scholarly journals NCMP-09. NEUROLYMPHOMATOSIS, A RARE INDEX MANIFESTATION OF CD5/CD10 NEGATIVE MATURE B CELL LYMPHOMA PRESENTING AS SUBACUTE SENSORIMOTOR LAMBOSACRAL POLYRADICULONEUROPATHY

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi181-vi181
Author(s):  
Maya Hrachova ◽  
Kong Xiao-Tang ◽  
Xiaohui Zhao

Abstract INTRODUCTION Neurolymphomatosis (NL) is a rare entity characterized by direct invasion of neoplastic cells into endoneurium of cranial or peripheral nerves, nerve roots or plexuses. It is most commonly seen with non-Hodgkin’s lymphoma with occurrence rate of 0.2%. Here we report a case of subacute length-dependent sensorimotor lumbosacral polyradiculoneuropathy secondary to NL as the first manifestation of CD5−/CD10− mature B-cell lymphoma. CASE REPORT: A 54-year old man with diabetes mellitus type 2, hypertension and psoriasis presented with subacute onset of progressive lower extremity weakness with pertinent examination findings of bilateral mildly asymmetrical distal greater than proximal lower extremities weakness, length depended hypoesthesia, fasciculations and decreased reflexes. EMG/NCS study showed active and chronic denervation changes concerning for multilevel lumbosacral polyradiculopathy. Routine blood investigations produced normal findings. CSF examination showed nucleated cells at 125 cells/HPF with lymphocyte predominance, protein at 538 mg/dL, and glucose 18 mg/dL. Flow cytometry identified mature CD5-/CD10- B cell lymphocytes. MRI spine identified marked enhancement, thickening, nodularity of cauda equine nerve roots and focus of myelopathy at the left posterior conus. Based on the clinical and radiographic findings a diagnosis of neurolymphomatosis was made and her underwent workup for systemic lymphoma. DISCUSSION Our case demonstrates the importance of comprehensive evaluation of sensorimotor polyradiculoneuropathy as rare entities such as neurolymphomatosis could mimic common etiologies.

2021 ◽  
pp. 199-201
Author(s):  
David N. Abarbanel ◽  
Ivan D. Carabenciov

A 78-year-old man sought care for saddle anesthesia, left lower extremity numbness, and bilateral lower extremity weakness. The sensory loss occurred suddenly, starting initially in the left perianal region and over the course of 3 hours extending down to involve the entirety of the left lower extremity. Symptoms were stable until 3 weeks later, when he had a few episodes of urinary incontinence. Diffuse, severe, bilateral, lower extremity weakness developed. The patient reported 6 months of intermittent night sweats. Serum studies were notable for pancytopenia and increased erythrocyte sedimentation rate and levels of ferritin and lactate dehydrogenase. Lumbar puncture showed a mildly increased protein concentration with normal blood cell count, glucose value, and cytologic and flow cytometry findings. Magnetic resonance imaging showed multifocal regions of increased T2 signal throughout the central nervous system including the cerebrum, cerebellum, upper cervical cord, lower thoracic cord, and conus medullaris. Gadolinium enhancement was present in the corpus callosum, cerebellum, and dorsal lower thoracic cord. One week later, 18F-fludeoxyglucose–positron emission tomography/computed tomography showed patchy 18F-fludeoxyglucose activity in the cerebral parenchyma, as well as 2 cutaneous, 18F-fludeoxyglucose-avid soft-tissue nodules. Fine-needle aspiration of 1 of these nodules indicated diffuse large B-cell lymphoma, with no dysplastic abnormalities identified on subsequent bone marrow biopsy. Incisional biopsy of the second soft-tissue nodule showed foci of diffuse large B-cell lymphoma adherent to the lumina of a few small arteries, consistent with a diagnosis of intravascular lymphoma. The patient was diagnosed with intravascular large B-cell lymphoma. At initial evaluation at an outside facility, empiric intravenous corticosteroids were administered. After the biopsy findings of intravascular large B-cell lymphoma, he was started on intermediate-dose methotrexate followed by rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone therapy. He continued to experience severe, bilateral, lower extremity weakness and sensory loss. Two months after diagnosis of intravascular large B-cell lymphoma, he died of medical complications from chemotherapy. Intravascular lymphoma is a rare lymphoma subtype that is typically of B-cell origin. The neoplastic cells preferentially grow within the lumen of blood vessels, potentially due to a lack of cellular machinery required for cellular extravasation and parenchymal invasion.


2016 ◽  
Vol 2016 ◽  
pp. 1-6
Author(s):  
Changxing Liu ◽  
Sean Delaney ◽  
Tamara N. Brown

Extranodal presentation of B-cell lymphoma is uncommon. Isolated primary epiglottic B-cell lymphoma is even rarer. To our knowledge, there has been only one description of isolated B-cell lymphoma presenting as a large epiglottic mass. We report an unusual type of B-cell lymphoma of the epiglottis, as it could not be subtyped based on routine staining and hybridization. The lymphoma presented as a large isolated globular mass pedicled to the epiglottis, occupying most of the oropharynx, but did not have any ball-valving effect or increased respiratory efforts. Initial radiographic findings were nonspecific. The diagnosis of B-cell lymphoma was determined by transoral incisional biopsy under local anesthesia. The condition was treated successfully with chemoradiation. The current standard of treatment for high grade B-cell lymphoma is concurrent chemoradiotherapy, with excellent prognosis. Although rare, B-cell lymphoma should be considered when investigating pedunculated hypopharyngeal masses.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii160-ii160
Author(s):  
Maya Hrachova ◽  
Mari Perez-Rosendahl ◽  
Xiao-Tang Kong

Abstract INTRODUCTION Primary Dural Based Marginal Zone B-Cell Lymphoma (MZBCL) is a subtype of PCNSL with an incidence rate ranging from 0.6 to 3% of all brain tumors. MZBCL typically presents as an extra-axial mass resembling meningioma. Here we report an unusual case of MZBCL with initial radiographic findings of mild nonspecific thickening tentorium and pachymeningeal enhancement surrounding 7th and 8th cranial nerves. CASE REPORT A 58-year old woman with clinical history of CMV infection, polyclonal gammopathy and unruptured left ICA aneurysm post coil embolization who presented for an evaluation of mild thickening and enhancement of the left tentorium cerebelli and 7th/8th nerve root complex. Differential considerations included inflammatory/autoimmune conditions (idiopathic hypertrophic pachymeningitis, neurosarcoidosis, Tolosa-Hunt syndrome), infections, structural lesion, benign or malignant neoplasm. Serum studies were normal. Multiple CSF studies were negative. Flow cytometry showed no malignant cells with few small lymphocytes. She was followed with the imaging surveillance for nine months until further increased thickening of dura on MRI. She underwent cerebellar dural biopsy that was consistent with MZBCL composed mainly of small CD20+ B-cells and negative MYD88. PET scan showed no systemic involvement. Bone biopsy revealed no evidence of lymphoma. The focal leptomeningeal enhancement improved significantly after she received four doses of systemic rituximab treatments. DISCUSSION Our case highlights the importance of surveillance and brain biopsy in cases of mild focal dura/pachymeningeal thickness and enhancement if no conclusive diagnosis has been established, as it might be one of the rare tumors such as MZBLC.


2022 ◽  
Vol 4 (1) ◽  
Author(s):  
Madalina Nussberger ◽  
Olaf Chan-Hi Kim ◽  
Sergio Cogliatti ◽  
Gautier Müllhaupt ◽  
Thomas Neumann

AbstractThe etiology of solid retroperitoneal mass may be autoimmune or neoplastic and should be investigated by imaging and histology. The spectrum of differential diagnoses includes retroperitoneal fibrosis and retroperitoneal tumors. As treatment for these entities differs substantially, early and accurate diagnosis is essential. We present a case of a 54-year-old woman admitted to our hospital with stroke-like symptoms. Suspecting vasculitis, magnetic resonance imaging of the head and abdomen was performed, which revealed circular enhancement of the internal carotid artery as well as retroperitoneal and periaortic masses. In light of the radiographic findings, an autoimmune process, such as retroperitoneal fibrosis, was hypothesized. Steroid treatment was initiated but did not lead to significant remission. Re-evaluation of the mass with fine-needle aspiration did not show malignant cells while diagnostic surgery and histological assessment revealed neoplastic lymphoproliferation. The final diagnosis was a non-Hodgkin B-cell lymphoma. Chemo- and immunotherapy were initiated. Follow-up abdominal computed tomography revealed significant remission of the retroperitoneal mass. Initially, the retroperitoneal mass was highly suspicious for RF. While imaging can be useful, obtaining histology should always be considered when there is an uncertain clinical presentation. Without histology, we would have missed a non-Hodgkin B-cell lymphoma in this case. Minimally invasive techniques such as fine-needle aspiration may be practical but can give false-negative results.


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