American Association of Neuropathologists annual meeting gets underway

I arrived in Atlanta this afternoon in anticipation of tomorrow's kick-off of the American Association of Neuropathologists annual meeting. I've already run into a number of neuropathologists from around the country, including: Howard Chang, William Taylor, Eddie Lee, Beatriz Lopes, Roberta Seidman, Doug Miller, Mary Fowkes, Clare Bryce, Ray Sobel, Tessa Hedley-White, and Leroy Sharer. Looking forward to a great meeting!

I caught up with the inimitable Dr. Leroy Sharer (right) while checking in for the meeting

Abstracts for the AANP annual meeting are now available online

The 95^th Annual Meeting abstracts are now available online here. They can also be found in the current June JNEN issue. 

Best Post of January 2019: Radiologically suspected meningioma turns out to be WHO grade II Pleomorphic Xanthoastrocytoma

The next in our "Best of the Month" series comes from January 18, 2019:

Rosethal fiber in oval. Eosinophilic granular body in rectangle

Rosenthal fibers can be seen in non-neoplastic "compressed" brain tissue. For example, I once saw Rosenthal fibers in the spinal cord adjacent to an epidural abscess.

Diagnostic Slide Session cases have been released

The American Association of Neuropathologists has released the cases for the 2019 Diagnostic Slide Session (DSS) cases, which will be held at the association's annual meeting on Saturday, June 8 from 8 to 11 pm. The session, which will be moderated by Drs. Caterina Giannini and Rebecca D. Folkerth, focuses on a discussion of 10 cases submitted by members from far and wide.

Helpful glossary of pathology terms

Mark Cohen's hidden genius as a classical guitarist

The neuropathology community may be aware of the extraordinary musical talents of Dr. Arie Perry, but there is another among us who has hidden genius as a classical guitarist. I present to you the inimitable Dr. Mark Cohen:

https://www.youtube.com/watch?v=VHfBmXbnKGA&feature=youtu.be

Case Western's Mark Cohen (right) with accompianist

Nota Bene: Since my original publication of this post, the inimitable Dr. Cohen emailed me the following: 

"Please credit my partner in crime (actually the brains behind the operation): Narayan Kumar (from Mumbai). We're both learning guitar online at Classical Guitar Corner Academy. Here's the backstory:

Introducing DuJu. DuJu in Mandarin has a few meanings, not all salutary. But it also means a special talent or insight, which we arrogated to ourselves, whether deserved or not -- although neither of us is Chinese. The preferred pronunciation is “Du-zhou”. Sounds and feels French. Which is always a good thing -- although neither of us is French. In a word, DuJu is American, French, Chinese and Indian. Hey, we are an international community here, right? We are probably pushing it when we suggest the name is also a combination of our origins – one born a HinDU and the other a JU, er, Jew. It signifies a welcome coming together of faiths in our troubled world through music -- although neither of us is religious. Whatever. DuJu it is. It’s been fun to do this and we have some bits of music under discussion for sharing with all of us. Barring soul-crushing, deafening silence from our illustrious colleagues here, DuJu hope to being continually fresh and entertaining. Performing around the world cannot be ruled out ;)"

A Jungian Approach to Individual Neuropathological Specimens

I was reading The Undiscovered Self (1958) by Carl Jung today, and noted that Jung's description of individual patients could also be applied to individual tumors:

"The statistical method shows the facts in the light of the ideal average but does not give us a picture of their empirical reality.... The distinctive thing about real facts, however, is their individuality.... There is and can be no self-knowledge based on theoretical assumptions, for the object of this knowledge is an individual - a relative exception and an irregular phenomenon. Hence, it is not the universal and the regular that characterize the individual, but rather the unique. He is not to be understood as a recurrent unit but as something unique and singular which in the last analysis can be neither known nor compared with anything else."

Consider this the next time you sign out a "bread and butter" glioblastoma!

Live PathCast Tomorrow: Rodriguez on Meningomas

Tuesday, April 30th - 8 AM EST Topic: Meningiomas Presented by Fausto Rodriguez, M.D., Associate Professor of Pathology, JHUSOM

This seminar can be accessed live through the following links at 8 AM on Tuesdsay: Facebook: https://www.facebook.com/events/355454475302665/ YouTube: https://www.youtube.com/channel/UCVxosS9hPP3ikMQXAE9Pamw/videos Website: http://pathologycast.com/

Sclerosing orbital lesions: broadening the differential diagnosis of "idiopathic sclerosing pseudotumor"

Sclerosing orbital lesion
(the biopsy revealed it to be Erdheim-Chester disease)

Sclerosing lesions of the orbit can present a diagnostic conundrum. Often submitted with a differential diagnosis that includes idiopathic sclerosing pseudotumor, it is incumbent upon the pathologist to rule out other diagnostic possibilities before designating the lesion as idiopathic. In order to survey the range of diagnoses for such specimens, we searched over a 10-year period for specimens submitted to the University of Colorado pathology department which either clinically or pathologically raised the possibility of idiopathic sclerosing pseudotumor. We found 31 specimens (from 27 patients) that met criteria for review. The diagnosis for each of the 27 patients was confirmed after review of medical records. For 11 patients, a final clinicopathologic diagnosis of idiopathic sclerosing pseudotumor was rendered. The remaining diagnoses included: Wegener's granulomatosis (3), IgG4-related disease (2), reactive change to a prior procedure (2), schwannoma (2), sclerosed vascular malformation (2), Erdheim-Chester disease (1), non-Langerhans cell histiocytosis NOS (1), sarcoidosis (1), fibromyxoma (1), and thyroid eye disease (1).  Based on this institutional review, the differential diagnosis of fibrosing lesions of the orbit is broad, encompassing autoimmune, reactive, neoplastic, malformative, and endocrine disease. However, the most common final diagnosis remains idiopathic sclerosing pseudotumor.

cIMPACT-NOW Update 4: diffuse gliomas characterized by MYB, MYBL1, or FGFR1 alterations or BRAFV600E mutation

This cIMPACT-NOW update was published this month:

Summary:
cIMPACT (Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy) has reviewed the status of WHO grade II IDH-wt/H3-wt diffuse gliomas, focusing on those with a BRAFV600E mutation, FGFR1 alteration, or a MYB or MYBL1 rearrangement, and recommends the use of an integrated diagnosis to combine their histologic and genetic features. 

The consortium recommends the use of an integrated diagnosis to combine their histologic and genetic features, as suggested in the following:

• Diffuse glioma, MYB-altered
• Diffuse glioma, MYBL1-altered
• Diffuse glioma, FGFR1 TKD-duplicated
• Diffuse glioma, FGFR1-mutant
• Diffuse glioma, BRAFV600E-mutant (but without CDKN2A/B deletion)
• Diffuse glioma, other MAPK pathway alteration

The full for cIMPACT Update 4 can be found at:

Ellison DW, Hawkins C, Jones DTW, Onar-Thomas A, Pfister SM, Reifenberger G, Louis DN.  cIMPACT-NOW update 4: diffuse gliomas characterized byMYB, MYBL1, or FGFR1 alterations or BRAF^V600E mutation. Acta Neuropathol. https://doi.org/10.1007/s00401-019-01987-0.  PMID: 30848347

"Although our cIMPACT committee sees the utility of distinguishing these diffuse gliomas in diagnostic practice, it also acknowledges that the overlap between their morphologic and genetic features and those of other neuroepithelial tumors could occasionally compromise an accurate diagnosis. These other tumors, including pilocytic astrocytoma, PXA, and DNT, could themselves benefit from a classification based upon their combined histologic and genetic features; indeed, it seems likely that such tumors will be the subject of future cIMPACT recommendations on their classification."

The full cIMPACT Steering Committee comprises Drs. Ken Aldape, Dan Brat, David Capper, David W. Ellison, Dominique Figarella-Branger, Cynthia Hawkins, Takashi Komori, David N. Louis, Catriona McLean, Werner Paulus, Arie Perry, Guido Reifenberger, Andreas von Deimling, and Pieter Wesseling.