Abstract
BACKGROUND: Clear-cell carcinoma, a rare malignancy of the minor salivary gland of the soft palate, is diagnostically challenging neoplasm due to its rarity and overlapping features with other neoplasms. We report a case of atypical presentation, diagnostic challenges, and long-term follow-up post-surgical resection of the tumor, which adds valuable insights to literature on this rare malignancy. CASE PRESENTATION: A 34-years-old Pakistani female came to the hospital with a 2 × 2 cm ulcerative lesion of soft palate. The lesion had no active bleeding and any associated discharge. However, during history taking, she reported pain and occasional bleeding from the lesion. DIAGNOSIS AND THERAPEUTIC INTERVENTIONS: Excisional biopsy was performed, and the specimen was sent for histopathological examination and immunohistochemistry, which confirmed the diagnosis of clear-cell carcinoma. Later on, radiological evaluation confirmed the diagnosis of hyalinizing variant of clear-cell carcinoma. Long-term follow-up revealed no recurrence and postoperative complications. CONCLUSION: This case report highlights the importance of thorough diagnostic evaluation and long-term follow-up in management of a rare oral malignancy. Histopathological examination and immunohistochemistry are crucial in differentiating clear-cell carcinoma from other malignancies with overlapping features. Surgical excision remains the primary treatment modality, with a favorable prognosis if diagnosed and managed adequately.