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Fulltext Synovial Sarcoma- An unusual cause of Heel Pain

Norhamdan, M.Y., Shahril, Y, Masbah, O., Siti Aishah, M.A.

Malays Orthop J, 2008;2(2):31-33.
MyJurnal

We report a case of 29-year-old female who presented with right heel pain that worsened over a period of two years. The onset of pain was followed by swelling at the medial aspect of right ankle. She was initially treated for plantar fasciitis with multiple steroid injections over the heel. Subsequent MRI revealed a well-defined heterogeneous lesion in continuity with the medial plantar nerve. Excision biopsy was performed and histopathological evaluation revealed monophasic synovial sarcoma. The patient subsequently underwent wide resection and free tissue transfer followed by radiotherapy and chemotherapy. This case highlights an unusual site and presentation of synovial sarcoma which led to delayed diagnosis and treatment.

Matched MeSH terms: Sarcoma, Synovial
Inflamatory pseudotumor of the bladder: a histopathological diagnostic challenge from its malignant variant

Muhammad Akbar, Praveen, S., Christopher, H.C.K., Goh, E.H., Swaminathan, Zulkifli, M.Z.

Journal of Surgical Academia, 2012;2(1):46-49.
MyJurnal

Inflammatory Pseudotumor (IP) of bladder is one of a variety of lesions which may arise from spindle cell lesions in the bladder. It is benign in nature compared to the other end of the spectrum of spindle cell lesions, such as sarcoma, sarcomatoid carcinoma and leiomyosarcoma. The diagnosis of IP and its differentiation from a malignant pathology is a diagnostic dilemma for Urologist and Pathologist as both entities share certain similar morphological and histo-pathological appearance as well as immuno-histo chemistry staining. It is however crucial to avoid misdiagnosis as the treatment option varies significantly between a radical or partial cystectomy with future surveillance cystoscopes. Hereby, we highlight a case of a benign variant of spindle cell tumour of bladder which needed second pathology review for confirmation and thus avoiding a radical cystectomy. We also review the literature on its presentation and emphasis characteristics differentiating a benign from a malignant pathology

Matched MeSH terms: Leiomyosarcoma; Sarcoma
Fulltext Primary Ewing’s sarcoma of the lumbosacral spine: a case report

Zamzuri, Z., Mohd Adham, S.Y., Saufi, M.A., Azian, A.A., Fadhli, M.

International Medical Journal Malaysia, 2012;11(1):47-50.
MyJurnal

Ewing's sarcoma is a rare tumor first discovered by James Ewing in 1921. It is more common in bone or skeletal component compared to soft tissue or extraosseous Ewing's sarcoma. Among soft tissue Ewing’s, spinal cord involvement is rarer with only nine cases reported. We report a case of nine-year-old Malay girl who presented with low back pain for two months following a fall with progressive neurological deficits of bilateral lower limb. Magnetic resonance imaging was suggestive of a well-defined margin of intradural extramedullary tumor. With nerve sheath tumor in mind, surgical excision with laminectomy L2-S1 was performed. Intraoperative finding was an extradural mass from L3-L5 with extension to bilateral neuroforamen. Histopathology report defined a round cell tumour of Ewing’s sarcoma from the mass.

Matched MeSH terms: Sarcoma, Ewing
Fulltext Radical resection of primary leiomyosarcoma of inferior vena cava

Ikhwan Sani Mohamad, Sivabalan Nadarajan, Azim Idris, Hairol Othman, Affirul Chairil, Razman Jarmin

Malaysian Journal of Medicine and Health Sciences, 2018;14(1):61-64.
MyJurnal

Leiomyosarcoma of the Inferior Vena Cava (IVC) is a rare soft tissue tumour which accounts for only 0.5% of all soft tissue sarcomas. The patients usually presented with non specific clinical signs and often diagnosed at advanced stage. We reported a case of a 58-year-old lady who presented with right sided abdominal pain for 6 months. CT scan showed large Level 2 IVC tumour which encased the right renal vein and had no clear plane of demarcation with the caudate lobe of the liver. She underwent radical resection of the IVC tumour with the right kidney removed en bloc. Vascular reconstruction was not performed as established collateral veins present. She was nursed in Intensive Care Unit for 2 days and discharged well at day 5 post operatively. Histopathological examination results revealed leiomyosarcoma of IVC.

Matched MeSH terms: Leiomyosarcoma; Sarcoma
Fulltext Disseminated AIDS-related Kaposi's sarcoma

Tang ASO, Teh YC, Chea CY, Yeo ST, Chua HH

Oxf Med Case Reports, 2018 Dec;2018(12):omy107.
PMID: 30487992 DOI: 10.1093/omcr/omy107

We present a case of disseminated Kaposi's sarcoma with both cutaneous and extracutaneous involvement in an HIV-infected patient with a relatively high CD4 count of 369 cell/mm3. He developed chronic diarrhea, constitutional symptoms, worsening bilateral pleural effusion with respiratory distress, and progression of skin lesions distributed over his chest and extremities. The temporal relationship between rapid clinical progression and initiation of HAART suggested the possibility of Kaposi's sarcoma-associated immune reconstitution inflammatory syndrome, which eventually resulted in the death of this patient.

Matched MeSH terms: Sarcoma, Kaposi
Fulltext Exploring Two Protocols of FISH Using Cytocell SYT-SSX Probe on Formalin Fixed Paraffin Embedded Tissue Sections

Yajid AI, Mohd Nafi SN, Salehan NA, Tuan Sharif SE

Asian Pac J Cancer Prev, 2020 May 01;21(5):1241-1245.
PMID: 32458628 DOI: 10.31557/APJCP.2020.21.5.1241

BACKGROUND: Chromosomal translocation t(X;18)(p11.2;q11.2) is the cytogenetic hallmark of synovial sarcoma and have been identified as an alternative diagnostic strategy in differentiating synovial sarcoma from other histologic mimics. This study was carried out to test the efficacy of two FISH protocols using the SYT-SSX break apart probe from Cytocell.
METHODOLOGY: Representative paraffin blocks of synovial sarcoma were utilized in this study. FISH study was performed on formalin-fixed paraffin embedded tissue sections using the SYT-SSX break apart probe from Cytocell, to detect two form of SYT-SSX transcript, SYT-SSX1 and SYT-SSX2. FISH protocol, including the hybridization was done following two different protocols, Cytocell FISH protocol and Optimized Dako FISH protocol.
RESULTS: Tissue samples subjected to FISH using Cytocell FISH protocol showed the absence of signal corresponding to the probe used. Utilizing Optimized Dako FISH protocol, the two signals (red and green) corresponding to the break-apart probes was detected. These findings suggested that Optimised Dako FISH protocol is more suited for use with the tested probe on paraffin embedded tissues in comparison to Cytocell FISH protocol.
CONCLUSION: Optimised Dako FISH protocol was noted to be more suited for detecting SYT-SSX FISH signals on paraffin embedded tissues in comparison to Cytocell FISH protocol.

Matched MeSH terms: Sarcoma, Synovial
Fulltext Primary Central Nervous System Fibrosarcoma

Vinodh VP, Harun R, Sellamuthu P, Kandasamy R

J Neurosci Rural Pract, 2017 Aug;8(Suppl 1):S111-S113.
PMID: 28936084 DOI: 10.4103/jnrp.jnrp_165_17

We report a rare case of a young female with primary brain fibrosarcoma, and to the best of our knowledge, we believe that only <50 cases have been reported or described worldwide so far. Fibrosarcoma is a malignant neoplasm, in which histologically the predominant cells are fibroblasts that divide excessively without cellular control and they can invade local tissues or metastasize. Primary central nervous system fibrosarcomas are very aggressive neoplasms and generally have a poor prognosis. This tumor is either from sarcomatous transformation of a meningioma or arises de novo within the brain parenchyma. Our patient, a 48-year-old woman, who presented with progressive speech disorder over the period of 4 months, showed a left temporoparietal lesion with surrounding edema and local mass effect. Total surgical resection was achieved. Histopathology revealed classical fibrosarcoma features and secondary screening revealed no other distant lesion as diagnosis of primary brain fibrosarcoma was established. This case is deemed to be extremely rare because most reports claim that recurrence is within 6 months with poor prognosis; however, this patient is currently recurrence-free at 3 years. This would suggest of the possibility for a relook into this disease's course and recurrence rate when complete excision is achieved. Due to extreme rarity of these tumors, more comparative studies will be needed to improve the disease outcome.

Matched MeSH terms: Fibrosarcoma; Sarcoma
Fulltext Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour of breast

Ikhwan SM, Kenneth VK, Seoparjoo A, Zin AA

BMJ Case Rep, 2013 Jun 21;2013.
PMID: 23813511 DOI: 10.1136/bcr-2013-009584

Primary primitive neuroectodermal tumour (PNET) and extraskeletal Ewing's sarcoma belongs to the Ewing's family of tumours. Primary tumours arising from breast are very rare. There are only a few case reports published on primary extraskeletal Ewing's sarcoma and PNET arising from breast. We present an extremely rare case of an inoperable primary Ewing's sarcoma arising from left breast with contralateral breast, lymphatic and lung metastasis.

Matched MeSH terms: Sarcoma, Ewing/diagnosis*; Sarcoma, Ewing/drug therapy; Sarcoma, Ewing/pathology
Fulltext Malignant Peripheral Nerve Sheath Tumor: Treat or Not Treat?

Bin Samsuddin MF, Bin Omar MA

Asian J Neurosurg, 2019 4 3;14(1):283-285.
PMID: 30937055 DOI: 10.4103/ajns.AJNS_332_17

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.

Matched MeSH terms: Sarcoma
Mycetoma or synovial sarcoma? A case report with review of the literature

Jimenez AL, Salvo NL

J Foot Ankle Surg, 2011 Sep-Oct;50(5):569-76.
PMID: 21616683 DOI: 10.1053/j.jfas.2011.04.014

Mycetoma, also commonly referred to as Madura foot, is statistically rare in the United States. However, it is endemic to other parts of the world. It is a pseudotumor characterized by a triad of tumefaction, draining sinuses, and grains. Two types exist, with each caused by different groups of organisms that require different treatment approaches. Therefore, the exact diagnosis and culture of the organism is vital to successful treatment outcomes. Synovial sarcoma, in contrast, is a malignancy much more commonly seen in the United States. It is characterized by a well-circumscribed, often palpable, mass that is usually well delineated on magnetic resonance imaging. It has characteristic histologic and genetic features that help distinguish it from other soft tissue masses. We present a case of a soft tissue mass diagnosed in the United States. The patient had several clinical and radiographic features of synovial sarcoma but the histologic outcome was mycetoma. The case is followed by a review of the published data.

Matched MeSH terms: Sarcoma, Synovial/diagnosis
Fulltext Malignant melanoma of the gastrointestinal tract presenting as a bleeding gastric ulcer

Noraidah M, Jasmi AY

Malays J Pathol, 2003 Jun;25(1):57-61.
PMID: 16196379

Malignant melanoma involving the gastrointestinal tract is diagnosed antemortem in only a small percentage of patients with the disease. Presenting symptoms are often non-specific, causing a diagnostic problem. The vast majority of such melanomas are metastatic from a cutaneous primary, however there is evidence that the tumour can arise de novo in the gastrointestinal system. We report a 74-year-old man with malignant melanoma with an unusual presentation simulating a symptomatic gastric ulcer. He presented with epigastric pain, haematemesis and melaena. Explorative laparotomy revealed a large ulcerated tumour with several pigmented satellite nodules in the proximal stomach, multiple ileal nodules and widespread nodal and liver metastases. Proximal gastrectomy and limited small bowel resection was performed. Histology revealed the tumour to be composed of nests of epithelioid cells with melanin pigment. The tumour cells showed immunohistochemical positivity for S100 protein and HMB45 antibodies. This report emphasizes that melanoma should be a diagnostic consideration in patients with gastric ulcer.

Matched MeSH terms: Sarcoma/pathology
Seroprevalence of human herpesvirus-8 (HHV-8) in countries of Southeast Asia compared to the USA, the Caribbean and Africa

Ablashi D, Chatlynne L, Cooper H, Thomas D, Yadav M, Norhanom AW, et al.

Br. J. Cancer, 1999 Nov;81(5):893-7.
PMID: 10555764

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.

Matched MeSH terms: Sarcoma, Kaposi/epidemiology*
Fulltext Keyhole limpet hemocyanin augmented the killing activity, cytokine production and proliferation of NK cells, and inhibited the proliferation of Meth A sarcoma cells in vitro

Sarker MM, Zhong M

Indian J Pharmacol, 2014 Jan-Feb;46(1):40-5.
PMID: 24550583 DOI: 10.4103/0253-7613.125164

Keyhole limpet hemocyanin (KLH) is a popular tumor vaccine carrier protein and an immunostimulant. The present study aimed to investigate the immunoregulatory activity of KLH on cytotoxicity, cytokines production, and proliferation of natural killer (NK) cells. Moreover, antiproliferative activity of KLH on Meth A sarcoma cells was studied.

Matched MeSH terms: Sarcoma, Experimental/pathology*
Fulltext Oxidative stress and antioxidant status in primary bone and soft tissue sarcoma

Nathan FM, Singh VA, Dhanoa A, Palanisamy UD

BMC Cancer, 2011;11:382.
PMID: 21871117 DOI: 10.1186/1471-2407-11-382

Oxidative stress is characterised by an increased level of reactive oxygen species (ROS) that disrupts the intracellular reduction-oxidation (redox) balance and has been implicated in various diseases including cancer. Malignant tumors of connective tissue or sarcomas account for approximately 1% of all cancer diagnoses in adults and around 15% of paediatric malignancies per annum. There exists no information on the alterations of oxidant/antioxidant status of sarcoma patients in literature. This study was aimed to determine the levels of oxidative stress and antioxidant defence in patients with primary bone and soft tissue sarcoma and to investigate if there exists any significant differences in these levels between both the sarcomas.

Matched MeSH terms: Sarcoma/metabolism*; Osteosarcoma/metabolism*
Fulltext Comparable frequency of BRCA1, BRCA2 and TP53 germline mutations in a multi-ethnic Asian cohort suggests TP53 screening should be offered together with BRCA1/2 screening to early-onset breast cancer patients

Lee DS, Yoon SY, Looi LM, Kang P, Kang IN, Sivanandan K, et al.

Breast Cancer Res, 2012;14(2):R66.
PMID: 22507745

Germline TP53 mutations cause an increased risk to early-onset breast cancer in Li-Fraumeni syndrome (LFS) families and the majority of carriers identified through breast cancer cohorts have LFS or Li-Fraumeni-like (LFL) features. However, in Asia and in many low resource settings, it is challenging to obtain accurate family history and we, therefore, sought to determine whether the presence of early-onset breast cancer is an appropriate selection criteria for germline TP53 testing.

Matched MeSH terms: Sarcoma/genetics
Characteristics of skin cancers among adult patients in an urban Malaysian population

Han WH, Yong SS, Tan LL, Toh YF, Chew MF, Pailoor J, et al.

Australas J Dermatol, 2019 Nov;60(4):e327-e329.
PMID: 31222718 DOI: 10.1111/ajd.13106

There has been a rising incidence of skin cancers among Asians in recent years. We present a retrospective analysis of 106 skin cancers and analysed the demography, clinical subtypes of skin cancers and surgical techniques used for skin cancer treatment. In our population, skin cancers were most frequently basal cell carcinomas and diagnosed among ethnic Chinese patients.

Matched MeSH terms: Sarcoma, Kaposi/epidemiology
Intracranial neoplasms in Malaysia

Selby R, Pereira N

Int Surg, 1973 Aug;58(8):536-41.
PMID: 4738062
Matched MeSH terms: Hemangiosarcoma/epidemiology; Sarcoma/epidemiology
Effective nutritional status screening in orthopaedic oncology patients and post-operative complications

Eu CW, Ajit Singh V, Yasin NF

J Orthop Surg (Hong Kong), 2019 5 16;27(2):2309499019847232.
PMID: 31084248 DOI: 10.1177/2309499019847232

PURPOSE: This study aims to determine the prevalence of malnutrition among orthopaedic oncology patients with malignant tumour during preoperative period.
METHODS: This is a prospective observational study involving patients from the orthopaedic oncology unit who were undergoing surgery. They were assessed with Patient Generated Subjective Global Assessment (PG-SGA), Malnutrition Screening Tool (MST) and 3-minute Nutritional Screening (3MinNS) questionnaires. Anthropometric data such as body mass index, mid upper arm circumference (MUAC) and blood parameters such as serum albumin, total lymphocyte count and haemoglobin were also investigated. Patients were then followed up for 3 months. Post-operative complications were divided into infectious and non-infectious groups. Length of stay and unplanned readmission were also documented.
RESULTS: Prevalence of malnutrition ranged from 13.3% to 45.8% under different nutritional assessment methods. Patients who were determined as malnourished were significantly associated with both infectious and non-infectious post-operative complications ( p < 0.001). PG-SGA and 3MinNS values were also significant in univariate and multivariate analysis, respectively. Low serum albumin (<35 g/L) was associated with post-operative infectious complications, especially surgical site infection ( p < 0.001), prolonged hospital stay ( p = 0.009) and unplanned readmission ( p = 0.017). 3MinNS and Charlson Comorbidity Index were predictive of non-infectious complications, whereas serum albumin and the presence of metastasis were predictive of infectious complications.
CONCLUSION: This pilot study of patients with soft tissue and bone sarcoma of upper and lower limbs showed that malnutrition is a significant independent factor related to infectious and non-infectious complications which leads to unplanned readmission and prolonged length of stay. Periodic screening using the PG-SGA or 3MinNS questionnaires, MUAC and evaluation of serum albumin levels is recommended during clinic session and pre-surgery assessment rounds to identify those predisposed to malnutrition and help in reducing incidence of post-operative complications.

Matched MeSH terms: Sarcoma/complications*
A nonsense mutation in exon 8 of the APC gene (Arg283Ter) causes clinically variable FAP in a Malaysian Chinese family

Mohamed Z, Ahmad R, Yoke NS, Zakaria Z, Ahmad H, Yew TH

Cancer Sci, 2003 Aug;94(8):725-8.
PMID: 12901799 DOI: 10.1111/j.1349-7006.2003.tb01509.x

The present study was carried out to characterize the causative genetic mutation in a medium-sized Malaysian Chinese pedigree of three generations affected with familial adenomatous polyposis (FAP). Clinical data and genetic studies revealed considerable phenotypic variability in affected individuals in this family. Blood was obtained from members of the FAP-01 family and genomic DNA was extracted. Mutation screening of the adenomatous polyposis coli (APC) gene was carried out using the single strand conformation polymorphism (SSCP) technique. The possibility of exon skipping was predicted by splicing motif recognition software (ESEfinder release2.0). SSCP results showed mobility shifts in exon 8 of the APC gene which segregated with affected members of the family. Sequence analysis revealed that the affected individuals are heterozygous for a C847T transition, whilst all the unaffected family members and control individuals are homozygous C at the same position. This nucleotide substitution generates a stop codon at amino acid position 283, in place of the usual arginine (Arg283Ter). We conclude that an Arg283Ter mutation in the APC gene is causative of the FAP phenotype in this family, although there is considerable variation in the presentation of this disease among affected individuals. Computational analysis predicts that this mutation occurs within sequences that may function as splicing signals, so that the sequence change may affect normal splicing.

Matched MeSH terms: Sarcoma Virus, Woolly Monkey
Leukosis and Marek's disease viruses of feral red jungle flow and domestic fowl in Malaya

Weiss RA, Biggs PM

J Natl Cancer Inst, 1972 Dec;49(6):1713-25.
PMID: 4119166
Matched MeSH terms: Avian Sarcoma Viruses

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