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Marginal zone lymphoma (MZL) is the most common indolent lymphoma primarily arising in the central nervous system (CNS). To date, 207 cases of primary CNS MZL (PCNSMZL) were published, mostly as single case reports or small case series. It most commonly presents as extra-axial dural-based masses, more frequently in middle-aged women, displaying an insidious onset, with a long history of symptoms preceding the diagnosis. PCNSMZL can be radiographically mistaken for meningioma. PCNSMZL consists of CD20+ , CD3- small B lymphocytes with varying degrees of plasmacytic differentiation and low proliferation index. Trisomy 3, but not MALT1 or IgH translocation, is a common genetic abnormality. Other recurrent genetic abnormalities involve TNFAIP3 and NOTCH2. Ethiopathogenesis was poorly investigated. Due to its rarity, standard of care remains to be defined; it exhibits an excellent prognosis after varied treatments, such as surgery, radiotherapy, chemotherapy or their combinations. Nevertheless, each treatment should be considered after an accurate analysis of overtreatment risk. Short follow-up is a major limitation in reported PCNSMZL cases, which restrains our knowledge on long-term results and iatrogenic sequels. This review was focussed on presentation, differential diagnoses, pathological findings, treatment options and clinical outcomes of PCNSMZL; recommendations for best clinical practice are provided.
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Linfoma de Zona Marginal Tipo Células B , Pessoa de Meia-Idade , Humanos , Feminino , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/terapia , Linfoma de Zona Marginal Tipo Células B/genética , Linfócitos B/patologia , Tecido Linfoide/patologia , Translocação Genética , Sistema Nervoso Central/patologiaRESUMO
Meningiomas develop from meningothelial cells and approximately account for more than 30 percent of central nervous system (CNS) tumors. They can occur anywhere in the dura, most often intracranially and at dural reflection sites. Half of the cases are usually at parasagittal/falcine and convexity locations; other common sites are sphenoid ridge, suprasellar, posterior fossa, and olfactory groove. The female-to-male ratio is approximately 2 or 3-1, and the median age at diagnosis is 65 years. Meningiomas are generally extremely slow-growing tumors; many are asymptomatic or paucisymptomatic at diagnosis and are discovered incidentally. Clinical manifestations, when present, are influenced by the tumor site and by the time course over which it develops. Meningiomas are divided into three grades. Grade I represents the vast majority of cases; they are considered typical or benign, although their CNS location can still lead to severe morbidity or mortality, resulting in a reported ten-year net survival of over 80%. Atypical (WHO grade II) meningiomas are considered "intermediate grade" malignancies and represent 5-7% of cases. They show a tendency for recurrence and malignant degeneration with a relevant increase in tumor cell migration and surrounding tissue infiltration; ten-year net survival is reported over 60%. The anaplastic subtype (WHO III) represents only 1-3% of cases, and it is characterized by a poor prognosis (ten-year net survival of 15%). The treatment of choice for these tumors stands on complete microsurgical resection in case the subsequent morbidities are assumed minimal. On the other hand, and in case the tumor is located in critical regions such as the skull base, or the patient may have accompanied comorbidities, or it is aimed to avoid intensive treatment, some other approaches, including stereotactic radiosurgery and radiotherapy, were recommended as safe and effective choices to be considered as a primary treatment option or complementary to surgery. Adjuvant radiosurgery/radiotherapy should be considered in the case of atypical and anaplastic histology, especially when a residual tumor is identifiable in postoperative imaging. A "watchful waiting" strategy appears reasonable for extremely old individuals and those with substantial comorbidities or low-performance status, while there is a reduced threshold for therapeutic intervention for relatively healthy younger individuals due to the expectation that tumor progression will inevitably necessitate proactive treatment. To treat and manage meningioma efficiently, the assessments of both neurosurgeons and radiation oncologists are essential. The possibility of other rarer tumors, including hemangiopericytomas, solitary fibrous tumors, lymphomas, metastases, melanocytic tumors, and fibrous histiocytoma, must be considered when a meningeal lesion is diagnosed, especially because the ideal diagnostic and therapeutic approaches might differ significantly in every tumor type.
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Neoplasias Meníngeas , Meningioma , Radiocirurgia , Humanos , Masculino , Feminino , Idoso , Meningioma/cirurgia , Meningioma/diagnóstico , Neoplasias Meníngeas/cirurgia , Diagnóstico por Imagem , Cabeça , Resultado do TratamentoRESUMO
BACKGROUND: Clinical experience is limited for primary central nervous system (CNS) lymphoma that arises from the dura mater, which is denoted with the term primary dural lymphoma (PDL). This study was aimed at determining the relative incidence, presentation, and outcomes of PDL. METHODS: The institutional databases of the Divisions of Neuro-Oncology at the Massachusetts General Hospital and the Yale School of Medicine were retrospectively searched for patients with primary CNS lymphoma. Patients with pathologically confirmed dural lymphoma and no evidence of primary cerebral or systemic involvement were identified. Clinical data, diagnostic findings, treatments, and outcomes were recorded. RESULTS: A total of 20 patients with PDL were identified, and they represented 6.3% of the individuals with primary CNS lymphomas (20 of 316). Histopathological examination of PDL revealed the following underlying subtypes: diffuse large B-cell lymphoma (10 of 20 patients), marginal zone lymphoma (6 of 20), follicular lymphoma (2 of 20), undefined B-cell non-Hodgkin lymphoma (1 of 20), and T-cell non-Hodgkin lymphoma (1 of 20). On imaging, all tumors appeared as extra-axial masses with avid contrast enhancement and mostly mimicked meningioma. The median apparent diffusion coefficient value was 667 ± 26 mm2 /s. Cerebrospinal fluid analyses and symptoms were nonspecific, and the diagnosis rested on tissue analysis. Therapeutic approaches included surgery, radiotherapy, and chemotherapy. The median overall survival was not reached after 5 years. Three patients were deceased at database closure because of tumor progression. The extent of tumor resection correlated positively with overall survival (P = .044). CONCLUSIONS: PDL is a rare variant of primary CNS lymphoma that can be radiographically mistaken for meningioma. The outcome is excellent with multimodality treatment, and aggressive surgery may convey a survival advantage in select cases.
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Neoplasias do Sistema Nervoso Central/etiologia , Neoplasias do Sistema Nervoso Central/terapia , Linfoma/etiologia , Linfoma/terapia , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias do Sistema Nervoso Central/diagnóstico , Neoplasias do Sistema Nervoso Central/epidemiologia , Proteínas do Líquido Cefalorraquidiano/análise , Dura-Máter/patologia , Feminino , Humanos , Estimativa de Kaplan-Meier , Linfoma/diagnóstico , Linfoma/epidemiologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuroimagem , Prognóstico , Radiocirurgia , Resultado do TratamentoRESUMO
Primary dural lymphoma (PDL) constitutes a rare subgroup within central nervous system lymphomas, defined by its exclusive confinement to the dura mater, without a concurrent brain or systemic lymphatic involvement. This distinctive localization presents diagnostic challenges. In this report, we present a series of 3 cases where initial radiological presentations resembled meningiomas. We meticulously analyze key differentiating imaging characteristics, in CT, morphological MRI, and spectroscopy imaging. And show that recognizing and understanding these nuanced features are pivotal in enabling accurate differentiation of PDL and facilitating timely clinical intervention.
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BACKGROUND: Chronic inflammation of the thorax, as in tuberculosis-related pyothorax, can cause secondary malignant lymphomas. However, primary malignant lymphoma of the central nervous system, specifically of the dura mater, developing after intracranial infection or inflammation has rarely been reported. Herein, the authors describe a case of primary dural lymphoma that developed secondary to subdural empyema, with an initial presentation mimicking a chronic subdural hematoma. OBSERVATIONS: A 51-year-old man had undergone single burr hole drainage for subdural empyema 2 years prior. The patient subsequently underwent multiple craniotomy and drainage procedures, with successful remission of the subdural empyema. He was subsequently referred to the authors' hospital approximately a year after his initial treatment because of a recollection of subdural fluid, which was suspected to be recurrent empyema. After another single burr hole drainage, which revealed only a subdural hematoma, a histopathological diagnosis of B-cell lymphoma of the dural/subdural membrane was made. Subsequent radiation therapy was completed, with good local control and no recurrence of the subdural hematoma confirmed at 2 months posttreatment. LESSONS: Intracranial lymphoma triggered by chronic inflammation is rare but should be considered a differential diagnosis in subdural hematomas for which the background pathology is unclear. https://thejns.org/doi/10.3171/CASE24153.
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Primary dural Hodgkin lymphoma (PDHL) is an extremely rare subset of Hodgkin lymphoma (HL). Its existence is controversial, as Hodgkin lymphoma is not traditionally thought to arise from the central nervous system (CNS) or its meninges and only 0.02% of patients with Hodgkin lymphoma have any CNS involvement. We report a case of a 71-year-old Caucasian man who presented with progressive fatigue and sudden onset slurred speech, disorientation, and memory loss. Brain imaging identified a large extra-axial right frontal mass, and he underwent urgent subtotal resection. Pathology and subsequent workup revealed Stage IAE classical Hodgkin lymphoma of the right frontal dura, with no extra-cranial disease or leptomeningeal spread detected. The patient was subsequently treated with ABVD chemotherapy (completed 2.5 of 4 planned cycles) and 36 Gy in 20 fractions of consolidative involved-site radiotherapy (ISRT). He has been followed for 5 years with no clinical or radiological signs of recurrence. This is the second confirmed case of intracranial PDHL reported in the literature, with the longest follow-up for any case of PDHL.
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Primary central nervous system lymphoma (PCNSL) is an aggressive form of extranodal non-Hodgkin lymphoma that arises in the brain parenchyma, eyes, meninges, or spinal cord in the absence of systemic disease. Primary dural lymphoma (PDL), in contrast, arises from the dura mater of the brain. PDL is usually a low-grade B-cell marginal zone lymphoma (MZL), whereas other types of PCNSL are usually high-grade large B-cell lymphoma. This specific pathological subtype has important therapeutic and prognostic implications, making PDL a distinct subtype of PCNSL. Herein, we report a case of PDL in an African American patient, in her late thirties, who presented to our emergency room with chronic headaches. An emergent magnetic resonance imaging (MRI) of the brain showed a dural-based homogeneously enhancing extra-axial mass along the left hemisphere, which was contained within the anterior and parietal dural mater. A surgical specimen was collected after an emergency debulking procedure. The flow cytometry, done on the surgical specimen obtained, was positive for CD19+, CD20+, and CD22+, but negative for CD5- and CD10-. These findings were consistent with a clonal B-lymphoproliferative disorder. The surgical pathology specimen immunohistochemistry was positive for CD20+ and CD45+, but negative for Bcl-6Cyclin D1- and CD56-. The Ki67 was 10-20%. These findings were consistent with extranodal MZL. Given the location and pathology, the patient was diagnosed with PDL. Due to MZL's indolent nature, location outside the blood-brain barrier, and known efficacy to bendamustine-rituximab (BR), we decided to treat our patient with BR. She completed six cycles without major complications, and her post-therapy brain MRI showed complete remission (CR). Our case adds to the sparse literature about PDL and highlights the efficacy of BR systemic chemotherapy on MZLs.
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Primary central nervous system lymphomas (PCNSLs) account for 2%-3% of all primary brain tumors. Lymphomas arising from the dura, without brain and other systemic involvement, constitute a tiny fraction of this rare group. Most dural lymphomas present as dural-based masses and an acute presentation as a subdural hematoma is exceptional, with only five such cases reported previously in the literature. We present the case of a 65-year-old female who was admitted with complaints of slurring of speech, visual disturbances, and reduced appetite for 2 days. Computerized tomography scan showed a crescentic hyperdense area in the left fronto-temporo-parietal region with no bony or extracranial involvement suggestive of acute subdural hematoma. There was a history of a similar episode 3 months back, wherein she was operated upon and followed up conservatively. Craniotomy for drainage was performed, and histopathology revealed a non-Hodgkin's Lymphoma. Primary lymphoma of the dura is a rare subtype of PCNSL. This case is being reported for its unusual presentation as a common entity-an acute subdural hematoma.
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Primary central nervous system lymphomas (PCNLSs) are malignant non-Hodgkin lymphomas solely affecting the central nervous system (CNS). Here, we present a rare case of extra- and intracranial manifestation without adjacent calvarial infiltration. We report a 67-year-old woman who presented with right leg paresis and hypoesthesia, facial hypoesthesia, focal epileptic seizures, and an indolent tumor on the left parietal scalp. MRI showed a left paramedian extra- and intracranial contrast-enhancing tumor with infiltration of the superior sagittal sinus, but without osseous infiltration on CT. The tumor was radiologically suspected to be a meningioma and resection was performed. Histological examination, however, revealed a diffuse large B-cell lymphoma (DLBCL). Thus, the patient received adjuvant treatment according to the MATRix protocol. We provide a detailed analysis of this rare case with a focus on preoperative radiological findings and differential diagnoses. To the best of our knowledge, this is one of only four published cases of DLBCL with extra- and intracranial manifestation without bone affection.
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Lymphomas of the central nervous system (CNS) are broadly classified into primary CNS lymphoma (PCNSL) and secondary CNS lymphoma (SCNSL). PCNSL refers to lymphoma restricted to the brain, leptomeninges, spinal cord, or eyes without evidence of it outside the CNS at primary diagnosis, while SCNSL refers to secondary CNS involvement by systemic lymphoma. The brain is the most common site of involvement and intracranial CNS lymphoma has a highly variable imaging appearance and varies according to the patient's clinical profile and immunity. This pictorial essay aims to illustrate the different facets of intracranial CNS lymphomas on neuroimaging. This enables radiologists to be familiarized with their key diagnostic features and differentiate from their differential diagnoses, leading to early diagnosis, and treatment.
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Primary dural lymphoma is a rare subtype of primary central nervous system lymphoma. Primary dural lymphoma may be radiologically misdiagnosed as it shares similar imaging characteristics with several pathologies, including meningiomas and subdural or epidural hematomas. We present a patient who was originally diagnosed with a subdural hematoma following a syncopal episode on computed tomography. Follow-up magnetic resonance imaging of the brain demonstrated heterogeneously enhancing dural-based mass overlying the left frontoparietal convexity associated with bidirectional dural tails, suggestive of a malignant meningioma. Neurosurgical histopathology revealed marginal zone B-cell lymphoma. This case represents the potential difficulty in diagnosing primary dural lymphoma, especially in the setting of uncertain clinical history and obscured imaging features.
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Primary dural lymphoma (PDL) is an extremely rare subtype of primary central nervous system lymphoma arising from the dura mater in absence of systemic disease. The most common histological type is the low-grade marginal zone lymphoma, whereas high-grade lymphomas are unusual. We present a case of primary diffuse large B-cell lymphoma, presenting as PDL in the posterior fossa, originating from the dura mater of the petrous bone covering the surface of the left cerebellum, a location not previously described. A 65-year-old woman presented with sudden onset of severe dizziness was admitted in otolaryngology department then transferred to neurosurgery ward. CT scan revealed a large lesion involving left cerebellum, subsequent MRI of the brain demonstrated an enhancing mass suggestive for petrous bone meningioma. The tumor was excised, and the histopathological examination unexpectedly revealed a diffuse large B-cell lymphoma. The patient received postoperative chemoradiotherapy. 20 months after surgery a good outcome was registered. Due to the rarity of primary dural lymphomas no standard treatment is available, however, gross total or subtotal resection followed by adjuvant therapy seems to be a good choice to manage the pathology.
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Neoplasias Cerebelares/diagnóstico por imagem , Dura-Máter/diagnóstico por imagem , Neoplasias Meníngeas/diagnóstico por imagem , Meningioma/diagnóstico por imagem , Idoso , Neoplasias Cerebelares/cirurgia , Dura-Máter/cirurgia , Feminino , Humanos , Neoplasias Meníngeas/cirurgia , Meningioma/cirurgiaRESUMO
Primary central nervous system lymphomas (PCNSL) are extranodal non-Hodgkin's lymphomas (NHL) confined to the brain, leptomeninges, eyes, or spinal cord. Primary leptomeningeal lymphoma (PLML), without parenchymal involvement, synchronous cerebrospinal, or systemic disease is rare. The estimated incidence of PLML is 7% of all PCNSL, which in turn accounts for about 2% of all primary brain tumors and 0.8% of all lymphomas. The incidence of PCNSL in Western countries is approximately five cases per million inhabitants per year, and less than 5% of all primary tumors of the central nervous system (CNS), although it is worth mentioning that the incidence seems to be increasing. The largest series of cases reported in the medical literature collect information from no more than nine patients; in these series, the median age at diagnosis is 57 years; in general, all patients present with cerebrospinal fluid alteration, and the median overall survival rate is close to eight months. With our case series, we aim at sharing the experience of four patients diagnosed and treated at the National Cancer Institute between 2010 and 2020, establishing a correlation of the clinical, imaging, and histopathological presentation, the response to treatment based on radiotherapy and chemotherapy, and the clinical outcomes reported in the medical records.
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A standard radiation therapy protocol for primary central nervous system marginal zone lymphoma (CNS-MZL) has not been established. The International Lymphoma Radiation Oncology Group suggested a radiation therapy dose of 30-36 Gy for lesions of well-defined CNS-MZL. We report a case of relatively low-dose whole brain radiation therapy (WBRT) for ill-defined CNS-MZL. A 56-year-old man who presented with sudden left-sided convulsions and impaired consciousness was diagnosed with CNS-MZL. The tumor had an ill-defined lesion, without cerebrospinal fluid involvement. WBRT, consisting of 25.2 Gy in 14 fractions, was administered owing to the difficulty in target delineation for focal radiation therapy. No chemotherapy was administered during the treatment course. After the 36-month follow-up period, the patient maintained complete remission without neurological disorders. This report describes the usefulness of relatively low-dose WBRT for ill-defined CNS-MZL.
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Primary central nervous system (CNS) marginal zone B-cell lymphoma (MZBCL) arising from the dural meninges is a rare but indolent disease. This malignancy can present in various ways, hence making it difficult to diagnose. Biopsy results dictate an appropriate treatment plan, which commonly consists of a combination of surgical resection, whole brain radiotherapy and systemic therapy.
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BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a relatively common tumor of the central nervous system. Although PCNSLs generally arise from the parenchyma, lymphomas arising primarily from the dura are termed dural lymphomas (DLs). Mucosa-associated lymphoid tissue is the most unique histological feature of DLs. Because of its rarity, the clinical characteristics of and treatments for DL have not been fully elucidated to date. CASE DESCRIPTION: A 31-year-old man with no past medical history presented with numbness in his left upper limb. Magnetic resonance imaging revealed a dural-based tumoral lesion and cervical lymphadenopathies. The lesion was diagnosed radiologically as a meningioma, and tumor resection was planned. However, an intraoperative pathological diagnosis showed neoplastic lymphocytes, and the planned total tumor resection was halted. Histologically, the tumor was characterized by areas of poorly defined follicular architecture consisting of medium and large centroblasts. These tumor cells were immunohistologically positive for CD10 and CD20 and negative for B-cell lymphoma (BCL) 2 and BCL6. Fluorescence in situ hybridization did not show evidence of an Immunoglobulin H/BCL2 fusion. The lesion was subsequently diagnosed as a pediatric-type follicular lymphoma (PTFL). Six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) plus 2 cycles of rituximab were administered. The patient showed no evidence of relapse at 12 months after diagnosis, and follow-up was ongoing at the time of this report. CONCLUSIONS: This very rare case of DL was originally diagnosed as a PTFL. The tumor could be treated by immunochemotherapy alone.
Assuntos
Dura-Máter/diagnóstico por imagem , Linfoma Folicular/diagnóstico por imagem , Neoplasias Meníngeas/diagnóstico por imagem , Adulto , Anticorpos Monoclonais Murinos/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Humanos , Linfoma Folicular/tratamento farmacológico , Masculino , Neoplasias Meníngeas/tratamento farmacológico , Prednisona/administração & dosagem , Rituximab , Vincristina/administração & dosagemRESUMO
OBJECTIVE: To report a case of marginal zone MALT lymphoma of the temporal dura mater, initially mistaken for temporal meningioma. CASE REPORT: A 60-year-old immunocompetent woman, followed for more than 10 years for temporal meningioma causing vertigo and mixed hearing loss, presented with cervical lymphadenopathy, revealing marked progression of an intracranial lesion, leading to a diagnosis of marginal zone MALT lymphoma based on histological examination of a cervical lymph node. Treatment with 6 cycles of rituximab and bendamustine allowed complete remission of cervical lymph node and intracranial lesions, confirming the diagnosis of temporal dural mater lymphoma. CONCLUSION: Primary dural lymphoma must be part of the differential diagnosis of meningioma. Long-term follow-up allows correction of the diagnosis.
Assuntos
Dura-Máter/patologia , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Antineoplásicos Alquilantes/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Cloridrato de Bendamustina/uso terapêutico , Diagnóstico Diferencial , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Pessoa de Meia-Idade , Rituximab/uso terapêutico , Resultado do TratamentoRESUMO
Dural lymphoma (DL) is a rare type of primary CNS lymphoma arising from the dura mater. The optimal treatment is uncertain. A retrospective review was performed on 26 DL patients. Seventeen patients underwent resection and nine had a biopsy. Twenty three patients could be assessed for a response to treatment after surgery. Thirteen received focal radiotherapy (RT), six whole brain RT (WBRT), three chemotherapy alone and one chemotherapy followed by WBRT. Twenty two achieved complete response (CR) and one a partial response (PR). Four patients relapsed (two local and two systemic). Median follow up was 64 months, with median progression free survival (PFS) and OS not reached. Three year PFS was 89% (95% CI 0.64-0.97). All patients are alive at last follow-up, demonstrating that DL is an indolent tumor with long survival. CR is achievable with focal therapy in the majority of cases, but there is a risk for relapses and long-term follow-up is recommended.