Carcinocythemia in a patient with rhabdomyosarcoma

Solid tumor cells are rarely seen in peripheral blood smears. When this occurs the term carcinocythemia is used. This report describes an 18-year-old female who presented with a painless lump in the labia majora associated with pancytopenia. Tumor cells were identified in the peripheral blood smear and the bone marrow aspirate showed a predominant population of small round vacuolated primitive cells, many of which formed clumps of varying sizes. Biopsies of the vulvar mass and bone marrow were interpreted as alveolar rhabdomyosarcoma. Review of the literature revealed 12 previously reported cases in whom carcinocythemia had been documented; rhabdomyosarcoma was the specific cell type involved in only two of these. The median time between detection of the leukemic phase of the tumor and death was 8.5 weeks, reflecting the fact that carcinocythemia, when it occurs, represents the terminal event of the disease. To our knowledge, our case is the third well documented case of rhabdomyosarcoma in leukemic phase so far reported. The clinical evolution as well as the management of this patient will be described in detail along with a review of the pertinent available literature.