SIX1 as a Novel Immunohistochemical Marker in the Differential Diagnosis of Rhabdomyosarcoma
(2023) In Fetal and Pediatric Pathology 42(5). p.723-734- Abstract
Background: Differential diagnosis of rhabdomyosarcoma (RMS) is challenging. Sineoculis homeobox homolog 1 (SIX1) is an oncogene involved in skeletal muscle differentiation. We compared protein expression patterns of SIX1 in RMS and its most common differential diagnoses. Methods: SIX1 immunohistochemistry in 36 RMS and in 33 tumors from seven differential diagnostic subtypes were evaluated. The fraction of SIX1 positive tumor cells was scored by three independent observers. Results: A majority (75%) of the evaluated RMS expressed SIX1 in at least 50% of tumor cells and all except one RMS had more than 25% positive tumor cells. Neuroblastoma had less than 1% SIX1 positive tumor cells. Gonadoblastoma, malignant rhabdoid tumor, and Ewing... (More)
Background: Differential diagnosis of rhabdomyosarcoma (RMS) is challenging. Sineoculis homeobox homolog 1 (SIX1) is an oncogene involved in skeletal muscle differentiation. We compared protein expression patterns of SIX1 in RMS and its most common differential diagnoses. Methods: SIX1 immunohistochemistry in 36 RMS and in 33 tumors from seven differential diagnostic subtypes were evaluated. The fraction of SIX1 positive tumor cells was scored by three independent observers. Results: A majority (75%) of the evaluated RMS expressed SIX1 in at least 50% of tumor cells and all except one RMS had more than 25% positive tumor cells. Neuroblastoma had less than 1% SIX1 positive tumor cells. Gonadoblastoma, malignant rhabdoid tumor, and Ewing sarcoma had 10% or less positive tumor cells. Pleuropulmonary blastoma exhibited 26–50% positive tumor cells and synovial sarcoma >50% positive cells. Conclusion: SIX1 immunohistochemistry is positive in most RMS, and occasionally in some tumors within the differential diagnoses of RMS.
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- author
- Ehinger, Daniel LU ; Frostberg, Hanna LU ; Larsson, Sofia and Gisselsson, David LU
- organization
- publishing date
- 2023
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- A-RMS, E-RMS, Rhabdomyosarcoma, SIX1
- in
- Fetal and Pediatric Pathology
- volume
- 42
- issue
- 5
- pages
- 723 - 734
- publisher
- Taylor & Francis
- external identifiers
-
- pmid:37224459
- scopus:85160254933
- ISSN
- 1551-3815
- DOI
- 10.1080/15513815.2023.2214806
- language
- English
- LU publication?
- yes
- id
- 13b3a1c2-cb89-4413-b2fe-665552413c94
- date added to LUP
- 2023-09-25 10:40:37
- date last changed
- 2024-06-28 08:01:48
@article{13b3a1c2-cb89-4413-b2fe-665552413c94, abstract = {{<p>Background: Differential diagnosis of rhabdomyosarcoma (RMS) is challenging. Sineoculis homeobox homolog 1 (SIX1) is an oncogene involved in skeletal muscle differentiation. We compared protein expression patterns of SIX1 in RMS and its most common differential diagnoses. Methods: SIX1 immunohistochemistry in 36 RMS and in 33 tumors from seven differential diagnostic subtypes were evaluated. The fraction of SIX1 positive tumor cells was scored by three independent observers. Results: A majority (75%) of the evaluated RMS expressed SIX1 in at least 50% of tumor cells and all except one RMS had more than 25% positive tumor cells. Neuroblastoma had less than 1% SIX1 positive tumor cells. Gonadoblastoma, malignant rhabdoid tumor, and Ewing sarcoma had 10% or less positive tumor cells. Pleuropulmonary blastoma exhibited 26–50% positive tumor cells and synovial sarcoma >50% positive cells. Conclusion: SIX1 immunohistochemistry is positive in most RMS, and occasionally in some tumors within the differential diagnoses of RMS.</p>}}, author = {{Ehinger, Daniel and Frostberg, Hanna and Larsson, Sofia and Gisselsson, David}}, issn = {{1551-3815}}, keywords = {{A-RMS; E-RMS; Rhabdomyosarcoma; SIX1}}, language = {{eng}}, number = {{5}}, pages = {{723--734}}, publisher = {{Taylor & Francis}}, series = {{Fetal and Pediatric Pathology}}, title = {{SIX1 as a Novel Immunohistochemical Marker in the Differential Diagnosis of Rhabdomyosarcoma}}, url = {{http://dx.doi.org/10.1080/15513815.2023.2214806}}, doi = {{10.1080/15513815.2023.2214806}}, volume = {{42}}, year = {{2023}}, }