Langerhans cell sarcoma, LCS

Definition

A neoplasm of Langerhans cells with overtly malignant cytology.

Equivalent normal cell

As for Langerhans cell histiocytosis.

Epidemiology

Exceedingly rare.

Clinical features

There is multiorgan involvement.

Histopathology

The malignant cells may show occasional nuclear grooves, but are not readily recognisable as Langerhans cells. Eosinophils are sparse. Lymph node involvement is commonly sinusoidal1.

Immunohistochemistry

As for Langerhans cell histiocytosis. S-100 is consistently positive. Positivity for CD1a may only be focal.

both should be positive	S-100	positive0; 9/91
both should be positive	CD1a	positive0; 7/71
CD45		variably weakly positive0, 4/91
CD68		variably weakly positive0, 9/91
lysozyme		variably weakly positive0, 7/71
myeloid markers	myeloperoxidase	0/81
myeloid markers	CD34	0/61
FDC-markers	CD21	0/91
	CD35	0/71
	CNA.42	4/71
T-lineage markers	CD3	0/91
B-lineage markers	CD20	0/91
	CD79a	0/81
	CD35	0/71
others	CD30	0/71

Differential diagnosis

Other histiocytic and dendritic cell neoplasms; see the immunohistochemical differentiation of histiocytic and dendritic cell neoplasms

Management

Prognosis

This is an aggressive neoplasm: about one half of cases prove fatal1.

References

0World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.

1Pileri, S.A., Grogan, T.M., Harris, N.L., Banks, P., Campo, E., Chan, J.K., Favera, R.D., Delsol, G., De Wolf-Peeters, C., Falini, B., Gascoyne, R.D., Gaulard, P., Gatter, K.C., Isaacson, P.G., Jaffe, E.S., Kluin, P., Knowles, D.M., Mason, D.Y., Mori, S., Muller-Hermelink, H.K., Piris, M.A., Ralfkiaer, E., Stein, H., Su, I.J., Warnke, R.A. and Weiss, L.M. Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology 2002;41:1-29.

This page last revised 24.12.2002.