Primary Cerebral Lymphomatoid Granulomatosis Progressing to Methotrexate-associated Lymphoproliferative Disease Under Immunosuppressive Therapy

Tanaka, Hiroaki; Furukawa, Shogo; Takeda, Yusuke; Shimizu, Naomi; Kawaguchi, Takeshi; Kawajiri, Chika; Hashimoto, Shinichiro; Takagi, Toshiyuki; Ito, Shingo; Ota, Satoshi; Kuwabara, Satoshi; Nakaseko, Chiaki

doi:10.2169/internalmedicine.54.2399

Cited by 9 publications

(4 citation statements)

References 17 publications

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“…However, to the best of our knowledge, this is the first report of high-grade primary CNS-LYG with IgH monoclonality that was successfully treated with rituximab monotherapy, resulting in a durable remission for >12 months. Furthermore, our patient has survived for 17 months from diagnosis, whereas primary CNS-LYG patients usually survive only for <1 year from diagnosis ( 7 ). A case whereby rituximab and temozolomide immunochemotherapy was initiated on high-grade primary CNS-LYG with pulmonary involvement, the tumor progressed and the patient is reported to have died 2 months after the initial presentation ( 14 ).…”

Section: Discussionmentioning

confidence: 96%

“…Immunocompromise (iatrogenic, HIV-infection related, reflecting congenital immune deficiency, e.g.) makes it possible for the reactivation of EBV and the development of LYG ( 3 , 4 , 7 ). In our patient, HIV infection was not relevant and no repeated infection from childhood was observed, thus making congenital immune deficiency unlikely.…”

Section: Discussionmentioning

confidence: 99%

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High-grade Primary Central Nervous System Lymphomatoid Granulomatosis: Successful Rituximab Monotherapy

et al. 2021

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The primary central nervous system (CNS) presentation of lymphomatoid granulomatosis (LYG) is rare, and no standard therapy for LYG with primary CNS symptoms exists. CNS-LYG patients usually survive for only less than a year from diagnosis. This is the first report of high-grade primary CNS-LYG with monoclonality that was successfully treated with rituximab monotherapy, resulting in a durable remission for more than 1year in a 66-year-old woman with pemphigus vulgaris who was also on immunosuppressive therapy.

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

High-grade Primary Central Nervous System Lymphomatoid Granulomatosis: Successful Rituximab Monotherapy

et al. 2021

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“…However, as far as we searched, there had been no report on MTX–LPD in the orbital area, which was seen in this present case (5). Central nervous lesions were reported in eight cases and were in various sites (e.g., cerebellum, medulla oblongata, subdural, and so forth), except in the pituitary (14, 15, 16, 17, 18, 19). Although we were not able to histopathologically confirm the MTX–LPD etiology of the pituitary lesion in this case, the pathologic background of pituitary lesion and the other lesions were likely the same because the same pathologic subtype had been reported in biopsy specimens of the other lesions and the pituitary lesion developed simultaneously with the other lesions.…”

Section: Discussionmentioning

confidence: 99%

Methotrexate-associated lymphoproliferative disorder with hypopituitarism and central diabetes insipidus

Aoshima

Nagayama

Takeshita

et al. 2019

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Patients treated with immunosuppressive drugs, especially methotrexate (MTX), rarely develop lymphoproliferative disorders (LPDs), known as MTX-related LPD (MTX–LPD). The primary site of MTX–LPD is often extranodal. This is the first reported case of MTX–LPD in the pituitary. A 65-year-old woman was admitted to our hospital with symptoms of oculomotor nerve palsy and multiple subcutaneous nodules. She had been treated with MTX for 11 years for rheumatoid arthritis. Computed tomography showed multiple masses in the orbit, sinuses, lung fields, anterior mediastinum, kidney, and subcutaneous tissue. Brain magnetic resonance imaging revealed a sellar mass. She was diagnosed with hypopituitarism and central diabetes insipidus based on endocrine examination. Although pituitary biopsy could not be performed, we concluded that the pituitary lesion was from MTX–LPD, similar to the lesions in the sinuses, anterior mediastinum, and subcutaneous tissue, which showed polymorphic LPD on biopsy. MTX was discontinued, and methylprednisolone was administered to improve the neurologic symptoms. After several weeks, there was marked improvement of all lesions, including the pituitary lesion, but the pituitary function did not improve. When pituitary lesions are caused by MTX–LPD, the possibility of anterior hypopituitarism and central diabetes insipidus needs to be considered. Further studies are needed to investigate the effectiveness of early diagnosis and treatment of MTX–LPD in restoring pituitary dysfunction. Learning points Pituitary lesions from MTX–LPD may cause hypopituitarism and central diabetes insipidus. Pituitary metastasis of malignant lymphoma and primary pituitary lymphoma, which have the same tissue types with MTX–LPD, have poor prognosis, but the lesions of MTX–LPD can regress only after MTX discontinuation. In cases of pituitary lesions alone, a diagnosis of MTX–LPD may be difficult, unless pituitary biopsy is performed. This possibility should be considered in patients treated with immunosuppressive drugs. Pituitary hypofunction and diabetes insipidus may persist, even after regression of the lesions on imaging due to MTX discontinuation.

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“…Based on the follow-up MRI findings over time, the treatment prescribed here seems to have succeeded in ameliorating the lesion with no evidence of recurrence for more than 10 months. Tanaka et al reported a case in which CNS-LYG progressed to methotrexate-associated lymphoproliferative disease (MTX-LPD) under immunosuppressive therapy [17]. According to the authors, cerebral T2 high-intensity lesions with indefinite diagnosis despite initial biopsy had been primarily treated with prednisolone alone and subsequently, prednisolone combined with methotrexate (7.5 mg weekly) for a recurrence in the CNS.…”

Section: Tablementioning

confidence: 99%

A case of central nervous system lesion pathologically characterized by angiocentric, T-cell-rich lymphoid cell infiltrates: a case report and literature review

et al. 2021

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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 highdose 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.

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Primary Cerebral Lymphomatoid Granulomatosis Progressing to Methotrexate-associated Lymphoproliferative Disease Under Immunosuppressive Therapy

Cited by 9 publications

References 17 publications

High-grade Primary Central Nervous System Lymphomatoid Granulomatosis: Successful Rituximab Monotherapy

High-grade Primary Central Nervous System Lymphomatoid Granulomatosis: Successful Rituximab Monotherapy

Methotrexate-associated lymphoproliferative disorder with hypopituitarism and central diabetes insipidus

A case of central nervous system lesion pathologically characterized by angiocentric, T-cell-rich lymphoid cell infiltrates: a case report and literature review

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