An 18-year-old Malay lady was treated with high dose steroids for three and a half years for idiopathic thrombocytopaenic purpura. At 21 years, after a series of relapses, a splenectomy was carried out. In addition, two nodules at the hilum of the spleen were also removed. Histological examination of these nodules revealed features of Kaposi's sarcoma. Kaposi's sarcoma resulting from immunosuppression by corticosteroids is rare. Our patient is the first such case reported in Malaysia.
Fifteen patients underwent surgery for cardiac tumours in General Hospital Kuala Lumpur between October 1984 and June 1989. Twelve of the patients had cardiac myxomas and underwent excision under cardiopulmonary bypass. Two patients had sarcoma, of which one was excised. The other was inoperable. Another patient had a metastalic malignant melanoma which was inoperable. Of the patients 10 were female and five male. Their ages ranged from 16 to 60 years. All were symptomatic and the commonest mode of presentation was exertional dyspnoea and palpitations. Two presented with cerebral embolisation. The three patients with malignant tumours had constitutional symptoms at the time of surgery. All patients had echocardiography pre-operatively to confirm the diagnosis of cardiac tumour. Only one patient underwent preoperative cardiac catheterisation and angiography. The surgical approach in all patients was through a median sternotomy and all except one were operated under cardiopulmonary bypass. There was no intraoperative embolisation. There was one perioperative death. Fourteen patients were followed up for periods ranging from one to 44 months. Three patients with malignant cardiac tumours died. One had recurrence of myxoma 21 months after the initial surgery. We conclude that excision of cardiac myxomas carry a very small risk following which patients have good prognosis. Malignant tumours carry a bad prognosis. From our experience, we conclude that echocardiography is an extremely accurate tool in the diagnosis of cardiac tumours.
Virus associated haemophagocytic syndrome (VAHS), a distinct clinico-pathologic entity, is characterised by systemic proliferation of non-neoplastic histotiocytes showing haemophagocytosis resulting in blood cytopaenia. It has been described in relation to several viruses earlier. Here we describe a young girl who developed this reactive process during the course of dengue haemorrhagic fever (DHF).
Clear cell sarcoma of kidney (CCSK) is a rare but distinct tumor of childhood frequently confused with Wilms' tumor (nephroblastoma). It has a characteristic histology, a marked predilection for metastasis to bone, and an aggressive clinical course with a high relapse rate in spite of surgical excision, chemotherapy and radiotherapy. We report the first histologically proven CCSK in a Malaysian patient. This was an 8-mth-old Malay boy who was clinically diagnosed to have stage I Wilms' tumor. Despite treatment, he developed multiple metastases 10 mths after initial presentation and died soon after. Emphasis is placed on recognizing this entity in view of (1) its naturally aggressive behaviour and (2) the prospect of improving prognosis with currently recommended intensified chemotherapeutic regimes. Its immunohistochemical profile of vimentin-positivity and negativity for epithelial membrane antigen, cytokeratin and Factor-8 related antigen is more in favour of a mesenchymal or glomerular origin than a tubular or vascular origin.
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.
This study explores immunohistochemical characteristics that may be of diagnostic value in differentiating clear cell sarcoma of the kidney (CCSK) from Wilms' tumor (WT) and may provide some insight into the histogenesis of CCSK. Formalin-fixed, paraffin-embedded sections of 8 CCSK and 9 WT were stained, using the standard avidin-biotin peroxidase complex method, for vimentin (VIM), Factor-8 related antigen (F8A), epithelial membrane antigen (EMA), desmin (DES), S-100 protein and Mac 387. CCSK cells consistently exhibited moderate to strong diffuse cytoplasmic positivity for VIM and were negative for F8A, EMA, DES, S-100 and Mac 387. In contrast, only patchy groups of stromal cells and primitive glomeruloid structures in WT exhibited VIM-positivity. Blastemal cells were VIM-negative. Stromal cells with rhabdomyomatous differentiation exhibited cytoplasmic positivity for DES. Epithelial cells of maturing tubular structures showed EMA-positivity whereas immature tubular structures were EMA-negative. Neither blastemal, stromal nor epithelial elements in WT were positive for F8A, S-100 or Mac 387. Podocytes and mesangial cells of glomeruli in 3 mid-trimester human abortuses (controls) exhibited moderate to strong VIM-positivity. The importance of differentiating CCSK from WT has been repeatedly emphasized because of its poorer prognosis and the necessity of adding Adriamycin to the chemotherapeutic regime. The consistent VIM-positivity of CCSK cells can be a useful feature in differentiating it from "blastemal-predominant" WT, with which it is often confused. Although vimentin expression by CCSK cells is consistent with a mesenchymal character, the possibility of a histogenetic link with glomerular podocytes or mesangial cells should also be considered.
Eight histologically-confirmed cases of clear cell sarcoma of the kidney (CCSK) were studied for possible mutations in the p53 tumor suppressor gene by the immunohistochemical demonstration of mutant p53 proteins using a monoclonal (DO7: Dako) and a polyclonal (AB565: Chemicon) antibody to p53 protein. All cases exhibited p53 protein nuclear immunopositivity, although in varying numbers of tumor cells and with different staining intensities. p53 protein (DO7 or AB565) was expressed in < 25% of the tumor cells in four (50%) of the cases, including the one case with a known long term survival of 13 years from the time of diagnosis. The other tumors showed p53 protein immunopositivity in > 25% of the tumor cells when stained with either DO7 or AB565 or both. The intensity of staining, graded on visual impression into weak, moderate or strong, did not correlate well with the ratio of positive staining tumor cells. While this study is unable to clarify the relative prevalence and importance of p53 mutational events in the pathogenesis of this aggressive renal tumor of childhood, it is reasonably suggestive that alterations in the p53 tumor suppressor gene do occur in CCSK.
Kaposi's sarcoma is an uncommon cutaneous neoplasm seen classically in elderly males of East European or Jewish extract. It has been known to be endemic in sub-Saharan Africa for many years. Numerous cases had been described in patients on long-term immunosuppressive therapy and in patients living with acquired immunodeficiency syndrome (AIDS). In spite of the increasing number of organ transplant recipients and people living with AIDS. Kaposi's sarcoma remains rare in Asia. We report two cases seen in Johor, Malaysia.
We report a rare case of undifferentiated (embryonal) sarcoma of the liver in a six-year-old girl who at presentation, had fever, right hypochondrium pain and hepatomegaly. The diagnosis was clinched by fine needle aspiration cytology and was subsequently reconfirmed by histopathological examination of the resected tumour. Pre-operative chemotherapy was given because primary resection was deemed not possible. The patient underwent a successful extensive hepatectomy followed by continuation chemotherapy
A 17 year old Malay student who is a known case of synovial sarcoma of left elbow (treated with an above elbow amputation) presented with duodenal obstruction. We report an unusual case of gastrointestinal tract metastases from synovial sarcoma. To our knowledge, there is no previous such report in the literature. The computed tomography (CT) and magnetic resonance imaging (MRI) features are described.