Malignant chondroid syringoma: A systematic review

Area of Science:

• Dermatology
• Surgical Oncology
• Pathology

Background:

• Malignant Chondroid Syringomas (MCS) are exceedingly rare malignant tumors originating from cutaneous sweat glands.
• Only 51 cases have been documented, highlighting their rarity and the need for better understanding of prognostic factors.
• Current diagnostic criteria lack established features to predict metastasis or mortality risk.

Purpose of the Study:

• To systematically review the literature to identify features of primary MCS tumors associated with metastasis or patient mortality.
• To evaluate the efficacy of common treatment modalities for MCS.
• To establish prognostic indicators for improved patient management.

Main Methods:

• A systematic literature review was conducted using Ovid Medline and Web of Science databases.
• Data from 47 case reports, comprising 51 unique patients, were collected and analyzed.
• Statistical analysis focused on correlating histopathologic and gross tumor characteristics with metastatic risk and mortality.

Main Results:

• No significant association was found between common malignant histopathologic findings (e.g., atypia, mitotic figures, necrosis) and metastatic risk or death.
• Gross tumor characteristics, specifically size greater than 5 cm and location on the trunk, were significantly associated with an increased risk of metastasis.
• Wide local excision was identified as the most effective treatment modality.

Conclusions:

• Histopathologic features alone are insufficient to predict metastasis or mortality in MCS.
• Tumor size (>5 cm) and truncal location are critical indicators of higher metastatic potential.
• Prompt wide local excision followed by vigilant surveillance is recommended for primary MCS, particularly for larger or trunk-based lesions.