Interesting association of neurofibroma with diffuse cystic lung disease (NF-DLD)

An unusual type of neurofibroma predominantly seen in children and young adults is diffuse neurofibroma. We present a 25-year-old female with recurring soft tissue masses in her right lower limb. MRI showed areas of T1 iso-intensity and T2 hyperintensity relative to skeletal muscle within the subcutaneous fat. These masses show marked enhancement post gadolinium administration. Histological examination of the excised mass showed diffuse neurofibroma. The rare nature of this tumour and the limited literature describing the imaging features make the diagnosis relatively difficult for a radiologist.

show abstract

“… 3 , 12 There have been reported retroperitoneal location and associations with diffuse cystic lung disease. 13 …”

Section: Outcome/follow-up/discussionmentioning

confidence: 99%

MRI evaluation of diffuse subcutaneous neurofibroma of the lower limb in a low resource setting

2018

View full text Add to dashboard Cite

show abstract

A Validated Model for the Imaging Diagnosis of Cystic Lung Disease

Miller¹,

Patterson²,

Sood³

et al. 2023

OJRad

View full text Add to dashboard Cite

Rationale and Objectives: Cystic lung disease may be accurately diagnosed by imaging interpretation of specialist radiologists, without other information. We hypothesized that with minimal training non-specialists could perform similarly to specialist physicians in the diagnosis of cystic lung disease. Methods: 72 cystic lung disease cases and 25 cystic lung disease mimics were obtained from three sources: 1) a prospective acquired diffuse lung disease registry, 2) a retrospective search of medical records and 3) teaching files. Cases were anonymized, randomized and interpreted by 7 diffuse lung disease specialists and 15 non-specialist radiologists and pulmonologists. Clinical information other than age and sex was not provided. Prior to interpretation, non-specialists viewed a short PDF training document explaining cystic lung disease interpretation. Results: Correct first choice diagnosis of 85% -88% may be achieved by high-performing specialist readers and 71% -80% by non-specialists and lower-performing specialists, with mean accuracies in the diagnosis of LAM (91%, p < 0.0001), BHD (93%, p < 0.0001), PLCH (89%, p < 0.0001) and LIP (92%, p < 0.0001). A strategy based on cyst appearance: simple cysts (LAM), peri-septal cysts (BHD), bizarre-shaped cysts (PLCH) and vascular indented cysts (LIP) gave non-specialists accuracies of 90% (p < 0.0001), 94% (p < 0.0001

show abstract

Interesting association of neurofibroma with diffuse cystic lung disease (NF-DLD)

Cited by 2 publications

References 1 publication

MRI evaluation of diffuse subcutaneous neurofibroma of the lower limb in a low resource setting

MRI evaluation of diffuse subcutaneous neurofibroma of the lower limb in a low resource setting

A Validated Model for the Imaging Diagnosis of Cystic Lung Disease

Contact Info

Product

Resources

About