Hyalinizing clear cell carcinoma of the soft palate. Case report

OBJECTIVES: Il Hyalinizing Clear Cell Carcinoma (HCCC) is a rare malignancy primarily affecting minor salivary glands, presenting diagnostic challenges due to its diverse histological features and potential overlaps with other tumor types. This report describes a case of HCCC in a young patient and discusses diagnostic and treatment approaches.

MATERIALS AND METHODS: A 25-year-old male presented with a lesion located on the soft palate. Diagnostic imaging included MRI and CT scans, which suggested a pleomorphic adenoma. A fine needle aspiration biopsy further supported this initial diagnosis. Wide local excision of the lesion was performed with intraoral access, and reconstruction used a dermal regeneration template. Postoperative histopathological examination confirmed HCCC, and immunohistochemical analysis was positive for CK7, p63, and p40, with an EWSR1 translocation identified in 97% of cells.

RESULTS: HCCC, despite being low-grade, poses diagnostic challenges due to its histological diversity. Positive immunohistochemical staining for markers such as PAS, CK, EMA, CAM 5.2, and p63, along with EWSR1-ATF1 gene rearrangement, are key in distinguishing HCCC from similar tumors. Surgical resection with clear margins remains the primary treatment, with radiotherapy reserved for cases with aggressive features. Given its 25% rate of cervical metastasis, neck dissection might be considered. This case had clear margins, so no adjuvant therapy was necessary. Follow-up is essential due to risks of recurrence or metastasis. Advanced diagnostic methods, including molecular analyses, are crucial for precise diagnosis and treatment guidance.

CONCLUSIONS: Management of HCCC requires a multidisciplinary approach, with surgical resection and immunohistochemical and molecular analyses ensuring optimal outcomes. This case highlights the importance of timely diagnosis and tailored interventions. Ongoing research on HCCC’s molecular mechanisms could improve diagnostic criteria and therapy options, enhancing prognosis for affected patients.

CLINICAL SIGNIFICANCE: HCCC’s low prevalence and diagnostic challenges underline the need for early diagnosis and comprehensive treatment. Detailed histopathological and immunohistochemical analyses, including EWSR1 translocation detection, are essential for distinguishing HCCC from other clear cell tumors, guiding effective treatment and reducing recurrence risk, thus improving long-term prognosis.

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