Extraosseus Plasmacytoma of the Pharynx with Localized Light Chain Deposition. Case Report

Zidar, Nina; Zver, Samo; Jurčić, Vesna

doi:10.1007/s12253-009-9218-1

Cited by 3 publications

(3 citation statements)

References 17 publications

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“…The clinical manifestations of aggregomas are related to the location of deposits. Rare cases of isolated aggregomas have also been reported in the brain, heart, pharynx and duodenum [1,8,9]. In 80 % of patients, the deposits are derived from kappa immunoglobulin light chain, as was the case with our patient.…”

Section: Discussionsupporting

confidence: 75%

Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region

et al. 2015

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Aggregomas are rare localized masses of monoclonal nonamyloid immunoglobulin light-chain deposits. To date, there have been only a few reports of isolated aggregomas, with the majority detailing renal, lymph node and brain deposition. We present a rare case of paraspinal aggregoma in a 67-year-old female who presented with a complaint of cough and chest pain. Imaging demonstrated a left-sided paravertebral mass extending from T7-T10. Pathological analysis showed lamellar deposition of extracellular eosinophilic material with an associated lymphoplasmacytic nonamyloid infiltrate. To our knowledge, this is the first report of a paraspinal aggregoma. While exceedingly rare, this tumor can be included in the radiologic differential diagnosis of paravertebral soft tissue tumors in adults. The observation of our case adds to the limited understanding of the etiology, pathogenesis, natural history, and treatment of nonamyloid light-chain depositions.

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Section: Discussionsupporting

confidence: 75%

Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region

et al. 2015

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“…This disorder was originally described by Randall et al (5) in 1976 in two patients with end-stage renal disease (ESRD) with granular deposition of free light chains that did not stain with Congo red on kidney pathologic evaluation. There have only been a few reports of isolated LCDD involving the brain (3), lungs (4), cervical lymph nodes (5), and pharynx (6). Here, we report one case of LCDD limited to the duodenal mucosa.…”

Section: Introductionmentioning

confidence: 99%

A Case of Isolated Light Chain Deposition Disease in the Duodenum

et al. 2012

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Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. LCDD is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have been few reports of isolated LCDD. We report a rare case of LCDD limited to a duodenal polyp. A 63-yr-old man visited our hospital for health screening without symptoms in 2009. On gastrofiberscopy, a duodenal polyp was observed. The biopsy showed diffuse infiltration by atypical plasma cells, which were positive for kappa-type light chains by immunohistochemistry. While the patient refused further management, we could find no evidence of recurrence until 2 yr after the initial diagnosis. It has been reported that isolated LCDD has relatively good prognosis compared to systemic LCDD. However, treatment for this disease has not been established yet.

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“…Localized MIDDs have been described in respiratory and gastrointestinal tracts, lymph nodes, bones, and brain, usually as tumour-like amyloid deposits designated amyloidomas or, much more rarely, as tumour-like non-amyloid deposits called aggregomas [19]. Diffuse LCDD restricted to a single organ has also been described [16].…”

Section: Introductionmentioning

confidence: 99%

Brain aggregoma with clonal B-cell perivascular proliferation detected by next-generation sequencing. A case report and review of the literature

Matjašič

Kavalar

Voršič

et al. 2021

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Light-chain deposition disease (LCDD), a rare type of monoclonal immunoglobulin deposition disease, can be presented as systemic or localized, very rarely affecting central nervous system (CNS). Only 10 cases of CNS-LCDD have been described so far. We present an eleventh case of cerebral tumour-like LCDD, called aggregoma, and compare it with previously reported cases. A 49-year-old patient was admitted to the hospital due to a first generalized epileptic seizure. Magnetic resonance imaging (MRI) showed focal lesion in the right occipital lobe. Abundant parenchymal aggregates of pale eosinophilic material were observed, Congo red negative, Thioflavin T moderately positive, and λ-light chain positive, but κ negative in immunofluorescence with mild perivascular lymphoplasmacytic infiltrates in the intervening brain tissue. Clonality testing by next-generation sequencing showed the monoclonal nature of B-lymphocytes. Electron microscopy showed a finely granular ultrastructure of the aggregates without deposition in the vessel walls. A whole-body workup did not show any extra-cerebral immune dyscrasias.

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Extraosseus Plasmacytoma of the Pharynx with Localized Light Chain Deposition. Case Report

Cited by 3 publications

References 17 publications

Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region

Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region

A Case of Isolated Light Chain Deposition Disease in the Duodenum

Brain aggregoma with clonal B-cell perivascular proliferation detected by next-generation sequencing. A case report and review of the literature

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