Primary intramedullary spinal cord lymphoma misdiagnosed as longitudinally extensive transverse myelitis: a case report and literature review

Abstract

Background Primary intramedullary spinal cord lymphoma (PISCL) is rare and easily misdiagnosed with the lack of typical clinical features and non-specific imaging manifestations. Case presentation A 49-year-old man was admitted to our hospital because of persistent limbs numbness, pinprick-like pain in the posterior neck and unsteady gaits. He has brisk tendon reflexes and positive Babinski’s sign. Magnetic resonance imaging (MRI) of the cervical spine showed an abnormal signal with aberrant reinforcement at medulla oblongata and the level of C1-C7. He was clinically diagnosed as longitudinally extensive transverse myelitis (antibody-negative). Steroid pulse therapy was administered and resulted in reduced symptoms. One month later, his situation was exacerbated compared to the onset. We launched a new cascade of steroid pulse therapy. But it did not improve his symptoms. Finally, the biopsy pathology confirmed PISCL. Chemotherapy, radiotherapy and zanubrutinib were administered and until now about 3 years into treatment the patient is still survival. Conclusions Based on our case and literature review, we recommend that spinal onset patients react ineffectively to standard immunoglobulins or hormonal treatments or experience a relapse after a short time relief should take PISCL into consideration.

Essentials for early diagnosis of primary intramedullary spinal cord lymphoma. How to suspect primary intramedullary spinal cord lymphoma early and proceed to invasive biopsy? A case report and literature review

“…[ 3 ] The PISCL subtype is an extremely rare pathology, accounting for <1.0% of PCNSLs. [ 1 , 3 , 4 , 7 , 10 ] Clinically, the disease is associated with progressive spinal cord symptoms and large spinal cord lesions on neuroimaging, and neuroimaging findings mimic other progressive neurological disorders such as other CNS tumors (e.g., malignant gliomas), demyelinating disease, autoimmune conditions, CNS infections, and various inflammatory lesions such as NMOSD. [ 7 , 11 ] MRI is the clinically preferred imaging modality for diagnosing PISCL.…”

“…[ 7 ] An invasive spinal biopsy is, therefore, necessary for accurate diagnosis. [ 1 , 3 , 7 , 11 ] Because the disease often responds to steroids in the early stages, clinicians are often hesitant to perform a biopsy, thus delaying diagnosis and treatment. [ 1 , 3 , 7 , 11 ] As reported by Hachicha et al ., the diagnosis previously took an average of approximately 16 months from symptom onset.…”

“…However, the low positive rate of cytology in cerebrospinal fluid (CSF) necessitates surgical biopsy for early and accurate diagnosis. [ 1 , 3 , 4 , 7 , 10 ] Here, we present a case of PISCL in which the diagnosis was confirmed by surgical biopsy, and we suggest useful indicators for the accurate diagnosis of this entity. Both 18 F-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) and levels of β2-microglobulin (β2-MG) in CSF appear very useful for rapid and accurate diagnosis, enabling early therapeutic intervention.…”

“…It is confined to the eye, brain, leptomeninges, and spinal cord, with no systemic involvement. [ 3 , 6 ] On the other hand, primary intramedullary spinal cord lymphoma (PISCL) is extremely rare, accounting for <1.0% of all malignant lymphomas that occur in the central nervous system (CNS). [ 10 , 11 ] The majority of patients present in their 50s, affecting primarily the chest and neck, with low long-term survival rates and high mortality rates.…”

“…[ 10 , 11 ] The majority of patients present in their 50s, affecting primarily the chest and neck, with low long-term survival rates and high mortality rates. [ 3 ] This pathology is associated with rapidly progressive spinal cord symptoms, so differentiation from various inflammatory diseases such as neuromyelitis optica spectrum disorders (NMOSDs) and neoplastic diseases such as malignant gliomas must be made as soon as possible. However, the low positive rate of cytology in cerebrospinal fluid (CSF) necessitates surgical biopsy for early and accurate diagnosis.…”