Amyloidoma of the CNS.

Cohen, Mark L.; Lanska, Douglas J.; Roessmann, Uros; Karaman, Boris A.; Ganz, Edward; Whitehouse, Peter J.

doi:10.1212/wnl.42.10.2019

Cited by 22 publications

(5 citation statements)

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“…Typically, scattered and small groups of plasma cells occur in the amyloid deposits. Localized AL amyloid can occur at virtually any site of the body, including the brain (26). Most common sites are eyelids, larynx, bronchi, skin, and urinary tract.…”

Section: Al Amyloidosismentioning

confidence: 99%

Localized AL amyloidosis: A suicidal neoplasm?

Westermark¹

2012

Upsala Journal of Medical Sciences

102

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Although AL amyloidosis usually is a systemic disease, strictly localized AL deposits are not exceptionally rare. Such case reports form a considerable body of published articles. Although both AL amyloidosis types are formed from an N-terminal segment of a monoclonal immunoglobulin light chain, a typical localized AL amyloid differs from the systemic counterpart by the morphological appearance of the amyloid, and presence of clonal plasma cells and of giant cells. In this article it is pointed out that localized AL amyloidosis (‘amyloidoma') represents a true plasma cell neoplasm and not a pseudotumor. The pathogenesis of localized AL amyloidosis may differ from that of the systemic type, a suggestion underlined by the fact that localized AL amyloidosis of kappa type is as common as that of lambda origin, in contrast to the systemic form where lambda chains constitute the overwhelming majority of cases. It is suggested that oligomeric assemblies of the produced immunoglobulin light chain are toxic to plasma cells, which in this way commit suicide.

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Section: Al Amyloidosismentioning

confidence: 99%

Localized AL amyloidosis: A suicidal neoplasm?

Westermark¹

2012

Upsala Journal of Medical Sciences

102

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“…Diagnosis depends on histological demonstration of amyloid in tissue with Congo red staining and subsequent differentiation between AA (secondary) and AL types. In most of the literature cases, the protein subunit of the amyloid was AL lambda [11, 16]. In our case, the amyloid type was determined immunohistochemically as AL lambda.…”

Section: Discussionmentioning

confidence: 62%

“…Laeng et al have theorized that amyloidomas are neoplasms of AL (primary) lambda producing B cell clone capable of terminal differentiation in reaction to unique antigens [15]. Cohen et al suggested that genetically predisposed microglia might be the source of amyloid when challenged by certain antigens [7, 16]. Diagnosis depends on histological demonstration of amyloid in tissue with Congo red staining and subsequent differentiation between AA (secondary) and AL types.…”

Section: Discussionmentioning

confidence: 99%

Trigeminal Amyloidoma: Case Report and Review of Literature

Hashmi

Dhanoa

Manapuram

2018

Cureus

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Amyloid is an abnormal insoluble protein that can deposit in extracellular space. It can involve nearly any organ system and may manifest as a systemic process or focal lesion (amyloidoma). We present a rare case of localized amyloidosis with trigeminal nerve being the only site of involvement and no evidence of systemic disease. We also review literature relevant to trigeminal amyloidoma and make recommendations for diagnosis and treatment.

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“…Hence cerebral amyloidoma is a rare phenomenon [6]. However, Cohen et al postulated that genetically predisposed microglia might be stimulated to produce amyloid protein when challenged by certain antigens [7].…”

Section: Discussionmentioning

confidence: 99%

“…The histopathology of amyloidoma has been described as amyloid protein deposition with a surrounding cell infiltrate composed of monotypic kappa or lambda producing plasma cells without cytologic atypia, and few mature T lymphocytes and macrophages of the foreign body type. Apple-green birefringence under polarized light and Congo red staining is a pathognomonic feature that is universal to all forms of amyloid [6][7].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Cerebral Amyloidoma Mimicking a Hemorrhagic Malignant Central Nervous System Neoplasm

Kapoor

Gowda

Kaur

et al. 2020

Cureus

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Cerebral amyloidoma is a rare form of amyloidosis with a localized tumor like an amyloid deposition in the brain composed of insoluble fibrillary protein with cross beta-sheet conformation. Its usual presentation includes vision loss, seizures, behavioral changes, cognitive decline, and recurrent headaches. It has a benign course with a slow progression, and it is not associated with dissemination. We report a case of a 65-year-old Caucasian woman who presented with symptoms of progressively worsening cognitive dysfunction of six months' duration. From CT of the brain, it was found that she had a right frontal and left parietal hemorrhagic mass with a large amount of vasogenic edema and a midline shift. MRI showed heterogeneously enhancing hemorrhagic mass of 5.2 cm x 2.6 cm x 3.6 cm in size, with a satellite lesion. Initially, this was suspected to be a high-grade glioma vs. metastatic hemorrhagic lesions. She underwent stereotactic biopsy of the mass, and histopathology was consistent with cerebral amyloidoma with marked IgA lambda plasma cell differentiation. She did not have any evidence of systemic amyloidosis, and therefore, she is being clinically observed with a regular follow-up and annual CT surveillance. She has remained stable over the past two years, although she has residual cognitive dysfunction. Cerebral amyloidoma can mimic malignant central nervous system (CNS) neoplasms and should be considered as a differential of any single or multiple mass lesions occurring in the white matter region of the brain with a characteristic appearance of "hyperdense lesions" on CT. It is a benign disease with no metastatic potential that usually resolves entirely after resection.

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Amyloidoma of the CNS.

Cited by 22 publications

References 0 publications

Localized AL amyloidosis: A suicidal neoplasm?

Localized AL amyloidosis: A suicidal neoplasm?

Trigeminal Amyloidoma: Case Report and Review of Literature

A Rare Case of Cerebral Amyloidoma Mimicking a Hemorrhagic Malignant Central Nervous System Neoplasm

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