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Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature

BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is an extremely rare malignant tumor caused by proliferation of antigen-presenting follicular dendritic cells. The tumor most commonly arises in lymph nodes, with fewer than 30% of cases originating in extranodal sites. CASE PRESENTATION: We repor...

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Autores principales: Akel, Reem, Fakhri, Ghina, Salem, Rana, Boulos, Fouad, Habib, Khaled, Tfayli, Arafat
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6006627/
https://www.ncbi.nlm.nih.gov/pubmed/29928216
http://dx.doi.org/10.1159/000489602
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author Akel, Reem
Fakhri, Ghina
Salem, Rana
Boulos, Fouad
Habib, Khaled
Tfayli, Arafat
author_facet Akel, Reem
Fakhri, Ghina
Salem, Rana
Boulos, Fouad
Habib, Khaled
Tfayli, Arafat
author_sort Akel, Reem
collection PubMed
description BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is an extremely rare malignant tumor caused by proliferation of antigen-presenting follicular dendritic cells. The tumor most commonly arises in lymph nodes, with fewer than 30% of cases originating in extranodal sites. CASE PRESENTATION: We report the case of a 39-year-old previously healthy male patient who presented with paraneoplastic pemphigus in the setting of a large intraperitoneal mass with no associated lymphadenopathy. The biopsy results showed a cellular proliferation of mildly to moderately atypical oval-to-spindle-shaped cells admixed with a variably dense lymphoplasmacytic infiltrate suggestive of FDCS versus inflammatory myofibroblastic tumor. A pathology review following total resection of the abdominal mass confirmed the diagnosis of FDCS. The tumor cells showed positivity for CD35, CD21, CD45, and CD68, negative ALK staining, and limited smooth muscle actin immunoreactivity. The surgery was complicated by severe pneumonia and acidosis, resulting in a prolonged ICU stay and death. CONCLUSION: It is essential to maintain a high index of suspicion for FDCS and include it in the differential diagnosis of a spindle cell neoplasm, especially in the setting of paraneoplastic pemphigus. The histological and immunohistochemical features should be sufficient to confirm the diagnosis of FDCS.
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spelling pubmed-60066272018-06-20 Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature Akel, Reem Fakhri, Ghina Salem, Rana Boulos, Fouad Habib, Khaled Tfayli, Arafat Case Rep Oncol Case Report BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is an extremely rare malignant tumor caused by proliferation of antigen-presenting follicular dendritic cells. The tumor most commonly arises in lymph nodes, with fewer than 30% of cases originating in extranodal sites. CASE PRESENTATION: We report the case of a 39-year-old previously healthy male patient who presented with paraneoplastic pemphigus in the setting of a large intraperitoneal mass with no associated lymphadenopathy. The biopsy results showed a cellular proliferation of mildly to moderately atypical oval-to-spindle-shaped cells admixed with a variably dense lymphoplasmacytic infiltrate suggestive of FDCS versus inflammatory myofibroblastic tumor. A pathology review following total resection of the abdominal mass confirmed the diagnosis of FDCS. The tumor cells showed positivity for CD35, CD21, CD45, and CD68, negative ALK staining, and limited smooth muscle actin immunoreactivity. The surgery was complicated by severe pneumonia and acidosis, resulting in a prolonged ICU stay and death. CONCLUSION: It is essential to maintain a high index of suspicion for FDCS and include it in the differential diagnosis of a spindle cell neoplasm, especially in the setting of paraneoplastic pemphigus. The histological and immunohistochemical features should be sufficient to confirm the diagnosis of FDCS. S. Karger AG 2018-05-31 /pmc/articles/PMC6006627/ /pubmed/29928216 http://dx.doi.org/10.1159/000489602 Text en Copyright © 2018 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Case Report
Akel, Reem
Fakhri, Ghina
Salem, Rana
Boulos, Fouad
Habib, Khaled
Tfayli, Arafat
Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature
title Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature
title_full Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature
title_fullStr Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature
title_full_unstemmed Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature
title_short Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature
title_sort paraneoplastic pemphigus as a first manifestation of an intra-abdominal follicular dendritic cell sarcoma: rare case and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6006627/
https://www.ncbi.nlm.nih.gov/pubmed/29928216
http://dx.doi.org/10.1159/000489602
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