Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s Palsy
Epithelioid hemangioendothelioma is a rare tumor of endothelial differentiation most commonly arising in soft tissue, liver, and lung, following a variable clinical course. Most cases are characterized by a t(1;3)(p36;q23-25) resulting in WWTR1-CAMTA1 fusion. Only five epithelioid hemangioendothelio...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2022-01-01
|
| Series: | Case Reports in Pathology |
| Online Access: | http://dx.doi.org/10.1155/2022/5687190 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832558321218879488 |
|---|---|
| author | Landon J. Kunzelman Shweta Agarwal Nathan Boyd Cory J. Broehm |
| author_facet | Landon J. Kunzelman Shweta Agarwal Nathan Boyd Cory J. Broehm |
| author_sort | Landon J. Kunzelman |
| collection | DOAJ |
| description | Epithelioid hemangioendothelioma is a rare tumor of endothelial differentiation most commonly arising in soft tissue, liver, and lung, following a variable clinical course. Most cases are characterized by a t(1;3)(p36;q23-25) resulting in WWTR1-CAMTA1 fusion. Only five epithelioid hemangioendothelioma have been previously reported arising in the salivary glands. None have presented as Bell’s palsy. In the current case, a 37-year-old female presented with a longstanding complaint of pain and fullness in the right preauricular region and progressive episodes of Bell’s palsy and facial nerve weakness. Surgical resection showed a tumor comprised of atypical cells with occasional intracytoplasmic vacuoles in a fibromyxoid stroma. Immunohistochemical stains demonstrated the neoplastic cells expressed ERG, CD31, and CD34, confirming vascular differentiation. Fluorescence in situ hybridization revealed a t(1;3)(p36;q25), confirming a diagnosis of epithelioid hemangioendothelioma. At 12-month follow-up, the patient has no evidence of disease. |
| format | Article |
| id | doaj-art-6921d4d3b1c44a89bca643c3c1fa144c |
| institution | Kabale University |
| issn | 2090-679X |
| language | English |
| publishDate | 2022-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pathology |
| spelling | doaj-art-6921d4d3b1c44a89bca643c3c1fa144c2025-02-03T01:32:35ZengWileyCase Reports in Pathology2090-679X2022-01-01202210.1155/2022/5687190Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s PalsyLandon J. Kunzelman0Shweta Agarwal1Nathan Boyd2Cory J. Broehm3University of New MexicoUniversity of New MexicoUniversity of New MexicoUniversity of New MexicoEpithelioid hemangioendothelioma is a rare tumor of endothelial differentiation most commonly arising in soft tissue, liver, and lung, following a variable clinical course. Most cases are characterized by a t(1;3)(p36;q23-25) resulting in WWTR1-CAMTA1 fusion. Only five epithelioid hemangioendothelioma have been previously reported arising in the salivary glands. None have presented as Bell’s palsy. In the current case, a 37-year-old female presented with a longstanding complaint of pain and fullness in the right preauricular region and progressive episodes of Bell’s palsy and facial nerve weakness. Surgical resection showed a tumor comprised of atypical cells with occasional intracytoplasmic vacuoles in a fibromyxoid stroma. Immunohistochemical stains demonstrated the neoplastic cells expressed ERG, CD31, and CD34, confirming vascular differentiation. Fluorescence in situ hybridization revealed a t(1;3)(p36;q25), confirming a diagnosis of epithelioid hemangioendothelioma. At 12-month follow-up, the patient has no evidence of disease.http://dx.doi.org/10.1155/2022/5687190 |
| spellingShingle | Landon J. Kunzelman Shweta Agarwal Nathan Boyd Cory J. Broehm Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s Palsy Case Reports in Pathology |
| title | Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s Palsy |
| title_full | Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s Palsy |
| title_fullStr | Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s Palsy |
| title_full_unstemmed | Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s Palsy |
| title_short | Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s Palsy |
| title_sort | epithelioid hemangioendothelioma with wwtr1 camta1 fusion in the parotid gland presenting as bell s palsy |
| url | http://dx.doi.org/10.1155/2022/5687190 |
| work_keys_str_mv | AT landonjkunzelman epithelioidhemangioendotheliomawithwwtr1camta1fusionintheparotidglandpresentingasbellspalsy AT shwetaagarwal epithelioidhemangioendotheliomawithwwtr1camta1fusionintheparotidglandpresentingasbellspalsy AT nathanboyd epithelioidhemangioendotheliomawithwwtr1camta1fusionintheparotidglandpresentingasbellspalsy AT coryjbroehm epithelioidhemangioendotheliomawithwwtr1camta1fusionintheparotidglandpresentingasbellspalsy |