Nasopharyngeal hyalinizing clear cell carcinoma with EWSR1 rearrangements diagnosed by fluorescence in situ hybridization

Nasopharyngeal Hyalinizing Clear Cell Carcinoma: A Case Report and Review of the Literature

“…Salivary gland tumors make up less than 0.5% of all malignancies, and hyalinizing clear cell carcinomas (HCCCs) account for 1% of all salivary gland tumors, making a nasopharyngeal HCCC exceptionally rare. 1–3 HCCCs are low-grade malignant tumors originating from minor salivary glands. 1–7 They most frequently arise from the palate and tongue, but primary tumors have been reported in the nasal cavity, oral mucosa, parotid gland, and rarely, the nasopharynx.…”

“…1–3 HCCCs are low-grade malignant tumors originating from minor salivary glands. 1–7 They most frequently arise from the palate and tongue, but primary tumors have been reported in the nasal cavity, oral mucosa, parotid gland, and rarely, the nasopharynx. 1–3,7,8 A case series by Kauzman et al.…”

“…HCCC is more common in females and typically presents as an indolent small mass. 1–3 Local or distant metastases at presentation are uncommon, with no cases (0 of 10) of nasopharyngeal HCCC presenting with distant metastases. 1–3,5 When tumors arise in the nasopharynx, presenting symptoms are otorrhea, nasal congestion, epistaxis, and tinnitus.…”

“…1–3 Local or distant metastases at presentation are uncommon, with no cases (0 of 10) of nasopharyngeal HCCC presenting with distant metastases. 1–3,5 When tumors arise in the nasopharynx, presenting symptoms are otorrhea, nasal congestion, epistaxis, and tinnitus. 1–11 Treatment typically includes surgical resection and neck dissection if lymphadenopathy is noted on initial evaluation.…”

“…1–3,5 When tumors arise in the nasopharynx, presenting symptoms are otorrhea, nasal congestion, epistaxis, and tinnitus. 1–11 Treatment typically includes surgical resection and neck dissection if lymphadenopathy is noted on initial evaluation. 1,3,4 Overall, the prognosis of HCCC is favorable, especially if negative margins are achieved.…”