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Primary EBV-positive Hodgkin’s lymphoma of the CNS under azathioprine treatment

Case report and review of the literature

Primäres EBV-assoziiertes Hodgkin-Lymphom des ZNS unter Azathioprin

Fallbericht und Literaturübersicht

  • Case Study
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Abstract

Background

Retrospective and prospective cohort studies suggest that central nervous system involvement occurs in approximately 0.5 % of patients with advanced Hodgkin’s lymphoma. The isolated primary intracranial manifestation of Hodgkin’s lymphoma is an extremely rare finding, with few cases reported in the literature. Little is known about the optimal treatment and prognosis of these tumors. Here, we present a case report with a review of the literature.

Case presentation

A 47-year-old Caucasian man with persistent frontal headache and unspecific vertigo for half a month was diagnosed with nodular space-occupying lesions in the cerebellum. His medical history included multiple sclerosis, which was treated for 20 years with the immunosuppressive drug azathioprine. Further staging revealed no additional lesions suspected of being malignant. The patient underwent total tumor resection. Immunohistopathological examination showed Epstein–Barr virus-associated classic Hodgkin’s lymphoma. Diagnostic bone marrow punction excluded lymphoma involvement of the bone marrow. The patient had no B symptoms. Consequently, the patient was classified as having stage IEA disease according to the Modified Ann Arbor Classification of Hodgkin Lymphoma and received systemic chemotherapy followed by radiation therapy for the former cerebellar tumor region. He was in complete clinical remission atthe last follow-up 9 months after the initial diagnosis.

Conclusion

This case report and literature review suggest that multimodal treatment leads to a remarkable clinical outcome in Hodgkin’s lymphoma with intracranial involvement.

Zusammenfassung

Hintergrund

Retrospektive und prospektive Kohortenstudien deuten daraufhin, dass eine Beteiligung des zentralen Nervensystems (ZNS) in etwa bei 0,5 % der Patienten mit fortgeschrittenem Hodgkin-Lymphom auftritt. Die isoliert primär intrakranielle Manifestation des Hodgkin-Lymphoms ist extrem selten, mit wenigen bisher bekannten Fällen. Wenig ist auch über die optimale Therapie und Prognose dieser seltenen Entität bekannt. Wir präsentieren einen entsprechenden Fallbericht mit einer Literaturübersicht.

Fallbericht

Ein 47-jähriger Kaukasier mit persistierendem Stirnkopfschmerz und unspezifischem Schwindel präsentierte in radiologischen Untersuchungen mehrere noduläre Raumforderungen im Kleinhirn mit Kontakt zum 4. Ventrikel. Seine Krankengeschichte umfasste eine Multiple Sklerose, die seit 20 Jahren mit dem Immunsuppressivum Azathioprin behandelt wurde. Das weitere Staging ergab keine weiteren malignitätsverdächtigen Läsionen. Die tumorösen Läsionen wurden vollständig reseziert. Die Immunhistopathologie zeigte ein Epstein-Barr-Virus-assoziiertes klassisches Hodgkin-Lymphom. Die diagnostische Knochenmarkpunktion ergab keinen Hinweis auf eine Lymphombeteiligung. Der Patient zeigte keine B-Symptome. Somit wurde die Lymphomerkrankung des Patienten als Stadium IE A nach der modifizierten Ann-Arbor-Klassifikation für Hodgkin-Lymphome klassifiziert. Der Patient erhielt postoperativ eine Chemotherapie gefolgt von einer Bestrahlung der ehemaligen Tumorregion des Kleinhirns. Er erreichte eine Komplettremission, die in der letzten Follow-up-Untersuchung nach 9 Monaten weiter anhielt.

Schlussfolgerung

Dieser Fallbericht und die Literaturübersicht legen nahe, dass eine multimodale Behandlung zu guten klinischen Ergebnissen bei primär intrakraniellem Hodgkin-Lymphom führt.

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Conflict of interest

C. Henkenberens, A. Franzke, P. Raab, I. Oschlies, W. Klapper, and H. Christiansen state that there are no conflicts of interest.

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Authors and Affiliations

  1. Klinik für Strahlentherapie und Spezielle Onkologie, Medizinische Hochschule Hannover, Hannover, Germany

    Christoph Henkenberens MD & Hans Christiansen MD

  2. Klinik für Hämatologie, Hämostaseologie, Onkologie und Stammzelltransplantation, Medizinische Hochschule Hannover, Hannover, Germany

    Anke Franzke MD

  3. Institut für Diagnostische und Interventionelle Neuroradiologie, Medizinische Hochschule Hannover, Hannover, Germany

    Peter Raab MD

  4. Institut für Pathologie, Sektion Hämatopathologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel, Germany

    Ilske Oschlies MD & Wolfram Klapper MD

Authors
  1. Christoph Henkenberens MD
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  2. Anke Franzke MD
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  3. Peter Raab MD
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  4. Ilske Oschlies MD
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  5. Wolfram Klapper MD
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  6. Hans Christiansen MD
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Corresponding author

Correspondence to Hans Christiansen MD.

Additional information

The case report was presented at the 2013 DEGRO (“German Society for Radiooncology”) Annual Meeting in Berlin, Germany.

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Henkenberens, C., Franzke, A., Raab, P. et al. Primary EBV-positive Hodgkin’s lymphoma of the CNS under azathioprine treatment. Strahlenther Onkol 190, 847–852 (2014). https://doi.org/10.1007/s00066-014-0670-9

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