Pediatric Non-Myofibroblastic Primitive Spindle Cell Tumors with ALK Gene Rearrangements and Response to Crizotinib

Dinesh Rakheja; Jason Y. Park; Neil J. Fernandes; Tanya C. Watt; Theodore W. Laetsch; Rebecca R.J. Collins

doi:10.1177/10668969221080072

Pediatric Non-Myofibroblastic Primitive Spindle Cell Tumors with ALK Gene Rearrangements and Response to Crizotinib

Dinesh Rakheja, Jason Y. Park, Neil J. Fernandes, Tanya C. Watt, Theodore W. Laetsch, Rebecca R.J. Collins

Research output: Contribution to journal › Article › peer-review

5 Scopus citations

Abstract

We describe two poorly differentiated, non-myofibroblastic (SMA−, S100+, CD34±), spindle cell neoplasms with immunohistochemical positivity for ALK and with ALK gene rearrangements leading to PLEKHH2::ALK and CLTC::ALK fusions, respectively. ALK protein overexpression and/or gene fusions should be evaluated in poorly differentiated spindle cell neoplasms, even when there is an absence of a myofibroblastic phenotype. A positive ALK evaluation has therapeutic implications as both tumors responded to single-agent treatment with the tyrosine kinase inhibitor crizotinib.

Original language	English (US)
Pages (from-to)	706-715
Number of pages	10
Journal	International Journal of Surgical Pathology
Volume	30
Issue number	6
DOIs	https://doi.org/10.1177/10668969221080072
State	Published - Sep 2022

Keywords

ALK
CLTC
PLEKHH2
RNA sequencing
crizotinib
gene fusion
pediatric
spindle cell tumor

ASJC Scopus subject areas

Surgery
Anatomy
Pathology and Forensic Medicine

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10.1177/10668969221080072

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title = "Pediatric Non-Myofibroblastic Primitive Spindle Cell Tumors with ALK Gene Rearrangements and Response to Crizotinib",

abstract = "We describe two poorly differentiated, non-myofibroblastic (SMA−, S100+, CD34±), spindle cell neoplasms with immunohistochemical positivity for ALK and with ALK gene rearrangements leading to PLEKHH2::ALK and CLTC::ALK fusions, respectively. ALK protein overexpression and/or gene fusions should be evaluated in poorly differentiated spindle cell neoplasms, even when there is an absence of a myofibroblastic phenotype. A positive ALK evaluation has therapeutic implications as both tumors responded to single-agent treatment with the tyrosine kinase inhibitor crizotinib.",

keywords = "ALK, CLTC, PLEKHH2, RNA sequencing, crizotinib, gene fusion, pediatric, spindle cell tumor",

author = "Dinesh Rakheja and Park, {Jason Y.} and Fernandes, {Neil J.} and Watt, {Tanya C.} and Laetsch, {Theodore W.} and Collins, {Rebecca R.J.}",

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AU - Rakheja, Dinesh

AU - Park, Jason Y.

AU - Fernandes, Neil J.

AU - Watt, Tanya C.

AU - Laetsch, Theodore W.

AU - Collins, Rebecca R.J.

N1 - Publisher Copyright: © The Author(s) 2022.

PY - 2022/9

Y1 - 2022/9

N2 - We describe two poorly differentiated, non-myofibroblastic (SMA−, S100+, CD34±), spindle cell neoplasms with immunohistochemical positivity for ALK and with ALK gene rearrangements leading to PLEKHH2::ALK and CLTC::ALK fusions, respectively. ALK protein overexpression and/or gene fusions should be evaluated in poorly differentiated spindle cell neoplasms, even when there is an absence of a myofibroblastic phenotype. A positive ALK evaluation has therapeutic implications as both tumors responded to single-agent treatment with the tyrosine kinase inhibitor crizotinib.

AB - We describe two poorly differentiated, non-myofibroblastic (SMA−, S100+, CD34±), spindle cell neoplasms with immunohistochemical positivity for ALK and with ALK gene rearrangements leading to PLEKHH2::ALK and CLTC::ALK fusions, respectively. ALK protein overexpression and/or gene fusions should be evaluated in poorly differentiated spindle cell neoplasms, even when there is an absence of a myofibroblastic phenotype. A positive ALK evaluation has therapeutic implications as both tumors responded to single-agent treatment with the tyrosine kinase inhibitor crizotinib.

KW - ALK

KW - CLTC

KW - PLEKHH2

KW - RNA sequencing

KW - crizotinib

KW - gene fusion

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KW - spindle cell tumor

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Pediatric Non-Myofibroblastic Primitive Spindle Cell Tumors with ALK Gene Rearrangements and Response to Crizotinib

Abstract

Keywords

ASJC Scopus subject areas

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