Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)

Prestor, Borut

doi:10.1016/j.jocn.2005.07.018

Cited by 13 publications

(10 citation statements)

References 31 publications

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“…MRI scans were obtained in 259 patients (64%). Data on 396 patients from the literature were obtained from 142 reports 1, 5‐145. Patients were followed until death or from 12 months to 408 months (median follow‐up, 36 months) for those who reportedly were alive at the last follow‐up.…”

Section: Methodsmentioning

confidence: 99%

Value of anti–modified citrullinated vimentin and third‐generation anti–cyclic citrullinated peptide compared with second‐generation anti–cyclic citrullinated peptide and rheumatoid factor in predicting disease outcome in undifferentiated arthritis and rheumatoid arthritis

Linden

Woude

Ioan‐Facsinay

et al. 2009

Arthritis & Rheumatism

150

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Objective. Autoantibodies such as rheumatoid factor (RF) and anti-citrullinated protein autoantibodies (ACPAs) determined by testing with secondgeneration anti-cyclic citrullinated peptide (anti-CCP-2) are frequently measured in clinical practice because of their association with disease outcome in undifferentiated arthritis (UA) and rheumatoid arthritis (RA). Recently, 2 new ACPA tests were developed: third-generation anti-CCP (anti-CCP-3) and antimodified citrullinated vimentin (anti-MCV) autoantibody tests. To facilitate the decision on which autoantibody to test in daily practice, this study evaluated the capability of these autoantibodies and combinations of them to predict 3 outcome measures: progression from UA to RA, the rate of joint destruction in RA, and the chance of achieving sustained disease-modifying antirheumatic drug (DMARD)-free remission in RA.Methods. Patients with UA (n ‫؍‬ 625) were studied for whether UA progressed to RA after 1 year. Patients with RA (n ‫؍‬ 687) were studied for whether sustained DMARD-free remission was achieved and for the rate of joint destruction during a median followup of 5 years. Positive predictive values (PPVs) for RA development and for associations with the disease course in RA were compared between single tests (anti-CCP-2, anti-CCP-3, anti-MCV, and RF) and between combinations of these tests.Results. Among the single tests performed in patients with UA, anti-CCP-2 tended to have the highest PPV for RA development (67.1%), but the 95% confidence intervals of the other tests overlapped. Among the single tests in patients with RA, all 4 tests showed comparable associations with the rate of joint destruction and with the achievement of remission. In both ACPA-positive and ACPA-negative RA, the presence of RF was not associated with more joint destruction. For all outcome measures, performing combinations of 2 or 3 autoantibody tests did not increase the predictive accuracy compared with performing a single test.Conclusion. For clinical practice, a single autoantibody test is sufficient for risk estimation in UA and RA.Rheumatoid arthritis (RA) is considered to have an autoimmune origin because of the presence of selfreactive autoantibodies. In addition to rheumatoid facDr. van der Woude

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Section: Methodsmentioning

confidence: 99%

Value of anti–modified citrullinated vimentin and third‐generation anti–cyclic citrullinated peptide compared with second‐generation anti–cyclic citrullinated peptide and rheumatoid factor in predicting disease outcome in undifferentiated arthritis and rheumatoid arthritis

Linden

Woude

Ioan‐Facsinay

et al. 2009

Arthritis & Rheumatism

150

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“…Microscopic pathology of LDD is unique and well described,[ 1 3 13 ] therefore we did not do immunohistochemistry study in our present case. The histopathological findings consist of widening of folia and molecular layer, which is occupied by abnormal ganglion cell, absence of Purkinje cell layer and hypertrophy of granular cell layer.…”

Section: Discussionmentioning

confidence: 95%

“…[ 1 2 3 10 13 ] However, the precise margin of abnormal tissue of this tumor should be assessed under surgical microscope since well border lesion seen on MRI is not always apparent during surgical exploration. [ 13 ] During surgical exploration in some cases of LDD, the lesion is clearly distinguishable from the surrounding normal cerebellar tissue. [ 2 3 ] A well-circumscribed lesion allows neurosurgeons to safely and totally excise this lesion.…”

Section: Discussionmentioning

confidence: 99%

Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature

et al. 2016

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Dysplastic cerebellar gangliocytoma or Lhermitte-Duclos disease (LDD) especially in children are extremely rare. In this report we add one further case to this rare entity. A three year old boy with a history of cerebellar and brain stem compression signs was presented in unconscious condition. Computerized tomography (CT) scan revealed a well defined lesion mixed with area of calcification in the right cerebellum and severe obstructive hydrocephalus. Ventricular shunting was performed followed by gross total tumor removal three days after shunting. In the follow up period, the patient showed almost total resolution of all neurological deficits. MRI has been an imaging tool to preoperatively diagnose this disease; otherwise it would be uncertain preoperative diagnosis. However, in this report we would like to emphasize that not all uncertain diagnosis of LDD leads to palliative treatment. Well demarcated lesion may account for the safe surgical resection of this disease.

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“…Decompressive surgery has been successful both in relieving symptoms and in improving long term survival of patients from 2.5 to 11 years but the absence of tumor limits in the depth of the cerebellar hemisphere constitutes the major technical problem. [10] Intraoperative cytological opinion from the margin of lesion is helpful in such cases as the surgeon can know as to how far he should proceed. The best cytological details were seen with MGG stain and the best nuclear details including nuclear membrane outline and chromatin pattern was seen with PAP stain.…”

Section: Discussionmentioning

confidence: 99%

L Hermitte - Duclos Disease - Intraoperative Cytological Findings of a Rare Entity

Bansal¹,

Kaur²,

Malik³

2015

jemds

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OBJECTIVE: A rare case of LDD confirmed by biopsy is being reported. Review of the published cases shows that the disease usually manifests by the signs of increased intracranial pressure with inconstant cerebellar symptoms. MRI is the imaging modality of choice by now. As such, clinical problems after gross total or complete removal have not been reported so far, but herein we stress the importance of intraopeartive diagnosis of lesions that have no distinct border between the lesion and normal tissue. METHODS: After subtotal resection of lesion, small representative tissue was sent to pathology department intraoperatively in saline for squash smear cytology and postoperatively, whole tissue was sent in formalin for histopathology. The squash smears were stained with MGG, H&E, and PAP stain and report was rendered within 30 minutes. The morphological details as observed in the smears were correlated with Histopathology findings. RESULTS: Squash smears showed a fibrillary background in most of the smears with scattered ganglion cells having vesicular nuclei and prominent nucleoli. Some of the smears showed small dark granular cells representing internal granular cell layer. The corresponding histopathological findings showed widening of the molecular layer with abnormal myelination that is occupied by abnormal ganglion cells, absence of the Purkinje cell layer and hypertrophy of the granular cell layer. CONCLUSION: Squash smear preparation is a fairly accurate, simple and reliable tool for rapid intraoperative diagnosis of CNS lesions. It is helpful in defining the limits of resection in cases like LDD where margins are not well defined.

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Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)

Cited by 13 publications

References 31 publications

Value of anti–modified citrullinated vimentin and third‐generation anti–cyclic citrullinated peptide compared with second‐generation anti–cyclic citrullinated peptide and rheumatoid factor in predicting disease outcome in undifferentiated arthritis and rheumatoid arthritis

Value of anti–modified citrullinated vimentin and third‐generation anti–cyclic citrullinated peptide compared with second‐generation anti–cyclic citrullinated peptide and rheumatoid factor in predicting disease outcome in undifferentiated arthritis and rheumatoid arthritis

Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature

L Hermitte - Duclos Disease - Intraoperative Cytological Findings of a Rare Entity

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