Evidence map›Paper›PMID 34540726›Full record

ArticleAutopsy & case reports2021

Erdheim-Chester disease presenting at the central nervous system.

Sydney Fasulo, Mina Fransawy Alkomos, Rovena Pjetergjoka, Erinie M Mekheal, Sharon Awasthi, Sahithi Chittamuri, Vinod Kumar, Mehandar Kumar, Amer Akmal, Michael Maroules

Open access · diamondAbstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Autopsy & case reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
0.7field-weighted citation impact, top 30% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

3 citing papers in PubMed, 7 citations in OpenAlex.

  1. Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-Based Review.AJNR. American journal of neuroradiology · 2025
    Review
  2. Erdheim-Chester Disease.AJNR. American journal of neuroradiology · 2023
    Article
  3. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors at 1 institution in 2 countries.

Sydney FasuloSt. Joseph's University, Medical Center, Hematology and Oncology Department, Paterson, NJ, USA.ORCID 0000-0002-7791-4017
Mina Fransawy AlkomosSt. Joseph's University, Medical Center, Internal Medicine Department, Paterson, NJ, USA.ORCID 0000-0002-7669-1173
Rovena PjetergjokaSt. Joseph's University, Medical Center, Internal Medicine Department, Paterson, NJ, USA.ORCID 0000-0003-2771-9616
Erinie M MekhealSt. Joseph's University, Medical Center, Internal Medicine Department, Paterson, NJ, USA.ORCID 0000-0001-7829-4174
Sharon AwasthiSt. Joseph's University, Medical Center, Internal Medicine Department, Paterson, NJ, USA.
Sahithi ChittamuriSt. Joseph's University, Medical Center, Hematology and Oncology Department, Paterson, NJ, USA.
Vinod KumarSt. Joseph's University, Medical Center, Hematology and Oncology Department, Paterson, NJ, USA.ORCID 0000-0001-5745-1943
Mehandar KumarSt. Joseph's University, Medical Center, Hematology and Oncology Department, Paterson, NJ, USA.
Amer AkmalSt. Joseph's University, Medical Center, Pathology Department, Paterson, NJ, USA.
Michael MaroulesSt. Joseph's University, Medical Center, Hematology and Oncology Department, Paterson, NJ, USA.
St. Joseph’s University Medical Center · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis (LCH) that affects different body systems. It was recently recognized as a neoplastic disorder after identifying an activating mutation of the MAPK pathway. Neurological presentations of ECD are rare. We present a case of a 35-year-old male who presented to the emergency department with neck pain, headache and vomiting for 2 months; MRI showed multiple heterogeneous intracranial masses. Neurosurgery performed a suboccipital craniotomy, partially resected the cerebellar mass, and placed a parietal to frontal shunt catheter. Biopsy results from the cerebellar mass demonstrated cerebellar tissue involved by a diffuse proliferation of foamy histiocytes and spindle cells admixed with prominent lymphoplasmacytic infiltrate and positive for CD68, CD163, Factor XIIIa and Fascin. PET scan showed hypermetabolic uptake within the medullary portions of the diffuse abnormal lesions of the distal femurs, tibias, and fibulas, and cardiac MRI was nonsignificant. The patient was started on vemurafenib and continued to show improvement in a 3-month outpatient follow-up.

Indexed as

and Cerebellar neoplasmsErdheim-Chester Diseasenon-Langerhans cell histiocytosis

Identifiers

PMID34540726
PMCPMC8432387
OpenAlexW3197803502

What OpenQuestion holds

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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.