Primary Hepatic Small Cell Carcinoma: Two Case Reports, Molecular Characterization and Pooled Analysis of Known Clinical Data

• Primary hepatic carcinoma
• small cell cancer
• neuroendocrine tumor

Extrapulmonary small cell carcinoma (EPSCC) originating in the gastrointestinal tract is a very rare tumor, and since its first description in 1952, only a few hundred cases have been reported in the literature (1). The overall understanding of the clinicopathological characteristics of this rare disease is still meager. To our knowledge, no prospective trials exist for this disease and treatment recommendations are extrapolated from studies of small cell lung cancer (SCLC) or derived from single-Institution experiences (1-5).

EPSCC is primarily composed of poorly differentiated, or anaplastic cells, although segments of well-differentiated squamous or glandular tissue may also be present. It usually stains positively for the neuroendocrine markers chromogranin, synaptophysin and CD56 on immunohistochemistry and has a high proliferative index (Ki-67) (6-10). These tumors are usually very aggressive and characterized by poor survival. Outcomes after surgical resection appear to be poor as is the case with other poorly differentiated neuroendocrine tumors, and benefit from chemotherapy or radiation is not well- established (11, 12). No staging system has been specifically established for EPSCC of gastrointestinal origin. Some authors use the TNM system while most others have adopted the Veterans Administration Lung Study group staging that broadly classifies patients as having early-stage vs. extensive-stage disease (13, 14).

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Primary hepatic SCC (HSCC) is a very rare clinical entity and to the best of our knowledge, the molecular pathogenesis and optimal clinical management of this disease have yet to be elucidated. A retrospective cohort study of the Surveillance, Epidemiology and End-Results (SEER) database, which identified patients with primary HSCC revealed the presence of 16 cases reported in adults in the United States from 1973 to 2008 (11). Some of these cases did not have postmortem examinations, leaving a degree of uncertainty as to whether or not they were true primary hepatic small-cell cases. Systematic genomic analysis is difficult to perform as these tumors are rarely treated with surgery and obtaining fresh frozen tumor samples is challenging (15). Only recently have key somatic driver mutations been characterized for SCLC (15, 16). With limited available data, comparison of available molecular analysis in gastrointestinal and pulmonary SCC reveal certain similarities (17).

Overall, there appear to exist several unanswered questions pertaining to the molecular pathogenesis, clinical features, optimal treatment strategies and outcome of primary HSCC, the answers to which are likely to be relevant to EPSCC as a class of tumors. Herein we report clinical data and molecular characterization results of two cases of primary HSCC from our Institution, as well as a systematic review and outcomes analysis of all the cases of primary HSCC reported in the literature.

• Received September 23, 2015.
• Revision received November 9, 2015.
• Accepted November 12, 2015.

• Copyright© 2016 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved

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