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1/80. diagnosis of disseminated alveolar rhabdomyosarcoma using cytogenetics: case report.

    The clinical, histologic and cytogenetic features of a patient with the alveolar subtype of rhabdomyosarcoma (RMS) were investigated. The patient presented with a widely disseminated tumour including bone marrow involvement, and was a diagnostic dilemma. The presence of translocation (2;13)(q37;q14), which is strongly associated with alveolar RMS helped make the diagnosis. A review of other published cases confirms the strong association of (2;13) with alveolar RMS. The importance of considering RMS as a differential diagnosis in patients presenting with disseminated tumour as the only finding is stressed. This case also shows how cytogenetic investigation of similar patients may provide a diagnosis. ( info)

2/80. Resolved splenic accumulation of Tc-99m HMDP after recovery of disseminated intravascular coagulation in a patient with rhabdomyosarcoma.

    Unusual, intense splenic radioactivity was seen on bone scintigraphy with Tc-99m HMDP in a 14-year-old boy with alveolar rhabdomyosarcoma complicated by disseminated intravascular coagulation. Abnormal splenic radioactivity was resolved after recovery from the disseminated intravascular coagulation. During treatment of disseminated intravascular coagulation and tumors, the patient received repeated blood transfusions, resulting in iron overload, but this did not prevent the abnormal splenic uptake from resolving. This case indicates that disseminated intravascular coagulation may be a cause of splenic accumulation of bone-seeking agents, and that abnormal splenic uptake can be resolved. ( info)

3/80. Metastases to the breast from rhabdomyosarcoma: appearances on MRI.

    The authors report a case of blood-borne bilateral metastatic breast disease of alveolar rhabdomyosarcoma (RMS) in a 21-year-old patient. The possibilities of mammography, ultrasound, and MRI in the early detection of breast metastases and their appearance on these modalities are discussed. Whereas mammography rendered no additional information due to dense breast parenchyma and ultrasound showed only a solitary tumor without definite criteria of malignancy, multifocal bilateral spread was verified with MRI and early ring-like enhancement suggested malignancy. Therefore, we conclude that MRI may provide useful information in evaluating patients with sarcomas, even when there is no clinical evidence for metastatic disease of the breast. ( info)

4/80. Massive disseminated intravascular coagulation and hyperfibrinolysis in alveolar rhabdomyosarcoma: case report and review of the literature.

    A 68-year-old woman presented with profuse hemorrhage and other signs suggesting an acute leukemia. Histologic and cytogenetic evaluation of her bone marrow revealed alveolar rhabdomyosarcoma as the underlying cause of massive disseminated intravascular coagulation and hyperfibrinolysis. A review of the literature reveals that coagulopathy appears to be a common feature of alveolar rhabdomyosarcoma. ( info)

5/80. Clinical aspects of alveolar rhabdomyosarcoma with translocation t(1;13)(p36;q14) and hypotetraploidy.

    Although most cases of alveolar rhabdomyosarcoma (RMS) are characterized by the chromosomal translocation t(2;13)(q35;q14), several cases have been reported with a variant t(1;13)(p36;q14). We present the clinical, morphological and cytogenetic features of an alveolar RMS in a 4-year-old boy. Chromosomal analysis revealed a hypertriploid to hypotetraploid karyotype with a t(1;13)(p36;q14) in all tumor cells. It appears that alveolar RMS with t(1;13) occurs in younger children and displays a higher incidence to upper and lower extremity than tumors with t(2;13). ( info)

6/80. Parameningeal alveolar rhabdomyosarcoma with an isolated pancreatic metastasis.

    A 15-year-old girl with alveolar rhabdomyosarcoma of the paranasal sinuses was found to have a solitary visceral metastatic focus in the pancreas. This unusual occurrence is presented and discussed. ( info)

7/80. Alveolar rhabdomyosarcoma presenting with symmetrical bony and renal metastases in infancy.

    Alveolar rhabdomyosarcoma is a small round cell tumour in which may be radiologically and histologically difficult to differentiate from other small round cell tumours such as lymphoma, neuroblastoma and Ewing's tumours. We report a case in infancy of disseminated alveolar rhabdomyosarcoma with symmetrical renal and bony metastases. ( info)

8/80. Duchenne muscular dystrophy and concomitant metastatic alveolar rhabdomyosarcoma.

    The authors report the concomitant occurrence of Duchenne muscular dystrophy (DMD) and alveolar rhabdomyosarcoma (RMS). A 4-year-old boy presented with symptoms involving his neuromuscular system that affected primarily his left hip and leg. Duchenne muscular dystrophy was diagnosed. Seven months later, metastatic alveolar RMS in the ipsilateral pelvis was documented. The diagnosis of one major disorder affecting striated muscle (DMD) may have prevented the early detection of another (RMS). ( info)

9/80. Alveolar rhabdomyosarcoma metastatic to the breast: long-term survivor.

    The case history is described of an adolescent girl with alveolar rhabdomyosarcoma of the maxillary sinus, who was treated with radical radiotherapy and adjuvant chemotherapy. She relapsed in the breast and, after incomplete excision, received radical radiotherapy resulting in long-term survival with breast conservation. The characteristics of patients with metastatic rhabdomyosarcoma with breast involvement are discussed. In adolescent girls, the breast is postulated to be a preferential metastatic site. ( info)

10/80. Temporary remission of an alveolar rhabdomyosarcoma diagnosed and treated as acute leukemia.

    A 29-year-old man with alveolar rhabdomyosarcoma was considered to be suffering from acute leukemia. A bone marrow aspirate had revealed extensive infiltration by atypical blast-like cells which were interpreted as acute lymphoblastic leukemia. Although there was no confirmation of this diagnosis by immunophenotyping chemotherapy with a protocol suited for the treatment of acute lymphoblastic leukemia was started prior to histological analysis and resulted in a complete temporary remission after the first cycle. Histological analysis of a bone marrow biopsy revealed an alveolar rhabdomyosarcoma, as further confirmed by molecular genetic analysis. Two months after the end of chemotherapy, there was an extensive recurrence and the patient died one year after initial diagnosis with chemotherapy refractory disease. In conclusion, rhabdomyosarcoma should always be included in the differential diagnosis of systemic diseases with extensive bone marrow infiltration by tumor cells which could otherwise be misinterpreted as a haematological malignancy. ( info)
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