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Neuroendocrine tumor (NET) of the vagina in the light of WHO 2020 2-tiered grading system: clinicopathological report of the first described case

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Abstract

Up to now, a vaginal well-differentiated neuroendocrine tumor (NET) has never been well described in the literature. A 2-cm vaginal nodule morphologically revealed a proliferation of mild to moderately atypical eosinophilic epithelioid cells, without tumor cell necrosis. Immunohistochemistry (IHC) showed positivity for CK (AE1/AE3), chromogranin A, and synaptophysin, focal positivity for CDX2, and negativity for PAX8 and TTF1. Metastatic origin was excluded by imaging and the morphological context with benign mucinous glands as present in the surgical resection specimen. Considering mitotic index and Mib1/Ki67 (8 mitoses/2 mm2; >20%), the case was diagnosed as vaginal NET G2 in the light of the WHO 2020 grading system for the gynecologic neuroendocrine neoplasms (NENs). Ranges of Mib1-Ki67 are not yet standardized. Currently, mitotic index and tumor cell necrosis were taken into consideration for the grading system. Gynecologic NENs still represent a diagnostic challenge. A clinico-radiologic workup and an appropriate diagnostic path ruling out the metastatic nature are mandatory to achieve the diagnosis.

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Authors and Affiliations

  1. Dipartimento Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Unità di Gineco-patologia e Patologia Mammaria, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy

    Frediano Inzani, Angela Santoro, Giuseppe Angelico & Gian Franco Zannoni

  2. Department of Diagnostics and Public Health, G.B. Rossi University Hospital, Verona, Italy

    Maria Gaia Mastrosimini

  3. Division of Gynecology, Dipartimento Scienze della Salute della donna e del bambino e di Sanità pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy

    Valeria Masciullo

  4. Gynecologic Oncology Unit, Department of Woman, Child Health and Public Health, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome, Italy

    Vanda Salutari

  5. Istituto di Anatomia Patologica, Università Cattolica del Sacro Cuore, Rome, Italy

    Gian Franco Zannoni

  6. Department of Woman and Child Health, UCSC and Fondazione Policlinico Gemelli, Rome, Italy

    Gian Franco Zannoni

Authors
  1. Frediano Inzani
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  2. Angela Santoro
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  3. Giuseppe Angelico
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  4. Maria Gaia Mastrosimini
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  5. Valeria Masciullo
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  6. Vanda Salutari
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  7. Gian Franco Zannoni
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Contributions

FI and GFZ—study design, acquisition of pathological data; AS and GA—acquisition of pathological data, writing of the manuscript; VM and VS—acquisition of clinical data, surgical procedures; MGM—critical review of the manuscript. All authors have read and approved the manuscript.

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Correspondence to Gian Franco Zannoni.

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Inzani, F., Santoro, A., Angelico, G. et al. Neuroendocrine tumor (NET) of the vagina in the light of WHO 2020 2-tiered grading system: clinicopathological report of the first described case. Virchows Arch 480, 687–691 (2022). https://doi.org/10.1007/s00428-021-03078-6

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  • DOI: https://doi.org/10.1007/s00428-021-03078-6

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