Ewing sarcoma family of tumours: unusual histological variants and immunophenotypic characteristics |
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| Authors: | Hue-Tsi Wu Dhirendra Govender |
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| Affiliation: | 1. Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, ROC;2. Department of Pathology, School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC;3. Department of Obstetrics and Gynecology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC;1. School of Mechanical Engineering, The University of Leeds, Leeds LS2 9JT, UK;2. Statoil ASA, Sandsliveien 90, 5254 Bergen, Norway;1. University of Bern, Institute of Social and Preventive Medicine, Bern, Switzerland;2. World Health Organization, Department of HIV/AIDS, Geneva, Switzerland;1. Department of Dermatology and Radiotherapy, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista, Botucatu, SP, Brazil;2. Department of Pathology, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista, Botucatu, SP, Brazil |
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| Abstract: | Ewing sarcoma family of tumours are typified by small round blue cell morphology with variable but usually minimal neuroectodermal differentiation and non-random translocations involving the EWSR1 gene and a member of the ETS family of genes. Atypical morphological patterns may be seen in up to 20% of cases and include large cell, adamantinoma-like, spindle cell sarcoma-like (synovial sarcoma like), sclerosing, clear cell (hypernephroid)/anaplastic, and vascular like patterns. Immunophenotypically, CD99, vimentin, CAV1 and FLI1 are typically positive. Around half of the cases may express NSE and/or CD57. Low molecular weight cytokeratins (CK), S100, NB84, and CD117 are expressed less commonly (around 20%–40%). EMA, desmin, CD31, and synaptophysin expression are rare. CK7, CK20, D2-40, chromogranin, TDT, TTF, MYF4 appear to be consistently negative. Molecular confirmation is less sensitive. The correct diagnosis requires correlation between morphology and immunostaining, and may require molecular testing. |
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