Epithelioid leiomyosarcoma with rhabdoid features.

Area of Science:

• Oncology
• Pathology
• Genitourinary Oncology

Background:

• Leiomyosarcomas (LMS) are the most common uterine sarcomas.
• Rare variants include epithelioid LMS, myxoid LMS, and LMS with rhabdoid features.
• Rhabdoid cells, characterized by glassy cytoplasm and inclusion bodies, are typically found in infantile renal tumors.

Observation:

• A 56-year-old woman presented with a left inguinal mass.
• Microscopic examination revealed a tumor with spindle, epithelioid, and rhabdoid cell components.
• Immunohistochemistry showed myoglobulin and desmin positivity in rhabdoid cells, cytokeratin in epithelioid and some rhabdoid cells, and SMA in epithelioid and spindle cells.

Findings:

• Histopathologic and immunohistochemical findings support the diagnosis of epithelioid LMS with rhabdoid features.
• This represents a very uncommon variant of LMS, with few reported cases in the literature.
• Rhabdoid cell presence is associated with aggressive tumor behavior.

Implications:

• This case highlights the importance of recognizing rare LMS variants.
• Accurate diagnosis of epithelioid LMS with rhabdoid features is crucial for appropriate patient management.
• Further research into the behavior and treatment of such rare sarcomas is warranted.