Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass

Background. Erdheim-Chester disease is a rare histiocytic neoplasm associated with MAPK pathway mutations. Disease manifestation is variable often involving many different organs, mainly bone, retroperitoneum, the heart, and the central nervous system. Histological findings include foamy histiocytes...

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Main Authors: Antonio Maietta, Maryam Razmpoosh
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2020/3081824
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author Antonio Maietta
Maryam Razmpoosh
author_facet Antonio Maietta
Maryam Razmpoosh
author_sort Antonio Maietta
collection DOAJ
description Background. Erdheim-Chester disease is a rare histiocytic neoplasm associated with MAPK pathway mutations. Disease manifestation is variable often involving many different organs, mainly bone, retroperitoneum, the heart, and the central nervous system. Histological findings include foamy histiocytes in a fibrous stroma with scattered inflammatory infiltrate. Histiocytes are CD68 positive and S100 negative. Case Report. We report a case of Erdheim-Chester disease associated with small lymphocytic lymphoma presenting as a perirenal mass with a review of the recent literature. Conclusions. Erdheim-Chester disease rarely can be associated with other cancers, namely myeloid neoplasms. We report a case of Erdheim-Chester disease presenting with small lymphocytic lymphoma as a perirenal mass. The association of Erdheim-Chester disease with lymphoproliferative disorders needs to be elucidated.
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spelling doaj-art-3aa7262a330a4839b504b12cff0cc23b2025-02-03T06:43:38ZengWileyCase Reports in Pathology2090-67812090-679X2020-01-01202010.1155/2020/30818243081824Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal MassAntonio Maietta0Maryam Razmpoosh1Centre Hospitalier de l’université de Montréal (CHUM), 1100, rue Sanguinet, 7th Floor, F Pavillon, (Québec), Montréal, H2X 0C1, CanadaCentre Hospitalier de l’université de Montréal (CHUM), 1100, rue Sanguinet, 7th Floor, F Pavillon, (Québec), Montréal, H2X 0C1, CanadaBackground. Erdheim-Chester disease is a rare histiocytic neoplasm associated with MAPK pathway mutations. Disease manifestation is variable often involving many different organs, mainly bone, retroperitoneum, the heart, and the central nervous system. Histological findings include foamy histiocytes in a fibrous stroma with scattered inflammatory infiltrate. Histiocytes are CD68 positive and S100 negative. Case Report. We report a case of Erdheim-Chester disease associated with small lymphocytic lymphoma presenting as a perirenal mass with a review of the recent literature. Conclusions. Erdheim-Chester disease rarely can be associated with other cancers, namely myeloid neoplasms. We report a case of Erdheim-Chester disease presenting with small lymphocytic lymphoma as a perirenal mass. The association of Erdheim-Chester disease with lymphoproliferative disorders needs to be elucidated.http://dx.doi.org/10.1155/2020/3081824
spellingShingle Antonio Maietta
Maryam Razmpoosh
Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass
Case Reports in Pathology
title Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass
title_full Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass
title_fullStr Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass
title_full_unstemmed Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass
title_short Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass
title_sort erdheim chester disease and small lymphocytic lymphoma collision tumour presenting as a perirenal mass
url http://dx.doi.org/10.1155/2020/3081824
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