Clonal eosinophilia

FIP1L1-PDGFRA= CHIC2 del= imatinib 100
ETV6-PDGFRB = t(5;12)= imatinib 400
ZMYM2-FGFR1= t(8;13)= TKI+AML/ALL induction+allo
PCM1-JAK2= t(8;9)= ruxolitinib+allo
ETV6-FLT3/ABL1= t(12;13)/t(9;12)= dasatinib+allo

Lymphocytic= T-cell clone makes IL5= mepolizumab

Fluorescence Friday!! Guanylyl cyclase and PDGFRa+ cells surrounding a blood vessel in the esophagus. #fluorescentfriday #esophagus

La routourne a tourné...
Sorry. 🤷‍♂

⚡ Romain Ntamack

#HereToCreate
#PDGFRA

Polycyclic purpuric plaques in a patient with hypereosinophilic syndrome (HES). Biopsy showed necrotizing vasculitis with eosinophils. Look for FIPL1-PDGFRA (present in 10-14% of patients with HES). bit.ly/3oqrAqQ

🚫GIST: centered in muscularis propria, more plump/epithelioid cells, KIT >> PDGFRA mutant
🚫Plexiform fibromyxoma: centered in muscularis propria, plexiform nodules
🚫IMT: centered in mesentery, affects younger patients
✅IFP: centered in submucosa, +CD34 spindle cells 'onion…

🏴󠁧󠁢󠁷󠁬󠁳󠁿🇫🇷 C'était il y a 4 mois ce 22 février à Cardiff et c'est toujours autant un plaisir de revoir ces images 😍 #PDGFRA #NeFaisonsXV #XVdeFrance

GIST- small bowel

📍Small bowel = 2nd most common site of GIST
👴 = 🧓
🔪Well-defined, found in muscle wall, intact serosa and mucosa
🔬💜Mainly spindle, can be epithelioid or mixed
🔬💙DOG1, CD117, CD34 +; S100, Desmin -
🧬KIT, PDGFRA, NF1, BRAF

#GPOW #beardedPA #UofCPath

▪️INFLAMMATORY FIBROID POLYP▪️

• A mesenchymal neoplasm composed of spindle/stellate stromal cells w/ characteristic onion skin arrangement around vessels.

• Inflammatory infiltrate rich in eosinophils.

• CD34 positive.

• Associated w/ mutation in PDGFRA gene.

#GIpath

Is eosinophil dysplasia objective?
Adult male, unexplained hyperesosinophilia; no clonal abnormality (neg. BCR/ABL, JAK2, FGFR1, PDGFRA/B, ...); but these eosinophils are really ugly 😱
Q: Can chronic eosinophilic leukemia be diagnosed only on the basis of cytology / histology?

Le Garrec revient sur sa magnifique chistera lors de la rencontre face au Pays de Galles 🤯

#PDGFRA

This epithelioid gastrointestinal stromal tumor both invades the gastric mucosa and has spread to a lymph node. A succinate dehydrogenase (SDH)-deficient lesion was considered but the SDHB immunostain showed intact expression. The neoplasm was shown to have a PDGFRA mutation.

🏴󠁧󠁢󠁷󠁬󠁳󠁿🇫🇷 Et si vous preniez deux minutes pour revoir la fin de #PDGFRA depuis le banc des Bleus ? 😍 #XVdeFrance #NeFaisonsXV

Inflammatory Fibroid Polyp Essentials:
💛 Centered in submucosa
🍠Onion skinning fusiform cells around vessels with many eosinophils
💚 Can harbour PDGFRA mutations
💙 It's benign and pretty.
🌟Described by Czeck Josef Vanek in 1949, discoverer of pneumocystis pneumonia
#GIPath

Suspected ‘Diverticulum’ -> GIST

Prognostic factors (best for KIT/PDGFRA-mutant GISTs):
↗️Mitotic count 5mm2
➡️Tumor size
↘️Tumor site

Novel PDGFRA mutation in blastic plasmacytoid dendritic cell neoplasm; possible therapeutic implications onlinelibrary.wiley.com/doi/full/10.11… #BPDCN

Diagnostic Algorithm in Wild-type Gastrointestinal Stromal Tumors (wt-GISTs) 🔬

10–12% of all GISTs lack mutations in KIT and PDGFRA and are called wt-GISTs. This group is heterogeneous in their clinical phenotypes and molecular characteristics.

mdpi.com/2075-4418/11/2…

Myxoid Glioneuronal Tumor, PDGFRA p.K385 mutant in the septum pellucidum of a teenage boy. Originally described by David Solomon and colleagues UCSF Pediatric Brain Center (PMID: 31609499). Coming soon to WHO Edition 5! #neuropath #braintumor

こんな感じのexonsとfunctional domainsと腫瘍別の頻発変異を見比べる表が全ての遺伝子において1クリックで見られる場所がほしい。このPDGFRAの見比べ表をまとめるのにデータベースを3つ参照した。サイト数じゃなくて目線を移す回数の方が苦痛だった。

This gastric mass (no gastric tissue seen in images) is an epithelioid PDGFRA-mutated gastrointestinal stromal tumor. It is richly vascular with relatively monotonous cells. #UMiamiPath

🏴󠁧󠁢󠁷󠁬󠁳󠁿⚡️🇫🇷 𝗦𝗘𝗠𝗔𝗜𝗡𝗘 𝗗𝗘 𝗠𝗔𝗧𝗖𝗛 🏆

4ème match de ce Tournoi des #SixNations 2022 ! Direction Cardiff 🛫

📅 Vendredi 11.03 | 21H

#PDGFRA #NeFaisonsXV #MondayMotivation