Seroprevalence of HHV-8 has been studied in Malaysia, India, Sri Lanka, Thailand, Trinidad, Jamaica and the USA, in both healthy individuals and those infected with HIV. Seroprevalence was found to be low in these countries in both the healthy and the HIV-infected populations. This correlates with the fact that hardly any AIDS-related Kaposi's sarcoma has been reported in these countries. In contrast, the African countries of Ghana, Uganda and Zambia showed high seroprevalences in both healthy and HIV-infected populations. This suggests that human herpes virus-8 (HHV-8) may be either a recently introduced virus or one that has extremely low infectivity. Nasopharyngeal and oral carcinoma patients from Malaysia, Hong Kong and Sri Lanka who have very high EBV titres show that only 3/82 (3.7%) have antibody to HHV-8, demonstrating that there is little, if any, cross-reactivity between antibodies to these two gamma viruses.
Malignant peripheral nerve sheath tumours (also called neurofibrosarcomas) are a rare, highly aggressive soft tissue sarcomas that arise from the peripheral nerves or cells associated with the nerve sheath, such as Schwann cells, peri-neural cells and fibroblasts. It is representing 10% of all soft tissue sarcomas in which it is considered as an extremely rare malignancy, especially in patients with neurofibromatosis type I. In the general population, it affects approximately 1 in 100,000 people. This article is reporting a 56-year-old Malay female patient who is a known case of neurofibromatosis type I for 20 years, presented with a lower back, pruritic, gradually increasing swelling during the last five months. Last month before the presentation, the lesion rapidly grows, reaching a size of (15×15 cm), accompanied by foul-smelling discharge and pain exacerbated with movement. Although no history of preceding trauma or accident, the mass bleeds within contact. In conclusion, only a few cases of giant malignant peripheral nerve sheath tumours reported in the literature describing its location and growth progression. We present a massive, extremely rapid growth of cutaneous exophytic malignant peripheral nerve sheath tumours over the lower back.
Endometrial stromal sarcoma is a rare tumour of the uterus. We reported a case of a young lady with endometrial stromal sarcoma. She became pregnant while having the disease and delivered a healthy baby, her sixth, without any complication. A total abdominal hysterectomy with bilateral oopherectomy was performed subsequently. She refused any added treatment after the operation. To date, she is free of any recurrence.
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.
Currently, of less than 50 cases of head and neck follicular dendritic cell (FDC) sarcoma reported in the literature, 5 have been found to occur in the background of Castleman disease. We report another case of head and neck FDC sarcoma with emphasise on its associated lesions and review the outcome of treatment from the existing cases in the literature.
Ewing sarcoma is a primary bone malignancy that rarely occurs in the hand. Resection and reconstruction will usually result in reduced or loss of thumb function. We describe a case of successful transplantation of non- vascularized osteoarticular second metatarsal autograft following wide resection of Ewing sarcoma of first metacarpal in an 11-year-old girl. The capsule of the graft’s metatarso-phalangeal joint was repaired to achieve mobility of the joint. This enabled preservation of thumb function by a relatively simple surgical technique. Detailed surgical procedures and excellence function after 2 years following surgery are described.
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.
Endometrial stromal sarcoma (ESS) is a rare malignant tumour of the endometrium, accounts for less than 1% of all
uterine malignancies. Routinely, it is diagnosed morphologically, supported by immunomarkers of CD10 and
vimentin. CD56 is used widely in neuroendocrine tumour. In our current practice, CD56 is not used to support the
diagnosis of ESS. We present a case of a postmenopausal lady with advanced ESS who had expression of CD56
upon immunohistochemical study
Nodular fasciitis (NF) is an uncommon and benign tumour-like fibroblastic proliferation that is difficult to distinguish from sarcoma both clinically and histologically. In addition, it is a type of lesion characterised by having a potential for spontaneous regression. NF is frequently misdiagnosed due to its rapid growth, rich cellularity, and mitotic figure. Although NF is only rarely diagnosed in children, the head and neck represent the most common locations for NF among this population. The cause of NF remains unknown, however trauma is believed to be an important triggering factor. We describe an unusual case of NF in the posterior ethmoid sinus in a six-year-old boy with no history of trauma. The NF was incidentally noted on a computed tomography scan after the patient complained of a squint. The diagnosis of NF was also supported by histopathology and specific immunohistological staining. A surgical biopsy was performed, and no recurrence was observed after one year.
Synovial sarcoma (SS) is a malignant soft tissue tumour of uncertain histogenesis which is defined by the translocation t(X;18) that produces the fusion oncogenes SYT-SSX. The emergence of transducer-like enhancer of split 1 (TLE1) as a new immunohistochemical (IHC) marker for SS has offered an alternative to pathologists in differentiating SS from other histological mimics, especially in the setting of limited molecular facilities. We investigated the utility of IHC TLE1 expression against histomorphological features and other IHC markers in SS and non-SS tumours. Twenty-six cases of histologically diagnosed SS and 7 non-SS (for which SS was in the differential diagnosis) were subjected to TLE1 IHC staining, which was graded from 0 to 3+. Of the 26 SS cases, 12 each were biphasic and monophasic types and 2 were poorly-differentiated. TLE1 was expressed in 22/26 (84.6%) SS cases, of which 11/12 (91.7%) were biphasic, 10/12 (83.3%) monophasic and 1/2 (50%) poorly-differentiated tumours. Two of 7 (28.6%) non-SS cases were positive for TLE1. Immunopositivity of SS and non-SS cases for EMA were 20/26 (76.9%) and 2/7 (28.6%) respectively and for CK7 were 7/26 (26.9%) and 0/7 (0%) respectively. All cases were negative for CD34. Consistent histomorphological features for SS included mild nuclear pleomorphism, alternating tumour cellularity, fascicular growth pattern and thick ropy stromal collagen. In conclusion, TLE1 is not a stand-alone diagnostic IHC marker for SS. However, in the absence of molecular studies, it can contribute added diagnostic value in combination with morphological evaluation and other IHC markers such as EMA and CD34.
Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon, biologically aggressive soft tissue sarcomas of neural origin that poses tremendous challenges to effective therapy. MPNSTs are among the most challenging mesenchymal malignancies to treat with poor prognosis. They usually affect young and middle-aged adults, tend toward early metastasis, and often demonstrate resistance to chemotherapy. We present a case of a 23-year-old female who initially presented with the right temporal swelling for 1 month associated with constitutional symptom which progressively worsening. The right craniotomy and excision biopsy were done with histopathological examination results suggestive of MPNST. Thorax-abdominal-pelvic computed tomography and magnetic resonance imaging further revealed multiple metastatic lesions involving spine, retroperitoneal, pelvic, chest wall, and lungs. This case illustrates the typical presentation of MPNST with its known poorly outcome.
Osteoarticular allografts represent a reconstructive option after bone tumor resection around the knee in growing children. The major advantage is the chance to preserve the growth plate of the remaining bone, but the disadvantage is the high failure rate eventually requiring definitive prosthetic replacement at skeletal maturity. We retrospectively reviewed 22 patients who underwent osteoarticular allograft reconstructions of the distal femur (16) or proximal tibia (6). There were 12 females and 10 males with an average age at surgery of 11 years (7-15). The diagnosis was osteosarcoma in 19 cases and Ewing sarcoma in 3. All patients underwent pre- and post-operative chemotherapy. At an average follow-up of 103 months (12-167), 18 patients (82%) were alive and 4 had died (18%). We observed 10 allograft failures requiring prosthetic replacement, 6 in distal femur and 4 in proximal tibia reconstructions. At last follow-up 8 allografts (36%) were still in place. Overall allograft survival was 79.6% at five and 45.8% at ten years. In distal femur, allograft survival was 86.2% at five and 59.1% at ten years. In proximal tibia, allograft survival was 62.5% at 5 years and 31.2% at 67 months. Average limb shortening was 3 cm (0- 5) in 8 patients with the allograft still in situ and 2 cm (0-4) in 10 patients after prosthetic replacement. Average MSTS functional score of the whole series was 25 (83.7%). The MSTS score of patients after revision with prosthetic replacement was 24 (80%) while patients who still had the allograft retained had an average MSTS scores of 26.8 (89.3%). In conclusion, osteoarticular allograft reconstruction of the knee after bone tumor resection in pediatric age can be considered a temporary solution with the aim to limit limb length discrepancy before definitive prosthetic replacement after skeletal maturity.
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.
Natural history of abdominal wall soft tissue sarcoma is still poorly understood due to its rarity. In unpublished data of our institution, only seven cases of abdominal wall soft sarcoma with ICD-10 coding of 49.4 were found for past 10 years. We illustrate a case of juvenile fibrosarcoma of anterior abdominal wall. This is a case of young girl with anterior abdominal wall tumour, underwent wide local excision with immediate reconstruction. There are few options of surgical treatment for this case, but which is the best. It is always a challenge in managing young patient with giant abdominal wall defect in view of long term effect namely weakened abdominal wall, pregnancy related issue and risk of herniation and surgical site recurrence as well.
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.
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.
Hemangiopericytomas (HPCs) are uncommon tumours. We present the case of a 41-year-old female with multiple resections at different sites over the course of 11 years. The approach considerations, as well as treatment options and prognosis are discussed. A 41-year-old female with two previous resections for intracranial meningeal HPC in 2004 and 2008, as well as adjuvant radiotherapy, presented in 2015 with left intrathoracic and left hip recurrence confirmed by positron emission tomography/computed tomography (PET/CT). She underwent left proximal femur resection/reconstruction and video-assisted thoracoscopic surgery (VATS) resection of the intrathoracic tumour was attempted. She was discharged home on her 4th post-operative day with minimal pain. There were no neurosensory or motor deficits. Any patient who has been diagnosed with HPC in the past who develops new symptoms should be worked up for recurrence, regardless of the length of disease-free interval, as our case study suggested. There has yet to be a standardized follow-up regime due to the rarity of these tumours. HPC remains a rare soft tissue sarcoma with high recurrence rate. Planned VATS evaluation and resection is possible provided complete resection with clear surgical margins can be achieved, as clear surgical margins offer the best chance of survival.