Sclerosing odontogenic carcinoma is a rare and locally invasive malignant tumor of odontogenic origin. Due to its low incidence, the literature is limited to isolated case reports, making comprehensive understanding of its clinicopathologic features and management strategies challenging.

This systematic review aims to synthesize the existing case reports on SOC to elucidate its clinical, radiographic, histopathological, and immunohistochemical characteristics, and to provide insights into current diagnostic and therapeutic approaches.

A comprehensive literature search was conducted across three major databases: PubMed, Scopus, and Embase. A total of 95 records were initially identified (PubMed: n = 36; Scopus: n = 30; Embase: n = 29). After screening and eligibility assessment, 16 studies meeting inclusion criteria were selected for final analysis.

SOC predominantly involved the mandible. The most commonly reported symptom was swelling, either painless or painful. Radiographically, lesions often appeared as ill-defined or poorly marginated radiolucencies, reflecting their infiltrative nature. Frequent cortical bone involvement included thinning, resorption, and perforation. Histopathologically, SOC was characterized by cords, nests, and strands of bland epithelial cells in a densely sclerotic stroma. Immunohistochemically, tumors showed strong expression of high molecular weight cytokeratins (CK5/6, CK19, p63, AE1/AE3). The primary treatment modality was wide local excision with clear margins, commonly involving segmental mandibulectomy or maxillectomy based on tumor extent.

SOC is an exceptionally rare odontogenic malignancy with consistent clinical and pathological features across reported cases. Its locally aggressive nature underscores the importance of early diagnosis and wide surgical excision. This review consolidates existing case data to enhance understanding and aid clinicians in recognizing and managing this challenging neoplasm.