Primary paranasal sinus hyalinizing clear cell carcinoma: a case report

Hyalinizing clear cell carcinoma (HCCC) is a rare low-grade tumour of salivary glands that was first described as a distinct entity in 1994 by Milchgrub et al. EWSR1-ATF1 fusion was found to be specific for this tumour. The majority of the reported cases of HCCC arise from minor salivary glands with...

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Bibliographic Details
Published in:	Diagnostic pathology Vol. 12; no. 1; p. 70
Main Authors:	AlAli, Batool M, Alyousef, Mohammed J, Kamel, Ahmad Salah, Al Hamad, Mohammad A, Al-Bar, Mohammad H, Algowiez, Roaa M
Format:	Journal Article
Language:	English
Published:	England BioMed Central Ltd 25-09-2017 BioMed Central BMC
Subjects:	Adenocarcinoma Adenocarcinoma, Clear Cell - diagnostic imaging Adenocarcinoma, Clear Cell - genetics Adenocarcinoma, Clear Cell - pathology Cancer Care and treatment Case Report Case reports Case studies CAT scans Clear cell carcinoma Clear cell-type renal cell carcinoma Computed tomography Cytoplasm Diagnosis Diagnosis, Differential Differential diagnosis Epithelial cells EWSR1 gene EWSR1-ATF1 Excision (Surgery) Female FLI-1 protein Fluorescence Fluorescence in situ hybridization Gene Fusion Gene Rearrangement Glands Head and neck Health aspects Histology Histopathology Humans Hyalinizing clear cell carcinoma Hybridization analysis In Situ Hybridization, Fluorescence Kidney cancer Maxilla Maxillary sinus Medical imaging Metastases Metastasis Middle Aged Nose Oral cavity Paranasal sinus Paranasal Sinus Neoplasms - diagnostic imaging Paranasal Sinus Neoplasms - genetics Paranasal Sinus Neoplasms - pathology Paranasal sinuses Paranasal Sinuses - diagnostic imaging Paranasal Sinuses - pathology Quality Radiation Radiation therapy RNA-Binding Protein EWS - genetics S100 protein Salivary gland Salivary glands Septum Sinus Sinuses Surgery Tumors Saudi Arabia Hyalinizing clear cell carcinoma Salivary gland Clear cell carcinoma Paranasal sinuses EWSR1-ATF1
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Summary:	Hyalinizing clear cell carcinoma (HCCC) is a rare low-grade tumour of salivary glands that was first described as a distinct entity in 1994 by Milchgrub et al. EWSR1-ATF1 fusion was found to be specific for this tumour. The majority of the reported cases of HCCC arise from minor salivary glands within the oral cavity. Primary HCCC of the paranasal sinus is extremely uncommon. To our knowledge, only three cases have been reported in the English literature. Herein, we present a case of HCCC of the posterior ethmoid/maxillary sinus. A 63-year-old lady who presented with a long history of epistaxis. CT scan revealed a destructive mass in the left ethmoid/posterior maxillary sinus extending to the nasal cavity. Surgical excision was done and microscopic evaluation showed a tumour composed mainly of nests of clear epithelial cells separated by fibrocellular and hyalinized septa with extensive bone destruction. The tumour cells expressed CK5/6, EMA and p63 immunohistochemically but were negative for S100 protein, PAX-8, RCC and CK7. Sinonasal renal cell-like adenocarcinomas, myoepithelial carcinoma and metastatic renal cell carcinoma were excluded by radiological and immunohistochemical studies. Fluorescence in situ hybridization analysis revealed an EWSR1 gene rearrangement. Postoperative radiation was administrated and the patient did not show recurrence or distant metastasis 4 months after the surgery. Head and neck region have many tumours that demonstrate clear cell changes on histology. Thus, the differential diagnosis for HCCC is wide. Awareness of this rare entity and the possibility of it is arising in unusual location is necessary. EWSR1-AFT1 fusion, a consistent finding in HCCC, can be used to confirm the diagnosis.
Bibliography:	ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3
ISSN:	1746-1596 1746-1596
DOI:	10.1186/s13000-017-0659-7