Isolated CNS Hodgkin’s lymphoma: implications for tissue diagnosis

Abstract

CNS involvement in the setting of lymphoid neoplasia is a clinical situation that requires specific diagnosis due to the disparate treatment regimens recommended for neoplasms of specific lymphoid cell types. Cerebrospinal fluid (CSF) sampling may provide sufficient information to determine the presence of abnormal lymphoid cells but may not be able to further specify the malignant cellular population. In cases where abnormal clinical or radiographic features are present, accurate tissue diagnosis is essential. In this report, we define a rare case of primary CNS intramedullary Hodgkin's lymphoma without leptomeningeal dissemination diagnosed via resectional biopsy of a conus medullaris lesion. The patient received post-resection radiation therapy and subsequently demonstrated radiographic and clinical improvement. Lymphoid neoplasia within the CNS comprises a diverse group with varying response and survival rates. Treatment hinges upon accurate diagnosis as chemotherapy varies widely among Hodgkin's and non-Hodgkin's lymphoma. While CSF sampling may yield a positive result with sufficiency to diagnose an abnormal lymphoid cell population, tissue is necessary for further defining cellular pathology. In this report, we define a rare case of primary CNS intramedullary Hodgkin's lymphoma without leptomeningeal dissemination via resectional biopsy of a conus medullaris lesion. In cases where abnormal enhancement is found in eloquent CNS regions and lymphoid neoplasia is suspected, management often entails either stereotactic biopsy or CSF sampling. While CSF analysis may differentiate malignancy at a low rate, tissue diagnosis via paraffin block immunohistochemistry is necessary to further classify malignancy as primary or peripheral, Hodgkin's or non-Hodgkin's lymphoma, or other such as metastatic leptomeningeal dissemination and glioma. Within the subtypes of lymphoid neoplasms, treatment regimens vastly differ and thus accurate tissue diagnosis is paramount. We therefore present a rare case of primary CNS intramedullary Hodgkin's lymphoma without leptomeningeal disease in the setting of immunocompromise diagnosed via open resectional biopsy of the conus medullaris.

Primary intradural Hodgkin lymphoma of the conus medullaris and cauda equina: case report

“…Primary intracranial HL has been previously documented in the literature [ 21 ], and spinal extradural lesions have also been reported [ 22–26 ]. Primary HL of the conus medullaris has been reported once previously [ 27 ]; however, this case was not isolated to the spine, with evidence of brainstem involvement found on neuroimaging.…”

Hodgkin Lymphoma in Children and Adolescents: Advances in Pathology, Diagnosis, and Treatment Strategies

“…The study concluded that RT can be safely omitted in early stage HL in CR following CTR. RT with (20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35) proved to be sufficient in patients with <CR following CTR. [45] Radford et al reported the outcome of the randomized phase III trial to determine the role of FDG-PET imaging in clinical stages IA/IIA HL (The RAPID study).…”

“…Therefore, tissue sampling is required to make accurate diagnosis whenever possible. [24] Van Blydenstein et al from the University of Witwatersrand in South Africa published a case report of a 12-year-old boy with a history of painless left inguinal swelling and acute diplopia. An intensely enhancing lesion in the right midbrain was detected on MRI scan.…”

Intracerebral manifestation of iatrogenic, immunodeficiency-associated polymorphic B-LPD with morphology mimicking Hodgkin lymphoma: a case report and literature review

“…Among the 24 published reports on primary, isolated CNS manifestation of cHL between 1980 and 2020 , three were diagnosed in patients with underlying autoimmune diseases and long-term immunosuppressive treatment. Therefore, these cases fall into the same WHO category of "other iatrogenic immunodeficiency-associated lymphoproliferative disorders" (Table 1) [18,27,28] . Two patients (66 and 47 years) with myasthenia gravis had been treated with azacytidine for 12 and > 20 years, and the other patient (74 years) had received long-term treatment with methotrexate and infliximab for rheumatoid arthritis.…”