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      Cutaneous Carcinosarcoma: a Clinicopathologic and Immunohistochemical Analysis of 11 Korean Cases

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          Abstract

          Background

          Cutaneous carcinosarcoma is a rare biphasic tumor comprising malignant epithelial and heterologous mesenchymal elements. Data on the clinical and histopathologic characteristics of this tumor in Asian populations are not available. The purpose of this study was to investigate the clinicopathologic and immunohistochemical features of cutaneous carcinosarcoma in the Korean population.

          Methods

          We retrospectively reviewed the records of 11 patients with cutaneous carcinosarcoma who were diagnosed from 2006 to 2016.

          Results

          The mean patient age at diagnosis was 71.5 years (range, 43–96 years) and there was a men predilection. The most common site of cutaneous carcinosarcoma was the head and neck (8/11, 72.7%). Histopathologically, most tumors showed a characteristic morphology consisting of two types of tumor cells, varied differentiated epithelial cells (such as basal or squamous cells) and spindle cells with transition zones between the two components. These two cell types also demonstrated variable immunohistochemical characteristics.

          Conclusion

          Although the number of cases in this study was limited, our results provide valuable insight into the clinical and histopathologic characteristics of cutaneous carcinosarcoma in the Korean population.

          Graphical Abstract

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          Most cited references33

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          Sarcomatoid (spindle cell) carcinoma of the head and neck mucosal region: a clinicopathologic review of 103 cases from a tertiary referral cancer centre.

          Sarcomatoid carcinomas are biphasic tumors proven to be monoclonal dedifferentiated forms of conventional squamous carcinomas. This study evaluates their clinicopathologic characteristics in head and neck mucosal sites and the problems in distinguishing them from other spindle cell tumors. A total of 103 cases with a confirmed diagnosis of sarcomatoid carcinoma accessioned in the pathology department of a tertiary referral cancer centre over a period of 7 years (2004-2010) were studied. An algorithm used for their diagnosis is presented. Ages of the patients were 22-90 years (median 53 years), and male:female ratio was 3.7:1. Site distribution was oral cavity (n = 65, 63.1%), larynx (18, 17.5%), oropharynx/hypopharynx (12, 10.7%), maxilla (6, 5.8%) and metastatic nodes (2, 1.9%). A large number of patients (95%) presented with a mass lesion of less than 1 year duration. Histopathologically, epithelial differentiation was evident on morphology in 48 (46.6%) cases, only on IHC in 34 (33%) cases, and in 21 (20.4%) no epithelial differentiation was seen. Typically, tumors were polypoidal (92, 89.3%) and ulcerated (95, 92.2%) with cells arranged predominantly in fascicles (59, 57.3%) or storiform pattern (17, 16.5%) amidst collagenous (50, 48.5%) or myxoid matrix (35, 34%). Anaplasia (2+/3+) and mitosis >10 per 10 HPF were noted in 96 (93.2%) cases. IHC was done in 82 cases; 55 (66.7%) showed positivity for epithelial markers with aberrant expression of mesenchymal markers in 43 (41.7%). Diagnosis of sarcomatoid squamous carcinoma is challenging because of overlapping histopathological features with other spindle cell tumors. Understanding their clinicopathologic characteristics facilitates their diagnosis and appropriate clinical management.
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            Monoclonal origins of malignant mixed tumors (carcinosarcomas). Evidence for a divergent histogenesis.

            Malignant mixed tumors (carcinosarcomas) are examples of unusual neoplasms whose occurrences have been observed in increasingly diverse sites but whose pathogenesis remains a complete mystery. Two antithetical hypotheses that have been advanced to explain the histogenesis of these tumors include the convergence hypothesis, which proposes an origin from two or more stem cells (multiclonal hypothesis), and the divergence hypothesis, which proposes an origin from a single totipotential stem cell that differentiates into separate epithelial and mesenchymal directions (monoclonal hypothesis). To test these hypotheses, a novel strategy for the determination of clonality from as few as 100 tumor cells obtained by enzymatic digestion of either fresh or formalin-fixed, paraffin-embedded tissues and cell sorting was used that exhibited the polymerase chain reaction (PCR) in amplifying a 511-bp region located within the first intron of the human hypoxanthine phosphoribosyl transferase gene, a site that contains inactive X chromosomal obligately methylated HpaII/MspI sites and single-base allelic polymorphisms in 5% females. Carcinoma cells gated on the basis of fluorescein isothiocyanate (FITC)-anti-cytokeratin and sarcoma cells gated on the basis of FITC-antivimentin or FITC-anti-desmin were sorted to homogeneity on FACSTAR and then subjected to genomic DNA extraction and Hpa II digestion before PCR amplification and subsequent analysis of the product on denaturing gradient gel electrophoresis. The comigrations of the single homoduplexes generated from both the carcinoma cells and sarcoma cells in six different malignant mixed tumors obtained from four different organs indicated clonal identity and monoclonality in all cases. These findings of monoclonality were confirmed independently by two other methods of clonality determination. The findings of a monoclonal origin of carcinosarcomas support the single totipotential stem-cell-divergence hypothesis.
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              Sarcomatoid squamous cell carcinoma of the mucous membranes of the head and neck: a clinicopathologic study of 20 cases.

              Twenty cases of head and neck mucosal squamous cell carcinoma containing a prominent sarcomatoid element were reviewed with special attention to possible prognostic factors. Patients whose tumors invaded muscle, minor salivary or accessory respiratory glands, or bone had very poor survival rates, whereas those whose tumors were superficial and did not extend into any of these structures had excellent survival rates. A history of irradiation to the tumor site and tumor location in the oral cavity rather than the larynx, pharynx, nasal cavity, or nasal-associated structures were associated with invasiveness and thus with poorer survival. Aside from invasion, histologic features and gross configuration were not found to be of significant prognostic importance.
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                Author and article information

                Journal
                J Korean Med Sci
                J. Korean Med. Sci
                JKMS
                Journal of Korean Medical Science
                The Korean Academy of Medical Sciences
                1011-8934
                1598-6357
                26 December 2018
                07 January 2019
                : 34
                : 1
                : e5
                Affiliations
                [1 ]Department of Dermatology, Chonbuk National University Medical School, Jeonju, Korea.
                [2 ]Research Institute of Clinical Medicine of Chonbuk National University, Biomedical Research Institute of Chonbuk National University Hospital, Jeonju, Korea.
                [3 ]Department of Dermatology, Presbyterian Medical Center, Jeonju, Korea.
                Author notes
                Address for Correspondence: Seok-Kweon Yun, MD, PhD. Department of Dermatology, Chonbuk National University Medical School, Research Institute of Clinical Medicine of Chonbuk National University, Biomedical Research Institute of Chonbuk National University Hospital, 20 Geonji-ro, Deokjin-gu, Jeonju 54907, Republic of Korea. dermayun@ 123456jbnu.ac.kr
                Address for Correspondence: Kyung-Hwa Nam, MD. Department of Dermatology, Presbyterian Medical Center, 365 Seowon-ro, Wansan-gu, Jeonju 54987, Republic of Korea. kyung1212@ 123456hanmail.net
                Author information
                https://orcid.org/0000-0002-0216-301X
                https://orcid.org/0000-0002-8830-5479
                https://orcid.org/0000-0002-7173-7937
                https://orcid.org/0000-0002-9846-4668
                https://orcid.org/0000-0002-1498-3701
                Article
                10.3346/jkms.2019.34.e5
                6318441
                30618513
                fa4f3156-6062-4c40-8022-5552eec7e09a
                © 2019 The Korean Academy of Medical Sciences.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 22 March 2018
                : 10 October 2018
                Categories
                Original Article
                Dermatology

                Medicine
                cutaneous carcinosarcoma,korean population,pathology
                Medicine
                cutaneous carcinosarcoma, korean population, pathology

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