engleski

Rhabdomyosarcoma with bone marrow infiltration: case report

Rhabdomyosarcoma is the most common soft tissue sarcoma of children under 15 years of age. According to the WHO classification of soft tissue tumors the following types of rhabdomyosarcoma are subclassified: embryonal rhabdomyiosarcoma (60-70%), affects mainly children younger than 15 years of age, alveolar rhabdomyosarcoma (20-25%), occurs predominantly in adolescents and young adults and pleomorphic rhabdomyiosarcoma (5%), occurs predominantly in adults older than 40 years of age. The most common diagnostic problem is the least differentiated tumors with primitive, undifferentiated, small cells. These tumors are cytologically indistinguishable from other small blue cell tumors in children. A 14 years old girl was admitted to the hospital in September 2003. She complained about tiredness, myalgia and frequent occurrences of bruises. Physical examination revealed multiple petechiae and hematomas on the skin, an enlarged inguinal lymph node and splenomegaly. The laboratory finding showed increased WBC count - 24.8 x 109/L, low hemoglobin level- 80 g/L and platelet count of 8x 109/L. CT scan detected a large retroperitoneal tumor mass and enlarged tracheobroncihal, retroperitoneal and inguinal lymph nodes. The bone marrow aspirate smears showed massive infiltration by tumor cells. The immunocytochemical staining was positive for PAS, vimentin, desmin, CD 68H and negative for LCA, CD 19, CD 20, CD 3, CD 10, NSE, Ber EP 4, OIL RED and ANAE. In regard of morphollogical, cytochemical and phenotype characteristics, cytologist has diagnosed it is rhabdomyosarcoma. The biopsy of tumor was performed and it confirmed the diagnosis of alveolar subtype of rhabdomyosarcoma. Differential diagnosis of small blue cell tumors in children prents a problem cytomorphologically, especially if they diffusely infiltrate the bone marrow. Additional technologies (cytochemistry, immunocytochemistry, cytogenetics and molecular analysis) are of a significant help and in some cases even crucial for making a diagnosis.

Rhabdomyosarcoma; small blue cell tumors; FNAC; children;

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