RESUMO
Neurolymphomatosis (NL) is a rare neurologic manifestation of non-Hodgkin lymphoma (NHL) with poor prognosis. Investigations including MRI, PET/CT, nerve biopsy and cerebrospinal fluid (CSF) analysis can aid the diagnosis of NL. In this study, we presented a case of NL with co-existing myelin-associated glycoprotein (MAG) antibody. The patient first presented with symptoms of peripheral neuropathy involving multiple cranial nerves and cauda equina, and later developed obstructive hydrocephalus and deep matter lesions. He also had persistently positive MAG antibody, but did not develop electrophysiologically proven neuropathy and monoclonal immunoglobulin. The final brain biopsy confirmed diffuse large B cell lymphoma.
Assuntos
Glicoproteína Associada a Mielina , Neurolinfomatose , Humanos , Masculino , Neurolinfomatose/diagnóstico por imagem , Neurolinfomatose/diagnóstico , Glicoproteína Associada a Mielina/imunologia , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/complicações , Idoso , Autoanticorpos/sangue , Autoanticorpos/imunologia , Autoanticorpos/líquido cefalorraquidianoRESUMO
BACKGROUND: Primary central nervous system lymphoma is rare, and primary central nervous system T cell lymphoma is relatively uncommon, contributing to < 5% of all cases. Lymphomatosis cerebri, a rare subtype of primary central nervous system lymphoma, is characterized by extensive white-matter lesions on magnetic resonance imaging and nonspecific symptoms, such as cognitive decline and depression. Reports of lymphomatosis cerebri in adult T cell leukemia/lymphoma are limited. CASE PRESENTATION: A 49-year-old Japanese man gradually developed insomnia, anorexia, and weight loss over a 2-month period following work-related promotion. Initially diagnosed with depression, his condition rapidly deteriorated with cognitive decline and motor dysfunction. Despite various treatments, his symptoms persisted within a month. Upon admission, the presence of neurological abnormalities suggestive of a central nervous system disorder raised suspicion of a cerebral lesion. Diagnostic tests revealed extensive brain lesions on imaging and the presence of atypical lymphocytes (flower cells) in the cerebrospinal fluid. The patient was diagnosed with lymphomatosis cerebri due to adult T cell leukemia/lymphoma, a rare presentation in the literature. Due to irreversible brainstem damage and poor neurological prognosis, aggressive treatment was not initiated, and the patient died, with an autopsy confirming the diagnosis. CONCLUSION: Lymphomatosis cerebri with adult T cell leukemia/lymphoma is very rare. It is crucial to promptly consider lymphomatosis cerebri as a differential diagnosis, particularly in cases of rapid cognitive decline and poor treatment response. Recognition of lymphomatosis cerebri as an important differential diagnosis for cognitive decline, and depression is necessary for timely intervention and management. Further research is required to better understand this unique and rare presentation in adult T cell leukemia/lymphoma.
Assuntos
Neoplasias Encefálicas , Depressão , Leucemia-Linfoma de Células T do Adulto , Neurolinfomatose , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/etiologia , Depressão/diagnóstico , Depressão/etiologia , Diagnóstico Diferencial , Evolução Fatal , Leucemia-Linfoma de Células T do Adulto/diagnóstico , Leucemia-Linfoma de Células T do Adulto/complicações , Imageamento por Ressonância Magnética , Neurolinfomatose/diagnóstico , Neurolinfomatose/etiologiaRESUMO
RATIONALE: Primary adrenal lymphoma (PAL) is a very rare and highly aggressive disease. Neurolymphomatosis (NL) is a rare manifestation of lymphoma characterized by the infiltration of lymphoma cells into peripheral nerves, resulting in neurological symptoms. To date, there have been very few reported cases of PAL with NL. By reviewing the entire treatment process of the patient, we aim to enhance recognition of PAL complicated with NL and guide clinicians to pay attention to the diagnosis of such diseases. Early recognition and diagnosis of NL are crucial for appropriate management and treatment decisions. PATIENT CONCERNS: We report a case of PAL in a 64-year-old female whose initial symptoms were pain and weakness in the left leg, which progressively worsened. In the half month before admission, the patient also showed signs of cranial nerve damage, such as diplopia and facial asymmetry. DIAGNOSIS: Computed tomography of the abdomen revealed an occupying lesion in the left adrenal region. Electromyography and somatosensory evoked potential examination of the extremities suggested left lumbar plexus damage and complete damage to the right facial nerve. Adrenal biopsy confirmed diffuse large B-cell lymphoma. INTERVENTIONS: The patient was treated with the R-CHOP scheme (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) combined with lenalidomide. OUTCOME: After 6 rounds of chemotherapy, the symptoms improved slightly. However, the condition progressed, and the patient passed away 1 year later. LESSONS: Due to the nonspecific clinical presentation, patients with neurological damage should be alerted to the possibility of PAL and need to be evaluated thoroughly.
Assuntos
Linfoma Difuso de Grandes Células B , Neurolinfomatose , Feminino , Humanos , Pessoa de Meia-Idade , Neurolinfomatose/diagnóstico , Neurolinfomatose/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Rituximab/uso terapêutico , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/patologia , Vincristina/uso terapêutico , Ciclofosfamida/uso terapêutico , Prednisona/uso terapêutico , Doxorrubicina/uso terapêuticoRESUMO
Primary neurolymphomatosis is the direct infiltration of lymphomatous neoplastic cells into nerve roots and/or peripheral nerves. A 67-year-old man had a 24-month history of progressive and severe left lower limb neuropathic pain, ipsilateral ankle dorsiflexion weakness and gait disturbance. Gadolinium-enhanced MRI showed thickening and enhancement of the cauda equina, L5, S1 and S2 nerve roots. 18Fluorodeoxyglucose positron emission tomography showed concordant hypermetabolism. L5 nerve root biopsy confirmed diffuse large B-cell lymphoma. One cycle of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) chemotherapy resulted in remission, but this was not sustained. Primary neurolymphomatosis is rare and diagnostically challenging, and often the diagnosis is delayed. While biopsy is the gold standard for diagnosis, neuroimaging helps to characterise lesions and to determine the feasibility of biopsy.
Assuntos
Neurolinfomatose , Raízes Nervosas Espinhais , Humanos , Masculino , Neurolinfomatose/diagnóstico por imagem , Neurolinfomatose/patologia , Neurolinfomatose/diagnóstico , Idoso , Raízes Nervosas Espinhais/patologia , Raízes Nervosas Espinhais/diagnóstico por imagem , Biópsia/métodos , Imageamento por Ressonância Magnética , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Vincristina/uso terapêuticoRESUMO
BACKGROUND AND AIM: Neurolymphomatosis is defined as an infiltration of the peripheral nervous system (PNS) by malignant lymphoma cells. It is a rare entity and diagnosis is complicated especially when PNS involvement is the initial and leading symptom. To improve knowledge of the disorder and shorten the time to diagnosis, we report a series of nine patients without a history of hematologic malignancy, who were diagnosed with neurolymphomatosis after evaluation and workup of peripheral neuropathy. METHODS: The patients were included from the Department of Clinical Neurophysiology at Pitié Salpêtrière and Nancy Hospitals over a period of 15 years. Diagnosis of neurolymphomatosis was confirmed by histopathologic examination for each patient. We characterized their clinical, electrophysiological, biological, imaging, and histopathologic features. RESULTS: The neuropathy was characterized by pain (78%), proximal involvement (44%) or of all four limbs (67%), asymmetrical or with multifocal distribution (78%), abundant fibrillation (78%), a tendency to worsen rapidly, and significant associated weight loss (67%). Neurolymphomatosis was diagnosed principally on nerve biopsy (89%) identifying infiltration of lymphoid cells, atypical cells (78%), a monoclonal population (78%), and supported by fluorodeoxyglucose-positron emission tomography, spine or plexus MRI, cerebrospinal fluid analysis, and blood lymphocyte immunophenotyping. Six patients had systemic disease and three impairment limited to the PNS. In the latter case, progression could be unpredictable and may be diffuse and explosive, sometimes occurring years after a seemingly indolent course. INTERPRETATION: This study provides better knowledge and understanding of neurolymphomatosis when neuropathy is the initial presentation.
Assuntos
Neoplasias Hematológicas , Neurolinfomatose , Doenças do Sistema Nervoso Periférico , Humanos , Neurolinfomatose/diagnóstico , Neurolinfomatose/patologia , Sistema Nervoso Periférico/patologia , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/diagnóstico , Tomografia por Emissão de PósitronsRESUMO
Neurolymphomatosis (NL) is a rare condition caused by the lymphomatous or leukemic infiltration of nerves and manifests as neuropathy. Most often, NL is associated with B-lineage non-Hodgkin lymphoma (NHL) and only infrequently occurs in conjunction with T- or NK-lineage NHL. Extranodal NK/T-cell lymphoma (ENKTL)-associated NL is exceedingly unusual, with only 9 cases described in the English language literature, in addition to our case. Diagnosis of NL is challenging, as the entity can mimic neuropathies of more common etiologies, and an adequate biopsy may be difficult to obtain. Timely diagnosis demands a high index of suspicion, especially for patients without a history of hematologic malignancy. We expand upon a unique case of NL exclusively involving cranial nerves and cauda equina nerve roots, as the initial manifestation of ENKTL, and contextualize our findings within the framework of previously reported NK/T-lineage NL cases.
Assuntos
Nervos Cranianos , Linfoma Extranodal de Células T-NK/diagnóstico , Neurolinfomatose/diagnóstico , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-IdadeAssuntos
Linfoma Extranodal de Células T-NK , Células T Matadoras Naturais , Neurolinfomatose , Biópsia , Humanos , Células Matadoras Naturais/patologia , Linfoma Extranodal de Células T-NK/diagnóstico , Linfoma Extranodal de Células T-NK/patologia , Células T Matadoras Naturais/patologia , Neurolinfomatose/diagnóstico , Neurolinfomatose/etiologia , Neurolinfomatose/patologiaRESUMO
BACKGROUND: To report our experience with a group of patients referred for refractory CIDP who fulfilled "definite" electrodiagnostic EFNS criteria for CIDP but were found to have an alternate diagnosis. METHODS: Patients who were seen between 2017 and 2019 for refractory CIDP that fulfilled "definite" electrodiagnostic ENFS criteria for CIDP, but had an alternate diagnosis, were included. Patients who correctly had CIDP, anti MAG neuropathy, or MMN with conduction block, were excluded from the study. Demographics, clinical and electrophysiological characteristics, pertinent workup, final alternate diagnoses, and outcomes were collected. RESULTS: Seven patients were included: POEMS (n = 5), CANOMAD (n = 1), and neurolymphomatosis (n = 1). Most patients reported neuropathic pain and leg swelling (n = 6) or significant weight loss (n = 4). All patients had a monoclonal protein, and most patients who were tested had an elevated VEGF and CSF cyto-albuminologic dissociation. Electrophysiology showed pronounced intermediate more than distal demyelination, and axonal loss in the lower extremities. Response to steroids or IVIG varied, but some patients did respond to these treatments, especially early in the disease. CONCLUSION: Pain, systemic symptoms, suggestive electrophysiological findings, and/or a serum monoclonal protein should raise suspicion for CIDP mimics. Initial response to steroids or IVIG, over reliance on CSF, and electrophysiology findings can all be misleading.
Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Ataxia/diagnóstico , Neurolinfomatose/diagnóstico , Oftalmoplegia/diagnóstico , Síndrome POEMS/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
This series characterises nine patients with neurohistopathologically proven peripheral nerve neurolymphomatosis. A search of the hospital neuropathology database from 2002 to 2019 identified biopsy proven cases. Clinical data, investigation modalities, treatments, and outcomes were collated. Median age at neuropathy onset was 47 y, the neuropathy commonly as the initial lymphoma disease manifestation. Most (8/9) presented with painful asymmetrical sensory disturbance, with additional cranial nerve involvement in three. Neurophysiology typically demonstrated multiple axonal mononeuropathies. Cerebrospinal fluid protein was often raised (6/8). Magnetic resonance imaging suggested peripheral nerve infiltration in 6/9 and positron emission tomography CT in 4/9. Bone marrow biopsy was abnormal in 6/8. Treatment involved systemic or intrathecal chemotherapy and radiotherapy. Median survival was 23 mo. Neurolymphomatosis is a rare but important cause of neuropathy, particularly in those lacking systemic evidence of lymphoma as correct aggressive treatment can prolong survival. Nerve biopsy is essential to classify lymphoma type and rule out alternatives.
Assuntos
Neurolinfomatose/diagnóstico , Neurolinfomatose/terapia , Neoplasias do Sistema Nervoso Periférico/diagnóstico , Neoplasias do Sistema Nervoso Periférico/terapia , Adulto , Idoso , Biópsia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Condução Nervosa , Neurolinfomatose/patologia , Neoplasias do Sistema Nervoso Periférico/patologia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Neurolymphomatosis, the infiltration of the peripheral nervous system from lymphoid cells, represents an uncommon manifestation of lymphomas. We describe the challenging diagnostic work-up in a patient with neurolymphomatosis. A 58-year-old woman with previous breast diffuse large B-cell lymphoma treated with chemo- and radiation-therapy, presented with dysesthesias, neuropathic pain at left abdomen and thigh, and weakness at left lower limb 9 years after disease remission. Neurophysiology revealed left T10-L4 radiculo-plexopathy with no abnormalities at cerebrospinal fluid (CSF), nerve ultrasound, and 18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT). MR-neurography disclosed left rectus abdominis muscle atrophy, neurogenic edema, and denervation. Radiation-induced damage, paraneoplastic, infectious radiculo-plexopathies, and atypical chronic inflammatory demyelinating polyradiculoneuropathy were ruled out. Neurolymphomatosis was suspected, and the patient treated with rituximab with improvement. Despite treatment, the radiculo-plexopathy eventually extended to the right side and sacral roots. Later in the disease course, sural nerve biopsy confirmed the diagnosis. Maintenance therapy was continued, until cutaneous localizations occurred, requiring salvage therapy and autologous stem cell transplant. Although rare, neurolymphomatosis should be considered in all patients with lymphomas and unexplained peripheral nervous system involvement. Hematological, CSF, and neuroimaging findings may be unremarkable, and a high index of suspicion required in order to achieve the diagnosis.
Assuntos
Neoplasias da Mama/complicações , Linfoma Difuso de Grandes Células B/complicações , Neurolinfomatose/diagnóstico , Neurolinfomatose/etiologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Neurolinfomatose/patologia , Tomografia por Emissão de PósitronsAssuntos
Linfoma de Burkitt , Doenças dos Nervos Cranianos , Neurolinfomatose , Idoso , Linfoma de Burkitt/complicações , Linfoma de Burkitt/diagnóstico , Doenças dos Nervos Cranianos/complicações , Doenças dos Nervos Cranianos/diagnóstico , Feminino , Humanos , Neurolinfomatose/complicações , Neurolinfomatose/diagnósticoRESUMO
Primary adrenal lymphoma (PAL) is rare and known to have a predilection for central nervous system (CNS) relapse. A 70-year-old man with a 2-year history of primary aldosteronism presented because of a fever. He was hypotensive, and his adrenal glands were unequivocally enlarged. PAL was diagnosed. Despite showing an initial response to immunochemotherapy, progressive paralysis ensued. Magnetic resonance imaging findings were negative, and rituximab was ineffective. His debilitated condition hindered further chemotherapy. A postmortem examination revealed lymphoma relapse in the systemic peripheral nerves. The sequential presentation of two rare lymphomas implies that PAL might have a predilection for not only the CNS but also peripheral nerves.
Assuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/tratamento farmacológico , Antineoplásicos Imunológicos/uso terapêutico , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Neurolinfomatose/diagnóstico , Neurolinfomatose/tratamento farmacológico , Rituximab/uso terapêutico , Idoso , Evolução Fatal , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/tratamento farmacológicoRESUMO
INTRODUCTION: Malignant lymphocytic infiltration of the central nervous system (CNS) and peripheral nervous system (PNS) is diagnostically challenging and informs treatment and prognosis. CASE REPORT: We describe the clinical course of a 49-year-old man with CNS and PNS relapse of mantle cell lymphoma and the diagnostic modalities that enabled the diagnosis of neurolymphomatosis. CONCLUSION: This clinical phenotype reinforces previously reported presentations of neurolymphomatosis and the ability of multimodal diagnostics, when combined with clinical suspicion phenotype, to enable diagnosis of malignant lymphocytic infiltration of the CNS and PNS.
Assuntos
Neurolinfomatose/diagnóstico , Eletrodiagnóstico/métodos , Humanos , Linfoma de Célula do Manto/complicações , Masculino , Pessoa de Meia-Idade , Neurolinfomatose/complicações , Neurolinfomatose/diagnóstico por imagem , Radiografia/métodos , RecidivaAssuntos
Neuropatias do Plexo Braquial/diagnóstico , Linfoma Difuso de Grandes Células B/diagnóstico , Neurolinfomatose/diagnóstico , Idoso , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Imuno-Histoquímica , Linfoma Difuso de Grandes Células B/complicações , Imageamento por Ressonância Magnética , Neurolinfomatose/secundárioRESUMO
BACKGROUND: Neurolymphomatosis (NL) is a direct process of invasion of peripheral nerves by lymphoma. It occurs in roughly 5% of patients with lymphoma and represents a particularly difficult diagnostic dilemma when it is the presenting focal manifestation of occult lymphoma. CASE PRESENTATION: We present 3 examples of invasion of the lumbosacral plexus and its branches. These cases demonstrate a protean clinical picture with regards to the time relationship to the clinical course of lymphoma and the neuroanatomical extent of lumbosacral plexus invasion. We demonstrate the complementary role of different imaging modalities. A review of the literature summarizes 23 reports where lumbosacral plexus invasion was the index manifestation, at the time of first diagnosis or recurrence of lymphoma. This series confirms the strong preponderance of B-cell type (92%). There is a marked predilection for involvement of the sciatic nerve (74%), either focally or in a longitudinally extensive fashion, from the ischium to the popliteal fossa. There can also be restricted and discrete involvement of tibial and fibular branches. In recent years, ultrasound and CT have been given a more limited role, as screening tools or as a guide for biopsy. MRI neurography and PET-CT have become leading diagnostic modalities for diagnosis, staging and assessment of treatment response. CONCLUSION: The diagnosis of NL may be challenging, and it was once only reached at autopsy. Improved diagnostic imaging of focal or even asymptomatic disease offers new hope for earlier diagnosis and successful targeted therapy.
Assuntos
Plexo Lombossacral/patologia , Neurolinfomatose/diagnóstico , Idoso , Biópsia , Feminino , Fluordesoxiglucose F18 , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neurolinfomatose/terapia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , UltrassonografiaRESUMO
Neurolymphomatosis (NL) is an uncommon clinical condition, characterised by lymphomatous infiltration of the central and/or peripheral nervous system. Most often it is caused by B-cell non-Hodgkin's lymphoma (NHL). Clinically, patients usually present with neuropathy involving the nerve roots, plexuses, peripheral or cranial nerves. NL usually occurs as a complication of prior lymphoma, but it can also present in the form of relapsed lymphoma. It is important to diagnose and start early treatment in all cases of nodal or visceral (including neural) lymphoma with chemo and/or radiation therapy. The PET-CT and MRI can help in making diagnosis. We are presenting a case of 28-year male patient, diagnosed as diffuse large B-cell lymphoma on the background of follicular lymphoma, which initially responded to treatment but then presented with NL, based on clinical history and radiological findings which were confirmed by histopathology.
Assuntos
Neurolinfomatose/diagnóstico , Nervo Isquiático , Nervo Tibial , Adulto , Antineoplásicos/uso terapêutico , Biópsia , Diagnóstico Diferencial , Humanos , Imageamento por Ressonância Magnética , Masculino , Neurolinfomatose/tratamento farmacológico , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , UltrassonografiaAssuntos
Antineoplásicos Imunológicos/uso terapêutico , Linfoma de Burkitt/diagnóstico , Dor Facial/diagnóstico por imagem , Metotrexato/uso terapêutico , Neurolinfomatose/diagnóstico , Ponte/fisiopatologia , Rituximab/uso terapêutico , Nervo Trigêmeo/fisiopatologia , Linfoma de Burkitt/tratamento farmacológico , Linfoma de Burkitt/fisiopatologia , Quimioterapia Adjuvante , Descompressão Cirúrgica , Dor Facial/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neurolinfomatose/tratamento farmacológico , Neurolinfomatose/fisiopatologia , Parestesia/etiologia , Ponte/diagnóstico por imagem , Resultado do Tratamento , Nervo Trigêmeo/diagnóstico por imagemRESUMO
An 8-y 9-mo-old male Pug dog was presented because of anorexia, hindlimb ataxia, vomiting, and progressive weight loss. Clinical examinations revealed atrophic hindlimb muscles with decreased postural reaction, enlargement of the cardiac silhouette and megaesophagus on radiograph, and reduced cardiac contractility on ultrasonography. The dog died 10âd after the initial examination, and an autopsy was performed. Grossly, the heart was enlarged, with multifocal-to-coalescing extensive plaque-like areas of discoloration on the epicardial surface. On cross-section, the ventricles were moderately dilated, and discoloration extended into the myocardium. Peripheral lymph nodes were of normal size. Histologically, atypical lymphoid cells replaced the myocardium and also extensively infiltrated peripheral nerve bundles in various organs. With immunohistochemistry, the neoplastic cells exhibited strong immunoreactivity for CD3 and CD20, and were negative for CD8, granzyme B, CD79α, and Pax5. Double-label immunofluorescence confirmed co-expression of CD3 and CD20 by the neoplastic cells. Molecular clonality analysis presented a clonal T-cell receptor gamma gene rearrangement. The case was diagnosed as a CD3+/CD20+ peripheral T-cell lymphoma with prominent cardiac and peripheral nerve involvement, indicating neurolymphomatosis.