Table 1 |
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| Clinicopathological features of Langerhans cell sarcoma with only cutaneous involvement described in present and previous reports | ||||||||
| No. | Authors (yr.) | Diagnosis | Age (year)/Gender | Clinical presentation | Immunophenotype | EM/MA | Treatment | Outcome |
| 1 | Pileri SA (2002) [5] | Langerhans cell sarcoma | 50/Female | Multiple nodular lesions on skin | CD1a+/S-100+/CD68+/lysozyme+/CD20-/CD21-/CD45-/CD3-/CD30-/MPO-/EMA- | EBERs- | NA | NA |
| 2 | Pileri SA (2002) [5] | Langerhans cell sarcoma | 10/Female | Single nodular lesion on skin | CD1a+/S-100+/CD68+/CD20-/CD21-/CD45-/CD3-/CD30-/CD34-/MPO-/EMA- | EBERs- | Surgery and radiotherapy | Alive in complete remission |
| 3 | Misery L (2003) [6] | Malignant Langerhans cell tumor | 38/Female | Single red, hardened nodular lesion on skin | CD1a+/S-100+/CD68+/HAM56+/lysozyme+/CD45-/HMB45-/EMA-/CK-/CD20-/CD79- | Birbeck granules+ | Surgery with large excision | 2-year follow-up, no relapse/alive |
| 4 | The present case | Langerhans cell sarcoma | 48/male | Ulcerated and nodular lesion on skin | CD1a+/S-100+/langerin+/CD68(focal)+/CD56-/CD3-/CD20-/CD21-/MPO-/CK- | EBERs- | Surgery and chemotherapy with CHOP regimen | PR, 1-year follow-up, no relapse/alive |
EM, electron microscopy; MA, molecular analysis; EMA, epithelial membrane antigen; CK, cytokeratin; MPO, myeloperoxidase; EBERs, Epstein-Barr virus-encoded small RNAs; PR, partial remission; NA, not available.
Li et al. Diagnostic Pathology 2013 8:20 doi:10.1186/1746-1596-8-20
