Many studies on the role of apoptosis in cancer development and management have been undertaken. Apoptotic activity depends partly on the balance between anti-apoptotic (Bcl-2) and pro-apoptotic (Bax) activities. This study compared Bcl-2 and Bax expression in the tumour cells and endothelial cells of tumour blood vessels in soft tissue sarcoma, and examined the association of these with tumour characteristics.
Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space. The diagnosis of synovial sarcoma can be very challenging to the pathologists. We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II. The fine needle aspiration cytology of the mass was proved inconclusive. Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma was identified in our center by fluorescence in situ hybridization (FISH) technique. Repeat imaging revealed residual tumor. The patient successfully underwent a second excision of the residual tumor and received adjuvant radiotherapy.
An epidermal cyst is the most common type of cyst to occur in subcutaneous tissue. When its size is greater than 5 cm, it is recognised as a giant epidermal cyst. A subcutaneous giant epidermal cyst with intramuscular extension is extremely rare. The authors report a case of a 74-year-old man who presented with a painless, slow-growing left gluteal mass of 6-month duration. Examination revealed a large left gluteal mass that was fixed to the underlying structures. A small epidermal cyst with visible punctum was noted at the medial aspect of the mass. MRI demonstrated a large, lobulated left gluteal lesion measuring 20 cm×16 cm×10 cm. The lesion was partly within the gluteal maximus muscle and partly within the subcutaneous tissue. MRI and ultrasound features of the lesion were consistent with a giant epidermal cyst with intramuscular extension. The lesion was excised and histology confirmed the diagnosis.
Epithelioid sarcoma is a very infrequent soft tissue sarcoma involving predominantly distal extremities of adolescent and young adult. We hereby report a case of epithelioid sarcoma in a 34-year-old young adult who presented with 1-year history of a painful left upper limb associated subsequently with warm left forearm swelling and a Volkman contracture. He was treated as an inflamed soft tissue condition of the left upper limb. A computed tomography study showed presence of multiple hypodense lesions mainly in the flexor compartment of the left arm as well at the subcutaneous tissue, which measured 1 to 1.7 cm in diameter. Histological examination of the left upper limb mass showed nodular proliferation of epithelioid tumour cells and some with rhabdoid features surrounding a central zone of necrosis and was diagnosed as epithelioid sarcoma. Concurrent presence of epithelioid sarcoma and Volkman ischaemic contracture are rarely seen in clinical practice. The present case highlights the importance of the histology which can be confused with other types of sarcoma or chronic granuloma and even missed at times thereby causing diagnostic problems.
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.
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.
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).
Sarcomas usually frequented in the head and neck region of young adults. Trachea is a rare site, and due to scarce clinical data, its clinical outcome is unclear. We reported a case of 60-year old patient presented with progressive worsening shortness of breath, cough, and progressive worsening dysphagia. Computer tomography scan revealed extensive 2 lobulated soft tissue lesions within and surrounding the trachea at the T4 level. Rigid bronchoscopy with mass cryo-debulking was performed and ultimately synovial sarcoma was diagnosed. Shortness of breath was com- pletely relieved post-procedure.
The main role of the host immune system is to identify and eliminate cancer cells, which is a complex process, but it is not a fail-safe mechanism. Many sarcoma patients succumb to this disease despite treatments rendered. The aim of this pilot study was to compare the levels of CD4+ T-cells, T-regulatory (Treg) cells, and cytokines such as tumor necrosis factor-alpha (TNF-α), interferon-gamma (IFN-γ), interleukin-17A (IL-17A), and transforming growth factor-beta-1 (TGF-β1) in peripheral blood leukocytes of sarcoma patients and healthy controls. For gene expression studies, total ribonucleic acid (RNA) was extracted from peripheral blood leukocytes and genes that were differentially regulated in peripheral blood leukocytes of sarcoma patients compared with healthy controls were determined using a commercial T-helper cell differentiation quantitative polymerase chain reaction (qPCR) array. Flow cytometer analysis was performed on blood samples from 26 sarcoma patients and 10 healthy controls to identify the levels of CD4+ T-cells and T-reg cells. The level of cytokines in plasma and culture supernatant were quantified using commercial enzyme-linked immunosorbent assay (ELISA) kits. A marked reduction in the percentage of CD4+ T-cells (p = 0.037) and levels of TNF-α (p = 0.004) and IFN-γ (0.010) was observed in sarcoma patients. Gene expression analysis showed five genes (homeobox A10 (HOXA10), GATA binding protein 3 (GATA3), prostaglandin D2 receptor 2 (PTGDR2), thymocyte selection associated high mobility group box (TOX), and C-C motif chemokine receptor 3 (CCR3)) were dysregulated (p < 0.05) in sarcoma patients. This study suggests that T-helper-1 immune responses are reduced in sarcoma patients.
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.
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 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.
Adenokarsinoma paru-paru miliari adalah sangat jarang dan agresif. Adenokarsinoma paru-paru biasanya menunjukkan sifat ketulan pada peparu yang berkaitan dengan kerosakan paru-paru dan efusi pleura. Adenokarsinoma ini jarang jarang berpunca daripada tapak selain daripada tapak primer, tidak seperti kanser-kanser lain contohnya kanser tiroid, koriokarsinoma dan sarcoma. Kami membentangkan di sini kes seorang suri rumah berusia 50 tahun yang mengadu batuk-batuk, hilang selera makan dan sukar untuk bernafas selama 1 bulan. Perawat perubatan primer telah merawat beliau sebagai jangkitan kuman pada paru-paru. Disebabkan keadaan yang tidak pulih dan semakin melarat, beliau dibawa ke Jabatan Kecemasan. Oksigen beliau dikesan sebanyak 93% atas udara bilik. Berdasarkan sejarah pesakit dan penemuan X-ray paru-paru, rawatan untuk tuberculosis miliari telah di beri walaupun ujian Mantoux dan kahak negatif. Berikutan peningkatan tanda-tanda dan intubasi, bronkoskopi kemudian mendedahkan diagnosis adenokarsinoma paru-paru. Sebagai pelajaran, penampilan miliari radiografi tidak dikelirukan kepada tuberkulosis sahaja, kerana sebab-sebab berbahaya yang lain perlu disiasat. Bronkoskop dengan analisis histopatologi diperlukan, terutamanya jika Mantoux dan sputum adalah negatif.
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.
We would like to highlight an unusual clinical presentation of Kimura's disease, a rare, benign, idiopathic condition, usually seen as swelling and lesions of the head and neck region and commonly affecting young men of Asian descent. It is rare for this disorder to present with lesions on other parts of the body, especially the lower limbs. We report a 27-year-old man who presented with a large mass located at the inguinal region and extending down to the upper thigh. Due to the large size of the mass (28 x 18 cm), our provisional diagnosis was a soft tissue sarcoma. Open biopsy showed that the lesion was benign.
Biopsy is a crucial step in the management of musculoskeletal sarcoma. The surgical approach to the biopsy site is important, as the tract must be removed en bloc with the tumour during limb sparing surgery so as to reduce the risk of local recurrence. The biopsy tracts of 26 osteosarcoma patients were evaluated histologically for tumour infiltration. Horizontal sections of 1 mm thickness with 2 cm radius from were evaluated from each excised biopsy tract. Five out of 26 cases (19.2%) showed positive tumour infiltration. One case (3.85%) had tumour infiltration in the pseudocapsule, two cases (7.69%) had tumour infiltration into the muscle and two other cases (7.69%) had tumour infiltration into the subcutaneous tissue. None had skin infiltration. We conclude that resection of the entire biopsy tract is indeed mandatory for surgical treatment of osteosarcoma.
Follicular dendritic cell sarcomas (FDCS) are rare neoplasms that involve lymph nodes or extranodal sites. They show varied histological features and thus can be mistaken for carcinoma or sarcoma. Correct identification is important for further management. A 43-year-old Indian female presented with a three-month history of progressive swelling at the right inguinal region. It was excised completely and was reported as lymph node with metastatic poorly differentiated carcinoma based on Haematoxylin and eosin (H&E) stain findings. Computerized tomography (CT) scans of thorax, abdomen and pelvis were normal and did not reveal a primary site. Following this, the case was referred to one of the authors. The slides were reviewed and a variety of immunocytochemical markers were done. The tumour cells were negative for epithelial, melanocytic, neural, leucocyte and soft tissue tumour markers. They were immunopositive for CD21, CD35 and negative for CD68. Based on the immunocytochemical findings, a final diagnosis of FDCS was made. This case highlights the histological and immunophenotypical profile of a rare tumour which requires a high index of suspicion for diagnosis.
Limb salvage surgery is a treatment of choice for sarcomas of the extremities. One of the options in skeletal reconstruction after tumour resection is by using a recycled bone autograft. The present accepted methods of recycling bone autografts include autoclaving, pasteurization and irradiation. At the moment there is lack of studies that compare the effectiveness of various sterilization methods used for recycling bone autografts and their effects in terms of bone incorporation. This study was performed to determine the effects of different methods of sterilization on bone autografts in rabbit by radiological, biomechanical and histopathological evaluations.