S100 and CD34 Expressing Mesenchymal Neoplasm With Rare PLEKHH2::ALK Fusion and Response to ALK Inhibition.
Am J Surg Pathol
; 46(9): 1309-1313, 2022 09 01.
Article
in En
| MEDLINE
| ID: mdl-35288525
ABSTRACT
The PLEKHH2ALK fusion is a rarely reported gene fusion identified predominantly in lung adenocarcinomas. Tumors with this fusion have been reported to be of durable response to ALK inhibitors. We herein present the case of a 21-year-old woman with a histomorphologically heterogenous mesenchymal neoplasm of the pelvis, expressing both s100 and CD34, with subsequently identified PLEKHH2ALK fusion. To our knowledge, only a single mesenchymal neoplasm with this gene fusion has been previously reported. We propose that this tumor represents one with a novel ALK fusion in the emerging family of s100 and CD34 expressing mesenchymal neoplasms with oncogenic kinase alterations akin to NTRK -rearranged mesenchymal neoplasms, rather than inflammatory myofibroblastic tumor. Importantly, this tumor demonstrated a significant response to the ALK inhibitor brigatinib.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Neoplasms, Connective and Soft Tissue
/
Adenocarcinoma of Lung
/
Lung Neoplasms
Type of study:
Prognostic_studies
Limits:
Adult
/
Female
/
Humans
Language:
En
Journal:
Am J Surg Pathol
Year:
2022
Document type:
Article