PMID- 35039897 OWN - NLM STAT- MEDLINE DCOM- 20220627 LR - 20220627 IS - 1432-2307 (Electronic) IS - 0945-6317 (Linking) VI - 481 IP - 1 DP - 2022 Jul TI - Malignant gastrointestinal neuroectodermal tumor in head and neck: two challenging cases with diverse morphology and different considerations for differential diagnosis. PG - 131-136 LID - 10.1007/s00428-022-03274-y [doi] AB - Malignant gastrointestinal neuroectodermal tumor (MGNET) is a sarcoma typically involving the gastrointestinal tract with neuroectodermal differentiation and EWSR1-ATF1/CREB1 fusions. Recently, rare MGNET cases were reported in extragastrointestinal sites. We identified 2 cases of MGNET arising in unprecedented laryngeal and intracranial locations, respectively. Both cases showed spindle and epithelioid tumor cells with amphophilic to clear cytoplasm and occasionally prominent nucleoli, arranged in solid, fascicular, and pseudoalveolar patterns. Case 1 exhibited moderate to marked nuclear atypia and focal intraepithelial component. In contrast, case 2 comprised predominantly low-grade epithelioid cells with extensive pseudopapillary structures. Both tumors showed an S100/SOX10-positive and HMB45/melan-A-negative immunoprofile as well as EWSR1-ATF1 fusion. A chief obstacle in diagnosing case 1 was the histologic and immunophenotypic resemblance to melanoma. The striking pseudopapillary architecture and the intracranial location of case 2 rendered differential diagnoses including meningioma and ependymoma. With the peculiar locations and morphology, these cases posed great diagnostic challenge. CI - (c) 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Kuo, Chien-Tzu AU - Kuo CT AD - Department and Graduate Institute of Pathology, National Taiwan University Hospital, National Taiwan University College of Medicine, 7 Chung-Shan South Road, Taipei, 10002, Taiwan. FAU - Kao, Yu-Chien AU - Kao YC AD - Department of Pathology, Shuang Ho Hospital, Taipei Medical University, Taipei, Taiwan. AD - Department of Pathology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan. FAU - Huang, Hsuan-Ying AU - Huang HY AD - Department of Pathology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan. FAU - Hsiao, Cheng-Hsiang AU - Hsiao CH AD - Department of Anatomical Pathology, Cheng-Hsin General Hospital, Taipei, Taiwan. FAU - Lee, Jen-Chieh AU - Lee JC AUID- ORCID: 0000-0001-7739-5934 AD - Department and Graduate Institute of Pathology, National Taiwan University Hospital, National Taiwan University College of Medicine, 7 Chung-Shan South Road, Taipei, 10002, Taiwan. leejenchieh@ntuh.gov.tw. LA - eng GR - NTUH 108-S4295/National Taiwan University Hospital/ PT - Journal Article DEP - 20220118 PL - Germany TA - Virchows Arch JT - Virchows Archiv : an international journal of pathology JID - 9423843 RN - 0 (Biomarkers, Tumor) SB - IM MH - Biomarkers, Tumor/genetics MH - Diagnosis, Differential MH - *Gastrointestinal Neoplasms/pathology MH - Humans MH - Immunohistochemistry MH - In Situ Hybridization, Fluorescence MH - *Melanoma OTO - NOTNLM OT - ATF1 OT - Clear cell sarcoma OT - EWSR1 OT - Malignant gastrointestinal neuroectodermal tumor OT - Melanoma EDAT- 2022/01/19 06:00 MHDA- 2022/06/28 06:00 CRDT- 2022/01/18 06:06 PHST- 2021/08/27 00:00 [received] PHST- 2022/01/09 00:00 [accepted] PHST- 2021/12/02 00:00 [revised] PHST- 2022/01/19 06:00 [pubmed] PHST- 2022/06/28 06:00 [medline] PHST- 2022/01/18 06:06 [entrez] AID - 10.1007/s00428-022-03274-y [pii] AID - 10.1007/s00428-022-03274-y [doi] PST - ppublish SO - Virchows Arch. 2022 Jul;481(1):131-136. doi: 10.1007/s00428-022-03274-y. Epub 2022 Jan 18.