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Case Reports
. 2021 Jun;15(2):649-656.
doi: 10.1007/s12105-020-01204-5. Epub 2020 Jul 27.

Does Nodal Metastasis and Perineural Invasion Affect Local Control in Hyalinizing Clear Cell Carcinoma of the Oral Cavity? A Case Report with Long Term Follow-Up

John M Le  1 , Darya Gubarev  2 , Yedeh Ying  3 , Anthony Morlandt  3
Affiliations
Case Reports

Does Nodal Metastasis and Perineural Invasion Affect Local Control in Hyalinizing Clear Cell Carcinoma of the Oral Cavity? A Case Report with Long Term Follow-Up

John M Le et al. Head Neck Pathol. 2021 Jun.

Abstract

Hyalinizing clear cell carcinoma (HCCC) is a rare, low-grade neoplasm accounting for approximately 1% of salivary gland neoplasms. Histologically, it is characterized by a monomorphous population of clear cells arranged in sheets, nests, or cords, lacking ductal structures. Until recently, clear cell carcinoma of the oral cavity (OC) represented a diagnosis of exclusion when other head and neck pathologic entities such as epithelial-myoepithelial carcinoma or mucoepidermoid carcinoma could be ruled out, making definitive diagnosis by light microscopy and immunoprofiling a challenge. As a result, initial biopsies are often misclassified, and could result in under- or overtreatment. More recently, the presence of the EWSR1-ATF1 gene fusion has been adopted to definitively diagnose HCCC. Typically, HCCC demonstrates clinical indolence and responds well to curative surgical excision alone for localized disease, with adjuvant radiotherapy (RT) reserved for high risk features including perineural invasion, lymphovascular invasion, and regional cervical metastasis. The literature, however, lacks consensus regarding the role of adjuvant radiotherapy. In this article, we report a case of HCCC in a rare site involving the ventral tongue, with high risk features of perineural invasion and cervical nodal metastasis. The patient underwent surgical excision alone; declining adjuvant radiotherapy despite the high risk features, and was alive with no evidence of disease at the 42-month mark. Furthermore, we provide an update on the current prognostic indicators for HCCC, and emphasize the need for chromosomal analysis to achieve a definitive diagnosis.

Keywords: Clear cell carcinoma; EWSR1 fusion; Hyalinizing clear cell carcinoma; Nodal metastasis; Salivary gland tumor.

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Conflict of interest statement

The authors declare that they have no conflicts of interest.

Figures

Fig. 1
Fig. 1
Tumor on ventral surface of the gone (a) treated with wide local excision and primary closure (b). Patient regained excellent speech and swallowing function at 2-months postoperatively (c)
Fig. 2
Fig. 2
a The tumor is present in cords and nests of epithelial and clear cells with interspersed hyalinized stroma. Focal invasion of the overlying epithelium is seen (yellow arrow). b Perineural invasion of a small nerve (yellow arrow)
Fig. 3
Fig. 3
This image is of the EWSR1 break apart probe showing positive gene rearrangement in the tumors cells using FISH
See this image and copyright information in PMC

References

    1. Albergotti WG, et al. Hyalinizing clear cell carcinoma of the head and neck: case series and update. Head Neck. 2016;38(3):426–433. - PubMed
    1. Seethala RR, Stenman G. Update from the 4th edition of the World Health Organization classification of head and neck tumours: tumors of the salivary gland. Head Neck Pathol. 2017;11(1):55–67. - PMC - PubMed
    1. Hernandez-Prera JC, Kwan R, Tripodi J, et al. Reappraising hyalinizing clear cell carcinoma: a population-based study with molecular confirmation. Head Neck. 2017;39(3):503–511. - PubMed
    1. Antonescu CR, Katabi N, Zhang L, Sung YS, Seethala RR, Jordan RC, Perez-Ordoñez B, Have C, Asa SL, Leong IT, Bradley G. EWSR1-ATF1 fusion is a novel and consistent finding in hyalinizing clear-cell carcinoma of salivary gland. Genes Chromosom Cancer. 2011;50(7):559–570. - PubMed
    1. Zhao YN, Wang X, Liang FH, Zhang WJ, Song XT. Hyalinizing clear cell carcinoma of salivary glands: a retrospective study focused on uncommon morphology, immunohistochemistry, and detection of gene fusion using fluorescence in situ hybridization. Pathology. 2018;214(3):380–384. - PubMed

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