Primary paranasal sinus hyalinizing clear cell carcinoma: a case report

Abstract

Background: 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.

Case presentation: 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.

Conclusion: 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.

Keywords: Clear cell carcinoma; EWSR1-ATF1; Hyalinizing clear cell carcinoma; Paranasal sinuses; Salivary gland.

Fig. 1

Head CT scan. Coronal head CT scan showing a destructive lesion within the paranasal sinuses extending to the nasal cavity. The epicenter of the mass is seen within the left ethmoid/posterior maxillary and sphenoid sinus (a) with extension posteriorly to involve the anterior superior border of the clivus (b)

Fig. 2

Histological features of HCCC and FISH analysis. a - c, scanning magnification showing an infiltrative clear cell tumour associated with bone destruction (a, H&E, original magnification 40×), these polygonal to round clear cells are arranged in nests separated by hyalinized fibrocellular septa. Other smaller cells with eosinophilic cytoplasm are seen mostly at the periphery of the tumour (b, hematoxylin and eosin, original magnification 200×). Nuclei are round and central (c, hematoxylin and eosin, original magnification 200×). Diffuse cytoplasmic and membranous staining for CK5/6 (d, immunohistochemistry, original magnification 200×). FISH analysis using dual-color, EWSR1 break apart probe. The tumour cells show one green and one red signal, indicative of a rearrangement of EWSR1 region (e)