Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma?
(1) Background: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade neoplasm of the sinonasal tract. It is characterized by specific <i>PAX3</i> gene rearrangements and both myogenic and neural differentiation. The purpose of the study was to describe the histologic, immunohistoche...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2022-10-01
|
| Series: | Diagnostics |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-4418/12/10/2528 |
| _version_ | 1797473798615728128 |
|---|---|
| author | Olga Kuczkiewicz-Siemion Monika Prochorec-Sobieszek Maciej Rysz Aneta Wojnowska Monika Durzyńska |
| author_facet | Olga Kuczkiewicz-Siemion Monika Prochorec-Sobieszek Maciej Rysz Aneta Wojnowska Monika Durzyńska |
| author_sort | Olga Kuczkiewicz-Siemion |
| collection | DOAJ |
| description | (1) Background: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade neoplasm of the sinonasal tract. It is characterized by specific <i>PAX3</i> gene rearrangements and both myogenic and neural differentiation. The purpose of the study was to describe the histologic, immunohistochemical and molecular features of BSNS and indicate important clues for small incisional biopsy diagnostics. (2) Methods: Archival samples from patients with nasal cavities or ethmoid sinuses tumors were searched for BSNS cases. Inclusion criteria were the presence of spindle cell morphology and low-grade appearance. Both biopsy and resection specimens were stained for identical IHC panels including, i.a., S100, SMA, SOX10 and PAX3. FISH for <i>PAX3</i> and <i>SS18</i> was performed on biopsy specimens. (3) Results: BSNS diagnosis was made in 6 cases included in the study and confirmed by <i>PAX3</i> rearrangement by FISH in 5 specimens. The pattern of IHC expression was identical for paired biopsy and resection samples apart from one BSNS case. (4) Conclusions: Incisional biopsy seems to be a sufficient method to establish BSNS diagnosis in most cases. Characteristic morphological features together with S100, SOX10 and SMA as the screening markers are useful for confirming the diagnosis. In cases of divergent morphology and immunoprofile evaluation of <i>PAX3</i> rearrangement is vital. |
| first_indexed | 2024-03-09T20:21:34Z |
| format | Article |
| id | doaj.art-a14f519b66a9490485318e82fff4d008 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-09T20:21:34Z |
| publishDate | 2022-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-a14f519b66a9490485318e82fff4d0082023-11-23T23:46:50ZengMDPI AGDiagnostics2075-44182022-10-011210252810.3390/diagnostics12102528Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma?Olga Kuczkiewicz-Siemion0Monika Prochorec-Sobieszek1Maciej Rysz2Aneta Wojnowska3Monika Durzyńska4Department of Pathology, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, PolandDepartment of Pathology, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, PolandReconstructive Team, Department of Breast Cancer and Reconstructive Surgery, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, PolandDepartment of Pathology, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, PolandDepartment of Pathology, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland(1) Background: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade neoplasm of the sinonasal tract. It is characterized by specific <i>PAX3</i> gene rearrangements and both myogenic and neural differentiation. The purpose of the study was to describe the histologic, immunohistochemical and molecular features of BSNS and indicate important clues for small incisional biopsy diagnostics. (2) Methods: Archival samples from patients with nasal cavities or ethmoid sinuses tumors were searched for BSNS cases. Inclusion criteria were the presence of spindle cell morphology and low-grade appearance. Both biopsy and resection specimens were stained for identical IHC panels including, i.a., S100, SMA, SOX10 and PAX3. FISH for <i>PAX3</i> and <i>SS18</i> was performed on biopsy specimens. (3) Results: BSNS diagnosis was made in 6 cases included in the study and confirmed by <i>PAX3</i> rearrangement by FISH in 5 specimens. The pattern of IHC expression was identical for paired biopsy and resection samples apart from one BSNS case. (4) Conclusions: Incisional biopsy seems to be a sufficient method to establish BSNS diagnosis in most cases. Characteristic morphological features together with S100, SOX10 and SMA as the screening markers are useful for confirming the diagnosis. In cases of divergent morphology and immunoprofile evaluation of <i>PAX3</i> rearrangement is vital.https://www.mdpi.com/2075-4418/12/10/2528biphenotypic sinonasal sarcomaPAX3BSNSdifferential diagnosisimmunohistochemistrysinonasal tract |
| spellingShingle | Olga Kuczkiewicz-Siemion Monika Prochorec-Sobieszek Maciej Rysz Aneta Wojnowska Monika Durzyńska Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma? Diagnostics biphenotypic sinonasal sarcoma PAX3 BSNS differential diagnosis immunohistochemistry sinonasal tract |
| title | Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma? |
| title_full | Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma? |
| title_fullStr | Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma? |
| title_full_unstemmed | Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma? |
| title_short | Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma? |
| title_sort | small biopsy samples are they representative for biphenotypic sinonasal sarcoma |
| topic | biphenotypic sinonasal sarcoma PAX3 BSNS differential diagnosis immunohistochemistry sinonasal tract |
| url | https://www.mdpi.com/2075-4418/12/10/2528 |
| work_keys_str_mv | AT olgakuczkiewiczsiemion smallbiopsysamplesaretheyrepresentativeforbiphenotypicsinonasalsarcoma AT monikaprochorecsobieszek smallbiopsysamplesaretheyrepresentativeforbiphenotypicsinonasalsarcoma AT maciejrysz smallbiopsysamplesaretheyrepresentativeforbiphenotypicsinonasalsarcoma AT anetawojnowska smallbiopsysamplesaretheyrepresentativeforbiphenotypicsinonasalsarcoma AT monikadurzynska smallbiopsysamplesaretheyrepresentativeforbiphenotypicsinonasalsarcoma |