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 years old female who presented with a painless lump in the labia majora associated with pancytopenia. Tumor cells were identified int he 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 partinent available literature

Coexistent polycythemia vera and smoldering multiple myeloma: report of a case and review of the literature

Polycythemia vera is a myeloproliferative disorder rarely associated with other hematologic malignancies be sides leukemia. Very seldon, an immunoglobulin abnormality may be seen without clinical evidence of a coexistent plasma cell disorder. The association of polycytemia vera and multiple myeloma is extremely rare and in only six of the previously reported cases, both diagnoses were made simultaneously. We report the case of an asymptomatic 58 years old hipertensive male patient who during a routine lalboratory evaluation was found to have thrombocytosis (931,000/mm3) and mild leukocytosis (14,300/mm3). Bone marrow aspiration revealed an increased number of immature plasma cells with absent iron stores. Further laboratory studies were as follows: hemoglobin level 19.6gm/dl; white blood count 19,200/mm3 and platelet count 486,000/mm3. The total serum proteins were 9.1 gm/dl with a serum protein electrophoresis showing hyper gamma-globulinemia (3.2 gm/dl). Immunoglobulin quantitation showed an IgA of 3 gm/dl. The total red cell mass was 38.6cc/Kg and the arterial oxygen saturation was 93%. The leucocyte alkaline phosphatase test score was 271. These studies suggested the coexistence of two diseases: polycythemia vera and multiple mueloma. A detailed description of the clincial manifestations and the laboratory studies of this case as well as a reviwe of the pertinent literature form the basis of this report