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

Abstract

BackgroundHyalinizing clear cell carcinomas (HCCCs) are rare, low-grade, malignant tumors which most often arise from the minor salivary glands primarily in palate and tongue but can arise in any location with minor salivary glands including the nasopharynx.MethodsA case report of primary nasopharyngeal HCCC is presented. Because of the rarity of this tumor and location, a literature search was conducted to determine the most common presenting symptoms, treatment strategies, and outcomes.ResultsA 48-year-old man underwent biopsy of a 4.5 cm mass of the right nasopharynx with pathology suggesting an intermediate grade mucoepidermoid carcinoma. After discussing management with the patient, an endoscopic resection was performed. Final pathology revealed an HCCC which was confirmed after negative Mastermind-like 2 (MAML2) and positive Ewing sarcoma breakpoint region 1 (ESWR1) gene rearrangements on fluorescence in situ hybridization (FISH) studies. Literature review of other nasopharyngeal HCCC cases shows diverse presentation and overall excellent prognosis through surgical and radiation therapy.ConclusionHCCCs are rare, low-grade malignant tumors of the minor salivary glands and can present as a nasopharyngeal mass. Presenting symptoms are diverse but frequently involve otologic and sinonasal disturbances. HCCC is an indolent tumor with an excellent prognostic outcome when treated appropriately with surgical resection and adjuvant radiotherapy.

Clinical and Histopathologic Analyses of Nasopharyngeal Hyalinizing Clear Cell Carcinoma

“…Only 23 cases have been published in English, and most were single-case reports, 7,8,10,[14][15][16][17][18][19][20][21][22][23][24][25][26][27] with the complete clinical data reported for only 19. The clinical features of the 19 NHCCC cases were analyzed alongside those of the 26 new cases reported herein (N = 45).…”

Clear Cell Carcinoma of Minor Salivary Glands: The Nasopharynx, an Uncommon Site of Origin

“…Clear cell carcinoma, not otherwise specified, of minor salivary glands, also known as hyalinizing clear cell carcinoma, is an unusual primary malignancy of the nasopharynx [ 1 , 3 ]. To the best of our knowledge, there are 13 cases reported in the literature [ 5 ]. Only eight of them have complete clinical data reported (Table 1 ) [ 3 , 5 - 11 ].…”

“…To the best of our knowledge, there are 13 cases reported in the literature [ 5 ]. Only eight of them have complete clinical data reported (Table 1 ) [ 3 , 5 - 11 ]. These tumors are microscopically characterized by a monomorphous population of cells with optically clear cytoplasm with standard hematoxylin and eosin stains [ 2 ].…”

Case report: Successful management of primary hyalinizing clear cell carcinoma in nasopharynx: a report of 2 cases and system analysis

“…In a case series conducted by Kauzman et al, nasopharyngeal HCCC exhibited a 1% prevalence among 98 reported cases of HCCC in English literature (6). HCCC displays a higher incidence in females and typically manifests as a small, indolent mass (7). The occurrence of local or distant metastases at the time of presentation is infrequent, with no instances (0 out of 10) of nasopharyngeal HCCC presenting with distant metastases reported (6,8).…”

“…The occurrence of local or distant metastases at the time of presentation is infrequent, with no instances (0 out of 10) of nasopharyngeal HCCC presenting with distant metastases reported (6,8). Symptoms associated with nasopharyngeal HCCC include otorrhea, nasal congestion, epistaxis, and tinnitus (4,7). Standard treatment approaches involve surgical resection and neck dissection in the presence of lymphadenopathy during the initial evaluation.…”