Eight cases of clear cell sarcoma of kidney were seen in the Department of Pathology, University Hospital, Kuala Lumpur, Malaysia over the 16-year period from 1973 to 1989. Five of the patients were males. Six patients were Malay, one Chinese and one Indian. The patients' ages ranged from 8 months to 3 years. Clear cell sarcoma was the original diagnosis in two patients while six were diagnosed as blastemal-predominant Wilms' tumours at presentation. Metastases developed in five patients. Metastatic sites included the thoracic vertebra, skull, orbit, humerus, radius, ulna, shoulder, lung and liver. The prolonged survival, of 9 years and 9 months, seen in one patient despite omission of Adriamycin (doxorubicin) from the chemotherapeutic protocol is highlighted. We also emphasise the histological factors which are of help in differentiating clear cell sarcoma from Wilms' tumour.
Mesenchymal stem cells (MSCs) have garnered vast interests in clinical settings, especially in regenerative medicine due to their unique properties-they are reliably isolated and expanded from various tissue sources; they are able to differentiate into mesodermal tissues such as bones, cartilages, adipose tissues, and muscles; and they have unique immunosuppressive properties. However, there are some concerns pertaining to the role of MSCs in the human body. On one hand, they are crucial component in the regeneration and repair of the human body. On the contrary, they are shown to transform into sarcomas. Although the exact mechanisms are still unknown, many new leads have pointed to the belief that MSCs do play a role in sarcomagenesis. This review focuses on the current updates and findings of the role of MSCs in their transformation process into sarcomas.
Pleomorphic sarcoma is the most common sarcoma. Reports of outcome as well as evaluation of prognostic factors in the literature show great variation. We looked at our experience in treating this tumour at University Malaya Medical Center. This is a review of patients diagnosed with Pleomorphic Sarcoma from January 1990 to December 2005 at University Malaya Medical Center. Outcome measures studied are the overall survival, disease free survival and local recurrence of disease. Prognostic factors for survival and local recurrence which were studied are the tumour size, depth, stage, type of surgery, adjuvant therapy, and surgical margin. There were fifty four patients available for analysis of demographics. The mean age at presentation was 52.3 +/- 16.7 years. There were thirty male patients (56%) and twenty four female patients (44%) in the study population. The patients were predominantly Malay (44.4%) and Chinese (42.6%). There were two Indian patients (3.7%) and five patients from other races (9.3%). Thirty patients had disease affecting the extremities while six patients had disease affecting the trunk. Patients with tumour affecting the trunk had 100% mortality. In patients with tumour affecting the extremity, 46.7% presented with Stage 3 disease. The overall median survival was 39 months. The overall survival rate at 3 years was 53.3% and the 5 years was 30.0%. The disease free survival rate at five years was 27.6%. However, if patients who presented with metastasis were excluded, the 5 year survival rate was 60% while the disease free survival was 53.3%. Recurrence rate was 33.3%. Factors affecting survival was stage, size and location of tumour. No factors were found to correlate with higher local recurrence rate. In conclusion, Pleomorphic Sarcoma is a heterogenous disease with variable outcome. In our centre, late presentation with advanced disease significantly affects the overall outcome of this condition. Tumour size and location are important prognostic factors. Inherent tumour behavior and aggressiveness probably outweigh current treatment modalities as the most important prognostic factor in the management of Pleomorphic Sarcoma.
The authors present an interesting case under our follow-up who has had five different forms of tumours with different pathologies throughout his lifetime. He started off with hepatoma, followed by pleomorphic sarcoma of the thigh, adenocarcinoma of the prostate, meningioma and finally schwanoma. He is still alive to this date.