Fig 1 - uploaded by Ruozhuo Liu
Content may be subject to copyright.
Brain MRI. Multiple intracranial diffuse lesions show high signal intensity on axial T2-weighted images in the cortex and subcortical white matter of both the cerebral hemispheres, brain stem, and entire cerebellum (A-D) as well as punctate or linear enhancement on Gadolinium-perfused T1-weighted images (E-H) in some lesions.
Source publication
Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive lymphoproliferative disease which can involve multiple organs of the body and is most common in the lungs. Its pathological features are proliferation of large atypical B‐cells related to Epstein–Barr virus, T‐cell infiltration and tissue necrosis. This disease is rare, and L...
Context in source publication
Context 1
... magnetic resonance imaging (MRI) showed multiple lesions in the cortex and subcortical white matter of both sides of the frontal, temporal, parietal and occipital lobes, brain stem, entire cerebellum, and spinal cord. Some lesions demonstrated punctate or linear enhancement (Figs 1, 2B). Positron emission tomography-computed tomography (PET-CT) showed an increase in 18 fluorodeoxyglucose (FDG) metabolism in the right frontal lobe, parietal lobe and spinal cord at the C4 and T4 levels ( Fig. 2A). ...
Citations
... Los síntomas asociados con compromiso del SNC incluyen cefalea, trastornos de la conciencia, demencia, trastornos de la visión, neuropatía craneal, hemiplejía, ataxia y epilepsia [4]. La diseminación secundaria al SNC no es infrecuente (25-30%) [3], pero la afectación primaria y aislada del SNC (iCNS-LYG) es excepcional en la práctica clínica, con 49 casos reportados a nivel mundial hasta el 2019 [5]. A continuación, describimos el caso de una iCNS-LYG, en un paciente masculino de 21 años de edad. ...
... La LYG es una patología linfoproliferativa poco frecuente, en la cual la afectación primaria y aislada del SNC (iCNS-LYG) solo ha sido reportada en 49 ocasiones [5]. Se presenta con una mayor prevalencia en hombres que mujeres, con una relación 2:1, con una edad en promedio de 46.7 años, pero se ha presentado en rangos de edad entre los 16 y 74 años [5,6]. ...
... La LYG es una patología linfoproliferativa poco frecuente, en la cual la afectación primaria y aislada del SNC (iCNS-LYG) solo ha sido reportada en 49 ocasiones [5]. Se presenta con una mayor prevalencia en hombres que mujeres, con una relación 2:1, con una edad en promedio de 46.7 años, pero se ha presentado en rangos de edad entre los 16 y 74 años [5,6]. Las manifestaciones clínicas en la iCNS-LYG incluyen cefalea, diplopía, lesión de nervio craneal, epilepsia, deterioro cognitivo, ataxia y hemiplejía [5]. ...
La granulomatosis linfomatoide (LYG), es un trastorno linfoproliferativo angiocéntrico y angiodestructivo, el cual afecta múltiples órganos y es más común en los pulmones. Se caracteriza patológicamente por estar asociado con una proliferación de linfocitos B, relacionado con el virus de Epstein-Barr. La granulomatosis linfomatoide (LYG), afecta al sistema nervioso central de forma secundaria en un 25% a 30% de los casos, pero LYG que involucra de forma primaria y únicamente al SNC (iCNS-LYG) es extremadamente raro con solo 49 casos reportados en la literatura. Presentamos el caso de un joven de 21 años con iCNS-LYG, el cual se presenta con una manifestación clínica de su patología, su abordaje diagnóstico se realizó por medio de imágenes diagnósticas y se confirmó por medio de biopsia.
... It is rare for disease to be confined to the central nervous system (CNS). Recent review articles have found fewer than 50 reported cases [4]. ...
... Lesions are typically multifocal and cortically based. They may also have a diffuse appearance or involve subcortical white matter, basal ganglia, thalamus, sella, cerebellum, spinal cord, or cranial nerves, commonly causing cognitive decline, headache, and epilepsy [4]. ...
... Interestingly, our patient first presented with trigeminal and facial nerve involvement separated in time, later presenting as a mass. Combined diffuse and mass-like presentations have been reported in 6% of cases [4]. ...
Lymphomatoid granulomatosis is an Epstein-Barr virus-associated lymphoproliferative B-cell neoplasm that typically involves multiple organ systems. This disease is exceedingly rare when confined to the central nervous system (CNS), usually presenting as a mass lesion or diffuse disease, with no existing standard of care. We present the case of a 67-year-old patient who had a unique and insidious course of isolated CNS lymphomatoid granulomatosis. The disease first presented with cranial neuropathies involving the trigeminal and facial nerves that were responsive to steroids both clinically and radiographically. Two years later, the disease manifested as a parietal mass mimicking high-grade glioma that caused homonymous hemianopsia. The patient underwent craniotomy for resection and was treated with rituximab after surgery. The patient has achieved progression-free survival more than three years after the surgery. Surgical debulking and post-procedural rituximab resulted in favorable survival in a case of isolated CNS lymphomatoid granulomatosis. An intracranial mass preceded by steroid-responsive cranial neuropathies should raise suspicion for lymphoproliferative disorder.
... Multiple, sporadic, punctate infiltrative lesions and mass-like lesions are visualized on MRI scans of patients with CNS-LyG. A space-occupying effect with surrounding edema is obvious in mass-like lesions [14][15][16]. On contrastenhanced CT scans of patients with CNS-LyG, multiple punctate, linear, patchy, nodular and mass-like enhancing lesions can be observed, which may also evolve into irregular annular lesions. ...
Background
Lymphomatoid granulomatosis (LyG) is a rare extralymphatic lymphoproliferative disease characterized by lymphocytic invasion into vascular walls and damage to blood vessels. The lungs are affected in 90% of LyG cases, followed by the skin, central nervous system (CNS), kidneys and liver.
Case presentation
Here we report a case of a young woman with LyG, with CNS involvement as the initial clinical manifestation. Computer tomography (CT) scans showed multiple nodular, patchy and flocculent high-density shadows in both lungs without mediastinal lymph node enlargement. Magnetic resonance imaging (MRI) scans showed multiple abnormal signal intensities in the right cerebellar hemisphere, frontal, parietal and temporal lobes, and dorsal brainstem, which became patchy and annular after enhancement. The post-operative pathological analysis of lesion samples confirmed the diagnosis of grade II LyG.
Conclusions
LyG should be concerned in young adults showing multiple radiological brain and lung lesions. Resection and postoperative medication of steroid hormones and IFN-α may be effective in the treatment of LyG.
... Response to steroids, uveitis [192,204] Lymphomatoid granulomatosis Vasculitis, punctate enhancement [192,205] Meningeal carcinomatosis Cranial nerve involvement, leptomeningeal involvement, hypoglycorrachia [192] Metastases Brain mass or meningeal lesions [192] Meningioma Pachymeningeal involvement [192] Histiocytic diseases: Langerhans cell histiocytosis, Erdheim-Chester disease and Rosai-Dorfman syndrome Hypothalamic-pituitary involvement, brain mass lesions [90,192,206,207] Ependymoma Spinal cord involvement [192] Germinoma Hypothalamic-pituitary or optic nerve involvement [192] Glioma Brain mass lesion(s) [192] ...
Sarcoidosis is a systemic granulomatous disease of unknown cause characterized by a wide variety of presentations. Its diagnosis is based on three major criteria: a clinical presentation compatible with sarcoidosis, the presence of non-necrotizing granulomatous inflammation in one or more tissue samples, and the exclusion of alternative causes of granulomatous disease. Many conditions may mimic a sarcoid-like granulomatous reaction. These conditions include infections, neoplasms, immunodeficiencies, and drug-induced diseases. Moreover, patients with sarcoidosis are at risk of developing opportunistic infections or lymphoma. Reliably confirming the diagnosis of sarcoidosis and better identifying new events are major clinical problems in daily practice. To address such issues, we present seven emblematic cases, seen in our department, over a ten-year period along with a literature review about case reports of conditions misdiagnosed as sarcoidosis.
... It was enhanced by gadolinium in all cases, such as in brain LYG lesions. 2,12 In the MRI of CNS-LYG, punctate or linear enhancement 1,18 is a relatively characteristic feature. 1 Regarding the enhancement characteristics of spinal medullary lesions, one-third was diffuse massive, one-third was punctate, and onethird was the others. In a previous report, microhemorrhage by hypointensity of T2*weighted MRI is another characteristic feature of CNS LYG, 19 although there was no clear spinal lesion hemorrhage in our series. ...
... 2 LYG is characterized by small lymphocytes, plasma cells, histiocytes, and atypical lymphocytes along with areas with varying degrees of necrosis and vasculitis. 1 Because of the presence of predominant T cell infiltration, this disorder was initially thought to be a T cell disorder. 2 Subsequently, EBVencoded small ribonucleic acid expressed by atypical large B cells were identified by immunohistochemistry and combined immunohistochemistry with in situ hybridization. Additionally, LYG was redefined as a B-lymphocyte disease and related to the EBV infection. ...
... Additionally, LYG was redefined as a B-lymphocyte disease and related to the EBV infection. 2,20,21 According to the World Health Organization Classification of Lymphoid Neoplasms revised in 2016, LYG is ...
Objective
Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus-associated systemic angiocentric and angiodestructive lymphoproliferative disorder. It commonly involves the lungs and can also affect the skin, liver, kidney, and central nervous system. It can rarely occur in the spine, although the details are unclear. Here, we performed a systematic review of published cases (including our one case) of spinal LYG.
Methods
We performed a systematic search of studies in English on spinal LYG, focusing on its clinical features, imaging, and treatments, according to the PRISMA guidelines on the PubMed database. We identified 14 patients from the literature. We also found one case of isolated cervical LYG (grade 3) who was treated with steroid and radiation therapy for the spinal lesion after pathological diagnosis. We performed a pooled analysis of these 15 cases.
Results
The mean age was 43.4 years, and 13 patients were male out of the 15 cases. Brain lesions were present in 11 out of 12 intramedullary spinal lesions, and only 1 was an isolated spinal LYG case. Regarding the diagnostic methods, one case was not described. Of the 14 cases described, 12 cases were performed with biopsy (7 brain, 4 lung, and 1 spinal cord lesion biopsies), and 2 underwent surgical removal for an extramedullary lesion. In the overall prognosis from a mean follow-up period of 21.8 months, 4 cases died despite several treatments.
Conclusions
Spinal LYG is rare, particularly isolated spinal LYG. Thus, further accumulation of cases may be necessary to better understand its characteristics.
... La granulomatosis linfomatoide es un proceso linfoproliferativo de linfocitos B asociado al virus de Epstein-Barr (VEB), rodeado por un infiltrado de linfocitos T reactivos, con un patrón angiocéntrico y angiodestructivo [1][2][3] . Excepcionalmente, puede darse de forma aislada en el sistema nervioso central (SNC) [3][4][5] . ...
... La granulomatosis linfomatoide corresponde a una subclase de tumor de células B maduras 6 muy infrecuente 1,2,5,7 . Es más común en hombres entre los 40 y los 60 años [1][2][3][4][5]7 . Habitualmente afecta a inmunodeprimidos 1,2,7 , aunque también puede darse en inmunocompetentes 2,4,5 . ...
... debe diferenciarse del proceso linfoproliferativo postrasplante, proliferación de linfocitos B asociada también al VEB, pero que carece de infiltrado de linfocitos T reactivos y de necrosis 2,4,8,11,12 . En pacientes inmunocompetentes, la granulomatosis linfomatoide (lesión de componente necrótico) puede confundirse con el glioblastoma multiforme. ...
The term “primary CNS lymphoma” covers lymphomas that can occur either as solitary or as multifocal CNS lesions in primary intraparenchymal and meningeal locations. Thus, by definition, there is no co-existing systemic disease at the time of diagnosis, distinguishing these lesions from CNS involvement arising from systemic lymphomas (secondary CNS lymphomas). The 2021 WHO classification scheme includes the entities listed in Table 15.1. Despite impressive advances in the understanding of the etiology and pathogenesis of primary CNS lymphomas, the mainstay of their diagnostic assessment remains tissue-based classification using histological and immunohistochemical analysis of biopsy specimens. This chapter reviews the clinicopathological features and differential diagnosis of all these entities.
Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disease with angiocentric and angiodestructive infiltrates, and by definition, Epstein-Barr virus (EBV)-associated B-cell malignancy. It most frequently involves the lung, and in some cases, the lesions are confined to the central nervous system (isolated CNS-LYG). However, it remains a controversial disease in terms of pathophysiology, especially in those confined to the CNS. We report the case of a 37-year-old man with CNS lesion pathologically characterized by angiocentric, T-cell-rich lymphoid cell infiltrates that resembled CNS-LYG. The lesion was clinically aggressive with subacute onset and irregular ring-like enhancement on MRI. The resected specimen showed no cytological atypia, EBV-infected cells, or monoclonality for IgH and TCR gene rearrangements. Considering the possibility of latent malignancy, the patient was successfully treated with corticosteroid and chemoradiotherapy with high-dose methotrexate. The present case and the literature suggest that EBV-negative CNS lesions with angiocentric lymphoid infiltrates are probably heterogeneous in their pathogenesis, including those that could fit into the so-called CNS-LYG and those with T-cell predominance. The accumulation of similar cases is warranted for the classification and appropriate treatment of these lesions.
