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      Carcinosarcoma: A rare case report of a recurrent mass in the neck region

      case-report

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          Abstract

          Carcinosarcoma, a biphasic malignant mixed tumor, is an extremely rare neoplasm with >1% incidence. This aggressive malignancy is characterized by the presence of two components admixed with each other, i.e., the epithelial component and the mesenchymal component arising from a monoclonal/multiclonal origin or de novo. Most patients usually present between 60 and 65 years of age with no sex predilection. The authors present a case of carcinosarcoma arising as a mass in the neck region of a 14-year-old male. The case is been presented for its rarity of occurrence in the younger age group.

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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.

          Khaliqur Rahman, Suryawanshi Pallavi, Seethalakshmi Viswanathan … (2010)
          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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            Spindle cell (sarcomatoid) carcinomas of the larynx: a clinicopathologic study of 187 cases.

            D Heffner, Jacqueline Wieneke, Markku Miettinen … (2002)
            Laryngeal spindle cell (sarcomatoid) carcinomas are uncommon tumors, frequently misdiagnosed as reactive lesions or mesenchymal malignancies. The records of 187 patients with tumors diagnosed as laryngeal spindle cell (sarcomatoid) carcinoma were retrieved from the files of the Otorhinolaryngic Tumor Registry of the Armed Forces Institute of Pathology. There were 174 men and 13 women, 35-92 years of age (average, 65.6 years). Nearly all patients experienced hoarseness (n = 165 [88%] patients) for a mean duration of 11.0 months. Patients admitted to smoking (n = 162 [87%] patients) and/or alcohol use (n = 90 [48%] patients). Most tumors were glottic (n = 132 [71%]), T1 (n = 111 [59%]), 1 and polypoid (n = 185 [99%]), with a mean tumor size of 1.8 cm. Histologically, squamous cell carcinoma (n = 157 [84%]) was noted, ulcerated, and blended with the spindle cell component, which was most frequently arranged in a storiform pattern (n = 92 [49%] tumors). Foci of benign or malignant cartilage and/or bone (n = 13 [7%]) were noted in the spindle cell component. All patients were treated with surgery (n = 90 [48%] patients) or surgery with radiation (n = 97 [52%] patients). Recurrences developed in 85 (45%) patients. Overall, T1 glottic tumors managed by complete surgical eradication had the best outcome (mean follow-up, 7.8 years).
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              Monoclonal origins of malignant mixed tumors (carcinosarcomas). Evidence for a divergent histogenesis.

              X.-Y. Chang, S Barsky, Fabiano Thompson (1996)
              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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                Author and article information

                Journal
                Journal ID (nlm-ta): J Oral Maxillofac Pathol
                Journal ID (iso-abbrev): J Oral Maxillofac Pathol
                Journal ID (publisher-id): JOMFP
                Title: Journal of Oral and Maxillofacial Pathology : JOMFP
                Publisher: Wolters Kluwer - Medknow (India )
                ISSN (Print): 0973-029X
                ISSN (Electronic): 1998-393X
                Publication date (Print): May-Aug 2020
                Publication date (Electronic): 09 September 2020
                Volume: 24
                Issue: 2
                Pages: 327-331
                Affiliations
                [1 ]Department of Head and Neck Surgery Unit, Mahatma Gandhi Cancer Hospital, Miraj, Maharashtra, India
                [2 ]Department of Onco Surgery, Mahatma Gandhi Cancer Hospital, Miraj, Maharashtra, India
                [3 ]Department of Oral Pathology and Microbiology and Forensic Odontology, School of Dental Sciences, Krishna Institute of Medical Sciences, Karad, Maharashtra, India
                Author notes
                Address for correspondence: Dr. Sushma Bommanavar, Department of Oral Pathology and Microbiology, School of Dental Sciences, Krishna Institute of Medical Sciences, KIMSDU, Karad, Maharashtra, India. E-mail: drsushopath@ 123456gmail.com
                Article
                Publisher ID: JOMFP-24-327
                DOI: 10.4103/jomfp.JOMFP_120_20
                PMC ID: 7802836
                PubMed ID: 33456243
                SO-VID: 02cd5942-408a-4a4f-97da-2495fd3809cb
                Copyright © Copyright: © 2020 Journal of Oral and Maxillofacial Pathology
                License:

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                Date received : 26 March 2020
                Date revision received : 18 May 2020
                Date accepted : 08 June 2020
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Pathology
                Keywords: carcinosarcoma,head and neck neoplasm,mixed tumor,recurrent mass,supraclavicular region,young adult
                Data availability:
                ScienceOpen disciplines: Pathology
                Keywords: carcinosarcoma, head and neck neoplasm, mixed tumor, recurrent mass, supraclavicular region, young adult

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