Hodgkin Lymphoma Presenting with Dural Involvement

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HodgkinLymphoma (HL) is a B-cell neoplasm that rarely presents with central nervous system (CNS) extranodal manifestation. This presentation can occur as relapsed disease as well as at initial diagnosis. In this report, we present a patient with dural involvement of HL. Systemic chemotherapy with ABVD (doxorubicin, bleomycin, vinblastine, and decarbazine) in addition to intrathecal therapy (IT) with intrathecal chemotherapy may be a treatment option for patients with isolated dural involvement with CNS HL. There is no current consensus for treatment modality of CNS HL, however, our patient with dural disease was successfully treated with ABVD systemic chemotherapy and IT MTX and ARA-C and is now >12 months in an ongoing remission. The role of CNS penetrating agents for isolated dural involvement is unclear given it is not protected by the blood brain barrier (BBB). As evidenced by this case with appropriate therapy patients can achieve complete remission (CR) in CNS HL.

Hodgkin Lymphoma (HL) is a B-cell neoplasm that typically presents with enlarged lymph nodes and “B” symptoms. Many patients at presentation will have advanced disease and some will develop extranodal lesions. Central nervous system (CNS) involvement is an extremely rare extranodal manifestation of HL occurring at a frequency of 0.02-0.7% in patients. Although rare, there have been several cases describing CNS HL. The current knowledge of CNS HL and treatment approach is mostly derived from these case reports. Treatment has often included radiation, systemic chemotherapy, surgical resection, and combined modality therapy; however, no consensus has been reached about the best treatment option. Despite aggressive treatments, overall prognosis in patients with CNS HL is poor. In our case, we describe a patient with HL presenting with dural involvement.

A 38 year old male presented with a 5 week history of new onset headache. 

The patient underwent a magnetic resonance imaging (MRI) brain scan which demonstrated dural thickening, and enhancement along the anterior falx and the right tentorium (Figure 1A and D). Imaging was concerning for an infectious, inflammatory, or neoplastic process. He had a lumbar puncture, which was negative for infection and malignancy by flow cytometry and cytology. A bone marrow biopsy demonstrated hypocellularity with normal karyotype with no evidence of malignancy. The patient then underwent a computed tomography (CT) neck/chest/abdomen/pelvis and positron emission tomography (PET) scan, which demonstrated a single enlarged FDG-avid supraclavicular node (Figure 1G). The node was excised, and pathology demonstrated a lymph node with effaced architecture and nodules surrounded by thick collagen bands. These nodules were comprised of small lymphocytes with interspersed large mono- or binucleated Reed-Sternberg cells with prominent eosinophilic nucleoli and abundant light eosinophilic cytoplasm. There was focal retraction artifact present around the large cells, as well as scattered “mummified” cells. The Reed-Sternberg cells were CD15+ and CD30+, weakly PAX-5+, CD3-, CD20-, and CD45- . Background lymphocytes were composed of CD3+ T-cells and CD20+ B-cells. The excised lymph node pathology was diagnostic for nodular sclerosis classical HL (Figure 2). The patient was referred to oncology.