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      Effective Monotherapy with Amrubicin for a Refractory Extrapulmonary Small-Cell Carcinoma of the Liver

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          Abstract

          Small-cell carcinoma of the liver is a rare neoplasm, and no standard treatment for it has yet been established. A 72-year-old man with an extensive disease stage of small-cell carcinoma of the liver was treated with systemic chemotherapy consisting of cisplatin and etoposide (PE) followed by irinotecan. Although the masses were markedly decreased once after the sixth course of PE, amrubicin monotherapy as third-line chemotherapy was started because the hepatic masses had increased again. The administration of amrubicin was repeated in 8 courses with regression of the disease, resulting in a 26-month survival since the first-line chemotherapy was started. This is the first case report of a refractory EPSCC successfully treated with amrubicin.

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          Irinotecan plus cisplatin compared with etoposide plus cisplatin for extensive small-cell lung cancer.

          Kazumasa Noda, Yutaka Nishiwaki, Masaaki Kawahara … (2002)
          Irinotecan hydrochloride, a topoisomerase I inhibitor, is effective against small-cell lung cancer. In a phase 2 study of irinotecan plus cisplatin in patients with extensive small-cell lung cancer, there was a high response rate and a promising median survival time. We conducted a multicenter, randomized, phase 3 study in which we compared irinotecan plus cisplatin with etoposide plus cisplatin in patients with extensive (metastatic) small-cell lung cancer. The planned size of the study population was 230 patients, but enrollment was terminated early because an interim analysis found a statistically significant difference in survival between the patients assigned to receive irinotecan and cisplatin and those assigned to receive etoposide and cisplatin; as a result, only 154 patients were enrolled. The median survival was 12.8 months in the irinotecan-plus-cisplatin group and 9.4 months in the etoposide-plus-cisplatin group (P=0.002 by the unadjusted log-rank test). At two years, the proportion of patients surviving was 19.5 percent in the irinotecan-plus-cisplatin group and 5.2 percent in the etoposide-plus-cisplatin group. Severe or life-threatening myelosuppression was more frequent in the etoposide-plus-cisplatin group than in the irinotecan-plus-cisplatin group, and severe or life-threatening diarrhea was more frequent in the irinotecan-plus-cisplatin group than in the etoposide-plus-cisplatin group. Irinotecan plus cisplatin is an effective treatment for metastatic small-cell lung cancer.
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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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              Extrapulmonary small cell carcinoma.

              E Galanis, Jane E. Lloyd, S Frytak (1997)
              Extrapulmonary small cell carcinoma (EPSCCA) is often an underrecognized clinicopathologic entity, distinct from small cell lung carcinoma. The purpose of this study was to review the study institution's experience with EPSCCA with specific emphasis on the epidemiology and response to treatment of these uncommon neoplasms. Using the tumor registry database of the study institution, the authors retrieved and reviewed the records of all patients with EPSCCA treated between 1974 and 1994. Study eligibility required that the patients had a normal chest radiograph, computed tomography scan of the chest, sputum cytology, and/or negative bronchoscopy. Patients with well differentiated neuroendocrine carcinomas and Merkel cell carcinomas of the skin were excluded. Primary sites of EPSCCA in the current series were: gastrointestinal system in 29 patients, ear, nose, and throat in 14, genitourinary system in 12, internal genitalia in 10, upper respiratory system in 5, unknown primary-lymph nodes in 5, unknown primary-other in 2, the thymus in 3, and the peritoneum in 1. After the initial evaluation and confirmation of histologic diagnosis, 10 of 81 patients had been lost to follow-up. Of the remaining 71 patients, 54 had limited disease. Forty of the 54 patients underwent surgical treatment of their malignancy; 10 patients (25%) remained alive and disease free at least 3 years after surgery, whereas 30 patients (75%) relapsed with a median disease free survival of 6 months. Six patients of 54 with limited disease were treated with chemotherapy and radiation. Five of these 6 (83%) relapsed at a median time of 11.5 months. Another 8 of the total of 54 patients received only radiation therapy and all had disease recurrence at a median time of 5 months. In the group of patients with extensive or recurrent disease, platinum-based chemotherapy was employed in 22 patients. There was a 72% response rate with a median duration of 8.5 months. Seven patients had doxorubicin-based chemotherapy. There was a 57% response rate with a median duration of 4.5 months. In the current study group of patients, the 3-year disease free and overall survival rates were 26% and 38%, respectively, whereas the 5-year disease free and overall survival rates were each 13%. EPSCCA is usually a fatal disease, with a 13% 5-year survival rate. In a small percentage of patients, surgery can be curative if the tumor is small and confined to the organ of origin. Because of the poor overall outcome, one needs to consider the possible use of adjuvant chemotherapy in appropriate circumstances if surgery is to be employed. In most patients with limited disease, the combination of chemotherapy and radiation as the primary treatment can be as effective as surgery. EPSCCA is responsive to commonly employed regimens for small cell lung carcinoma; however, the responses are short-lived. The extent of disease at diagnosis represents the most sensitive predictor of survival.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Report Med
                Journal ID (publisher-id): CRM
                Title: Case Reports in Medicine
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 1687-9627
                ISSN (Electronic): 1687-9635
                Publication date (Print): 2009
                Publication date (Electronic): 16 June 2009
                Volume: 2009
                Electronic Location Identifier: 538081
                Affiliations
                1Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-Ku, Fukuoka 812-8582, Japan
                2Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-Ku, Fukuoka 812-8582, Japan
                3Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-Ku, Fukuoka 812-8582, Japan
                Author notes

                Recommended by Ramsey Cheung

                Article
                DOI: 10.1155/2009/538081
                PMC ID: 2729275
                PubMed ID: 19718248
                SO-VID: c546496b-db88-4f68-b37f-85bbdcafc8c9
                Copyright © Copyright © 2009 Taichi Isobe et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 7 February 2009
                Date accepted : 19 May 2009
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Medicine
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                ScienceOpen disciplines: Medicine

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