Primary paranasal sinus hyalinizing clear cell carcinoma: a case report

• 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
• ISSN: 1746-1596; 1746-1596
• DOI: 10.1186/s13000-017-0659-7

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.