Diagnostic red flags
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Diagnostic red flags : steroid-treated malignant CNS lymphoma mimicking autoimmune inflammatory demyelination. / Barrantes-Freer, Alonso; Engel, Aylin Sophie; Rodríguez-Villagra, Odir Antonio; Winkler, Anne; Bergmann, Markus; Mawrin, Christian; Kuempfel, Tania; Pellkofer, Hannah; Metz, Imke; Bleckmann, Annalen; Hernández-Durán, Silvia; Schippling, Sven; Rushing, Elisabeth J; Frank, Stephan; Glatzel, Markus; Matschke, Jakob; Hartmann, Christian; Reifenberger, Guido; Müller, Wolf; Schildhaus, Hans-Ulrich; Brück, Wolfgang; Stadelmann, Christine.
in: BRAIN PATHOL, Jahrgang 28, Nr. 2, 03.2018, S. 225-233.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Diagnostic red flags
T2 - steroid-treated malignant CNS lymphoma mimicking autoimmune inflammatory demyelination
AU - Barrantes-Freer, Alonso
AU - Engel, Aylin Sophie
AU - Rodríguez-Villagra, Odir Antonio
AU - Winkler, Anne
AU - Bergmann, Markus
AU - Mawrin, Christian
AU - Kuempfel, Tania
AU - Pellkofer, Hannah
AU - Metz, Imke
AU - Bleckmann, Annalen
AU - Hernández-Durán, Silvia
AU - Schippling, Sven
AU - Rushing, Elisabeth J
AU - Frank, Stephan
AU - Glatzel, Markus
AU - Matschke, Jakob
AU - Hartmann, Christian
AU - Reifenberger, Guido
AU - Müller, Wolf
AU - Schildhaus, Hans-Ulrich
AU - Brück, Wolfgang
AU - Stadelmann, Christine
N1 - © 2017 International Society of Neuropathology.
PY - 2018/3
Y1 - 2018/3
N2 - The presence of inflammation and demyelination in a central nervous system (CNS) biopsy points towards a limited, yet heterogeneous group of pathologies, of which multiple sclerosis (MS) represents one of the principal considerations. Inflammatory demyelination has also been reported in patients with clinically suspected primary central nervous system lymphoma (PCNSL), especially when steroids had been administered prior to biopsy acquisition. The histopathological changes induced by corticosteroid treatment can range from mild reduction to complete disappearance of lymphoma cells. It has been proposed that in the absence of neoplastic B cells, these biopsies are indistinguishable from MS, yet despite the clinical relevance, no histological studies have specifically compared the two entities. In this work, we analyzed CNS biopsies from eight patients with inflammatory demyelination in whom PCNSL was later histologically confirmed, and compared them with nine well defined early active multiple sclerosis lesions. In the patients with steroid-treated PCNSL (ST-PCNSL) the interval between first and second biopsy ranged from 3 to 32 weeks; all of the patients had received corticosteroids before the first, but not the second biopsy. ST-PCNSL patients were older than MS patients (mean age: ST-PCNSL: 62 ± 4 years, MS: 30 ± 2 years), and histological analysis revealed numerous apoptoses, patchy and incomplete rather than confluent and complete demyelination and a fuzzy lesion edge. The loss of Luxol fast blue histochemistry was more profound than that of myelin proteins in immunohistochemistry, and T cell infiltration in ST-PCNSL exceeded that in MS by around fivefold (P = 0.005). Our data indicate that in the presence of extensive inflammation and incomplete, inhomogeneous demyelination, the neuropathologist should refrain from primarily considering autoimmune inflammatory demyelination and, even in the absence of lymphoma cells, instigate close clinical follow-up of the patient to detect recurrent lymphoma.
AB - The presence of inflammation and demyelination in a central nervous system (CNS) biopsy points towards a limited, yet heterogeneous group of pathologies, of which multiple sclerosis (MS) represents one of the principal considerations. Inflammatory demyelination has also been reported in patients with clinically suspected primary central nervous system lymphoma (PCNSL), especially when steroids had been administered prior to biopsy acquisition. The histopathological changes induced by corticosteroid treatment can range from mild reduction to complete disappearance of lymphoma cells. It has been proposed that in the absence of neoplastic B cells, these biopsies are indistinguishable from MS, yet despite the clinical relevance, no histological studies have specifically compared the two entities. In this work, we analyzed CNS biopsies from eight patients with inflammatory demyelination in whom PCNSL was later histologically confirmed, and compared them with nine well defined early active multiple sclerosis lesions. In the patients with steroid-treated PCNSL (ST-PCNSL) the interval between first and second biopsy ranged from 3 to 32 weeks; all of the patients had received corticosteroids before the first, but not the second biopsy. ST-PCNSL patients were older than MS patients (mean age: ST-PCNSL: 62 ± 4 years, MS: 30 ± 2 years), and histological analysis revealed numerous apoptoses, patchy and incomplete rather than confluent and complete demyelination and a fuzzy lesion edge. The loss of Luxol fast blue histochemistry was more profound than that of myelin proteins in immunohistochemistry, and T cell infiltration in ST-PCNSL exceeded that in MS by around fivefold (P = 0.005). Our data indicate that in the presence of extensive inflammation and incomplete, inhomogeneous demyelination, the neuropathologist should refrain from primarily considering autoimmune inflammatory demyelination and, even in the absence of lymphoma cells, instigate close clinical follow-up of the patient to detect recurrent lymphoma.
KW - Journal Article
U2 - 10.1111/bpa.12496
DO - 10.1111/bpa.12496
M3 - SCORING: Journal article
C2 - 28213912
VL - 28
SP - 225
EP - 233
JO - BRAIN PATHOL
JF - BRAIN PATHOL
SN - 1015-6305
IS - 2
ER -