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“It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case

Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, le...

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Autores principales: van der Lely, Sarah J, Boorsma, Jeffrey, Hilhorst, Marc, Kers, Jesper, Roelofs, Joris, Jakulj, Lily, van Onna, Marieke
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7930597/
https://www.ncbi.nlm.nih.gov/pubmed/33407043
http://dx.doi.org/10.1177/0961203320981765
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author van der Lely, Sarah J
Boorsma, Jeffrey
Hilhorst, Marc
Kers, Jesper
Roelofs, Joris
Jakulj, Lily
van Onna, Marieke
author_facet van der Lely, Sarah J
Boorsma, Jeffrey
Hilhorst, Marc
Kers, Jesper
Roelofs, Joris
Jakulj, Lily
van Onna, Marieke
author_sort van der Lely, Sarah J
collection PubMed
description Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.
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spelling pubmed-79305972021-03-18 “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case van der Lely, Sarah J Boorsma, Jeffrey Hilhorst, Marc Kers, Jesper Roelofs, Joris Jakulj, Lily van Onna, Marieke Lupus Grand Round Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers. SAGE Publications 2021-01-06 2021-03 /pmc/articles/PMC7930597/ /pubmed/33407043 http://dx.doi.org/10.1177/0961203320981765 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Grand Round
van der Lely, Sarah J
Boorsma, Jeffrey
Hilhorst, Marc
Kers, Jesper
Roelofs, Joris
Jakulj, Lily
van Onna, Marieke
“It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case
title “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case
title_full “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case
title_fullStr “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case
title_full_unstemmed “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case
title_short “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case
title_sort “it’s not lupus”. a placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. a grand round case
topic Grand Round
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7930597/
https://www.ncbi.nlm.nih.gov/pubmed/33407043
http://dx.doi.org/10.1177/0961203320981765
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