Multifocal Langerhans cell sarcoma involving epidermis: a case report and review

Area of Science:

• Dermatopathology
• Oncology
• Cancer Biology

Background:

• Langerhans cell sarcoma (LCS) is a rare malignant neoplasm derived from Langerhans cells.
• Cutaneous involvement, particularly primary multifocal LCS, presents unique diagnostic and therapeutic challenges.

Observation:

• A case of primary multifocal Langerhans cell sarcoma involving the inguinal and waist regions of a 41-year-old male patient was analyzed.
• Histopathological examination revealed neoplastic cells with highly malignant cytological features, including irregular shapes, abundant eosinophilic cytoplasm, and large, irregular nuclei with prominent nucleoli.
• Immunophenotyping demonstrated tumor cell expression of CD1a, Langerin (CD207), S-100 protein, CD68, and vimentin, while lacking pan-T/B cell and epithelial markers.

Findings:

• The reported LCS case exhibited aggressive behavior, characterized by rapid local recurrence and distant metastasis to the lungs.
• Despite treatment with radiation and chemotherapy, the patient succumbed to the disease within one year of diagnosis.
• Accurate diagnosis relies on meticulous histopathological evaluation and comprehensive immunohistochemical analysis of tumor cell markers.

Implications:

• This case underscores the aggressive nature of Langerhans cell sarcoma, particularly in its multifocal epidermal presentation.
• Early and accurate diagnosis through histopathology and immunohistochemistry is crucial for appropriate patient management and prognosis.
• Further research into the pathogenesis and treatment strategies for LCS is warranted to improve patient outcomes.