, survived 106 months. The other patient, treated with radical esophagectomy and postoperative cisplatin-based chemotherapy, survived 64 months. The median survival of the 9 patients treated with surgery as part of the treatment protocol in our series was 9 months. Lieberman and colleagues [12] reviewed 12 patients with small cell esophageal carcinoma and found survivals of 0.5 to 10 months in patients treated with chemotherapy alone, 1 to 10 months in patients treated with chemoradiotherapy, and 24 months in patients treated with esophagectomy and chemotherapy. Law and associates [3] reported a median survival of 15 months in 4 patients treated surgically. Craig and colleagues [13] reported a mean survival of 20 months in 7 of 16 patients treated with esophagectomy with or without chemotherapy or radiotherapy.
Our study showed a similar trend, with all patients treated with surgery (with or without chemotherapy/radiotherapy) alive at a mean follow-up of 14.3 months, 1 of the patients with lung metastases. Among the patients treated nonsurgically (radiotherapy, chemotherapy, or a combination of both), no patient was alive at 10 months. However, the seemingly better survival in patients treated with surgery may be erroneous due to the small sample size and the short follow-up of the patients treated with surgery. There is also an obvious selection bias because only patients with limited-stage disease were considered for surgery and patients with extensive disease were considered for only nonsurgical treatment. Radiotherapy alone has been found to have disappointing results and rarely should be used as the sole treatment modality [10].
The median overall survival reported in world literature ranges from 4.2 to 18.5 months [13–16]. The overall median survival in our patients was 6 months. In a study by Bennouna and associates [17], survival in 10 patients was reviewed with respect to the stage of disease at presentation. In limited stage, the median overall survival was 18.5 months, whereas survival was 11 months for extensive-stage disease. In a comprehensive study by Casas and colleagues [18], the authors compiled data on 199 patients with small cell carcinoma of the esophagus to evaluate prognostic factors that influence survival. A significant difference in survival was noted between patients with limited-stage disease and those with extensive-stage disease (p < 0.0001). Univariate analysis of limited-stage disease showed three significant prognostic factors: age (60 years or older), tumor size (5 cm or larger), and type of treatment (with combined local and systemic treatment, median survival was 20 months, whereas with only local treatment, 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. Clearly, more data are required to formulate authoritative treatment recommendations.
Conclusion
Small cell carcinoma of the esophagus is a rare disease. The carcinoma arises from amine precursor uptake and decarboxylation cells of neuroectodermal origin. The disease is aggressive and is associated with a poor prognosis. Most patients present in advanced stages of disease, and about half have metastatic disease on presentation. Presently, no clear consensus exists for the treatment of these patients, although the role of systemic chemotherapy is well established. Surgery after neoadjuvant chemotherapy improves overall survival in limited-stage disease. The role of radiotherapy is not yet well defined. Better reporting of these rare tumors worldwide, with a separate registry, will help understand the tumor biology and optimize treatment strategy.
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