Lege Artis Medicinae

[RETROPERITONEAL LIPOSARCOMA]

NÉMETH Hajnalka, KOVÁCS Erzsébet, SÁPY Péter, PÁSZTOR Éva, DEZSŐ Balázs, SZŐLLŐSI Zoltán, PFLIEGLER György

DECEMBER 20, 2007

Lege Artis Medicinae - 2007;17(12)

[INTRODUCTION - Liposarcoma is a malignant soft tissue tumour, which represents less than 0.1% of all human cancers. Approximately 20% of liposarcomas arise in the retroperitoneum. Radical surgical excision is potentially curative, making it the first choice of treatment. For local tumour control palliative chemo- and/or radiotherapy can be used. CASE REPORT - A 28-year-old man with a history of weight loss, fatigue and abdominal tightness was diagnosed to have a giant dedifferentiated retroperitoneal liposarcoma, which showed aggressive growth and gave local recurrences. Multiple surgical excisions were performed, the first two times with curative, subsequently with palliative intent. To reduce the retroperitoneal tumour mass, several chemotherapeutic regimens were applied with complementary radiotherapy. As a result of the combinational therapy, tumour growth stopped temporarily, pressure pain subsided and the patient's quality of life was satisfactory. Finally, distant metastases developed in the bones of the right hip, in the lung and on the serous membranes and after 44 months of follow-up the patient died. CONCLUSION - It has been postulated that ifosfamide and doxorubicin based combined chemotherapy prevents or postpones the development of distant metastases. Considering the significant risk of local and distant recurrences, the use of ifosfamide-doxorubicin based combined chemotherapy is recommended in highgrade retroperitoneal liposarcoma, even following complete surgical excision.]

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[Myelodysplastic syndrome is a heterogeneous group of acquired clonal disorders of the haematopoietic stem cell characterized by ineffective haematopoiesis, peripheral cytopenia, and a high risk of progression to acute leukaemia. It is a common malignant disease with an increased incidence in the elderly population. Classification is based on a 1999 WHO recommendation, in which morphological features as well as clinical and cytogenetic characteristics are taken into account. Combined with the International Prognostic Scoring System (1997), it is suitable to predict prognosis and response to therapy. Clinical features include symptoms caused by anaemia, infections, and bleeding. Diagnosis is based on peripheral cytopenia and dysplastic morphology, as well as normal or increased cellularity in the bone marrow, with more than 10% of dysplastic cells. The verification of cytogenetic abnormalities is important both for confirming the diagnosis and predicting the prognosis. When designing the treatment strategy, it is essential to take the risk of leukaemia into account. On the other hand, the general state of the patient and the presence of accompanying diseases should also be considered. The goal of the treatment is to increase cell count and to decrease transfusion requirement, eventually to improve quality of life. Supportive therapy is an essential part of the management. In addition, growth factors, immunosuppressive and immunomodulatory agents, low-dose chemotherapy may be applied. Today, cure can only be achieved by allogenic stem cell transplantation. Recent findings in the epigenetic intracellular regulation allowed the definition of new therapeutic targets to develop drugs such as inhibitors of DNA methyltransferase and histone deacetylase.]

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