2021 Volume 60 Issue 4 Pages 229-234
Background : Langerhans cell sarcoma (LCS) is an extremely rare high-grade sarcoma composed of cells resembling Langerhans cells. Herein, we report a case of LCS with a splenic metastasis.
Case : A Japanese male patient in his 70s presented with a mass in his right shoulder. The tumor was resected and diagnosed by histopathology as a LCS. About 1.5 years later, enhanced CT revealed a mass in the spleen, which was diagnosed by ultrasound-guided fine needle aspiration cytology and biopsy as a metastasis from the LCS. Cytology showed a large number of isolated atypical cells of heterogeneous shapes, from round to polygonal, with abundant cytoplasm which showed faint light green staining. Multinucleated cells were also evident. The tumor cells showed a high N/C ratio ; the nuclei varied in size and shape, with increase in fine granular chromatin and contained atypical mitoses. Histopathological examination revealed relatively uniform atypical cells with nuclei containing convolutions and grooves and numerous atypical mitoses. Immunohistochemistry revealed positive staining for CD1a and S100P, partially positive staining for Langerin, and a ki-67 labeling index of c.a. 60%.
Conclusion : Diagnosis of Langerhans cell sarcoma may be very difficult. Detailed morphologic observations and immunohistochemistry and/or immunocytochemistry for specific markers such as Langerin would be helpful for an accurate diagnosis.
