Clear cell sarcoma and selective IgM deficiency: a case report.
A case of clear cell sarcoma of tendons and aponeuroses and a co-existent IgM deficiency is reported. The tumor arose in the Achilles tendon with metastases to the skin, bone, and lymph nodes. The tumor, examined by light and electron microscopy, consisted of glycogen-containing clear cells with melanotic and amelanotic features. There was no detectable serum IgM. The IgA levels were normal and IgG levels were elevated. Peripheral blood lymphocytes contained a normal amount (4.5%) of IgM-bearing cells. Cultured mononuclear cells from the patient suppressed production of IgM by normal lymphocytes, suggesting a role of suppressor cells in the IgM deficiency. The co-existence of soft tissue sarcomas and immunoglobulin deficiency states is reviewed.