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Malignant Peripheral Nerve Sheath Tumour (malignant + peripheral_nerve_sheath_tumour)
Selected AbstractsCytomorphological study of soft tissue neoplasms: role of fluorescent immunocytochemistry in diagnosisCYTOPATHOLOGY, Issue 5 2005B. Rekhi Objectives:, Exact categorization of soft tissue tumours (STTs) on smears requires application of various ancillary techniques. This study was aimed at evaluating the role of fluorescent immunocytochemistry (FICC) in cyto-diagnosis of 30 STT cases. Methods:, Thirty cases of soft tissue tumours were included in the present study. All cases were subjected to routine Giemsa and Papanicolaou stain. Extra smears were made and kept for fluorescent immunostaining. A panel of cytoskeletal antibodies, tagged with FITC (Fluorescein isothyocynate), was employed in all these cases. Fluorescent immunostained smears were examined under Zeiss Confocal Laser scanning microscope, using double immunofluorescence (red-green). Finally, all cases were subjected to biopsy and again immunoperoxidase staining. Results:, Among the 30 cases in the present study, unaided cytological diagnoses ranged from ,spindle cell' tumour in four (13.3%) cases, benign and malignant spindle cell tumour in 17 (56.6%) cases, to malignant mesenchymal tumour in nine (30%) cases. FICC helped in further correct categorization of 25/30 (83.3%) cases viz. leiomyoma (three), benign neurogenic tumour (six), schwannoma (one), dermatofibrosarcoma protuberans (three), synovial sarcoma (two), rhabdomyosarcoma (two), malignant fibrous histiocytoma (five) and malignant peripheral nerve sheath tumour (three). Aggressive fibromatosis was found to be a missed diagnosis in two cases. Overall concordance between cyto-diagnosis with FICC, and histopathology results was 83.3% (P < 0.05). Conclusion:, Fluorescent immunocytochemistry is a significant ancillary technique for making a rapid and specific diagnosis of STT, as required for their timely management. Incorporation of a wide panel of antibody markers with clinico-cytological correlation is recommended in forming an exact diagnosis in these cases. [source] Integrative genomic analyses of neurofibromatosis tumours identify SOX9 as a biomarker and survival geneEMBO MOLECULAR MEDICINE, Issue 4 2009Shyra J. Miller Abstract Understanding the biological pathways critical for common neurofibromatosis type 1 (NF1) peripheral nerve tumours is essential, as there is a lack of tumour biomarkers, prognostic factors and therapeutics. We used gene expression profiling to define transcriptional changes between primary normal Schwann cells (n,=,10), NF1-derived primary benign neurofibroma Schwann cells (NFSCs) (n,=,22), malignant peripheral nerve sheath tumour (MPNST) cell lines (n,=,13), benign neurofibromas (NF) (n,=,26) and MPNST (n,=,6). Dermal and plexiform NFs were indistinguishable. A prominent theme in the analysis was aberrant differentiation. NFs repressed gene programs normally active in Schwann cell precursors and immature Schwann cells. MPNST signatures strongly differed; genes up-regulated in sarcomas were significantly enriched for genes activated in neural crest cells. We validated the differential expression of 82 genes including the neural crest transcription factor SOX9 and SOX9 predicted targets. SOX9 immunoreactivity was robust in NF and MPSNT tissue sections and targeting SOX9 , strongly expressed in NF1-related tumours , caused MPNST cell death. SOX9 is a biomarker of NF and MPNST, and possibly a therapeutic target in NF1. [source] PKC-theta expression in Ewing sarcoma/primitive neuroectodermal tumour and malignant peripheral nerve sheath tumourHISTOPATHOLOGY, Issue 3 2009Gu Hyun Kang No abstract is available for this article. [source] Intra-abdominal spindle cell lesions: a review and practical aids to diagnosisHISTOPATHOLOGY, Issue 5 2001A Al-Nafussi Intra-abdominal spindle cell lesions: a review and practical aids to diagnosis Intra-abdominal spindle cell lesions are uncommon and often present a diagnostic challenge. An important group of such lesions are the gastrointestinal stromal tumours. Other intra-abdominal spindle cell lesions include fibromatosis, various sarcomas,in particular, leiomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumour,and, in women, endometrial stromal sarcoma. Less common lesions are inflammatory myofibroblastic tumours, the mesenteric spindle cell reactive lesions, retroperitoneal fibrosis, and solitary fibrous tumour. A variety of intra-abdominal tumours of nonmesenchymal origin may have a spindle cell/sarcomatoid morphology; these include sarcomatoid carcinoma, malignant melanoma and, in women, sarcomatoid granulosa cell tumour. Finally, metastatic sarcomas from pelvic or extra-abdominal organs need also be considered. A set of practical aids to the diagnosis of intra-abdominal spindle cell lesions is presented to assist pathologists dealing with such lesions, particularly with regards to the consideration of differential diagnoses. [source] Vascular reconstruction in lower limb musculoskeletal tumoursANZ JOURNAL OF SURGERY, Issue 9 2009J. Ian Spark Abstract Background:, Individual experience in the investigative, planning and operative aspects of lower limb musculoskeletal tumours is often small, making comparison between results difficult. The aim of the study was to describe the recent experience of a single tertiary referral unit performing limb salvage surgery, to identify areas of concern that are amenable to intervention and to provide clinicians an understanding of the surgical options. Methods:, Nine patients with peripheral limb musculoskeletal tumours are described. Four patients had a leiomyosarcoma, and one each of osteosarcoma, synovial chondrosarcoma, synovial sarcoma, liposarcoma and recurrent malignant peripheral nerve sheath tumour. Results:, Thirty-day mortality was nil. Two patients (one with a leiomyosarcoma and one with an osteosarcoma) died at 6 months follow-up because of pulmonary metastases. One patient with synovial chondrosarcoma developed a local recurrence and underwent an above-knee amputation. Six patients at 18 months follow-up are alive with no evidence of local recurrence and a functional lower limb. Conclusion:, These cases are a challenge to the clinicians, radiologists and pathologists. Review by a multidisciplinary team can produce successful results with low post-operative morbidity and mortality. Longer follow-up is required to determine the long-term implications. [source] Parapharyngeal malignant peripheral nerve sheath tumour in conjunction with ganglioneuroblastoma involving the mandibleANZ JOURNAL OF SURGERY, Issue 6 2009Faruque Riffat No abstract is available for this article. [source] Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2005L. Valeyrie-Allanore Summary Background, Neurofibromatosis 1 (NF1) is a common genetic disorder with variable clinical manifestations and an unpredictable course. Plexiform neurofibromas are common complications of NF1. Their malignant transformation is the main cause of mortality in adult patients with NF1. Objectives, To identify clinical factors associated with malignant transformation of plexiform neurofibromas. Methods, Using the database of our neurofibromatosis clinic we included in a retrospective study all patients with NF1 having at least one peripheral nerve sheath tumour for which they underwent surgery or surgical biopsy. Predictive values for malignant transformation of three clinical symptoms, i.e. pain, enlargement of mass and neurological symptoms, were evaluated in association with histological parameters. Results, Of 69 patients studied, 48 had at least one plexiform neurofibroma and 21 had a malignant peripheral nerve sheath tumour. Only enlargement of the tumour had high negative and positive predictive values for malignant transformation: 0·92 and 0·95, respectively. In multivariate analysis, tumour enlargement was independently associated with malignant transformation (odds ratio 167·8, 95% confidence interval 14·0,2012·1). Conclusions, From a practical point of view, pain, neurological deficit and enlargement of a pre-existing peripheral nerve sheath tumour in NF1 must lead to deep surgical biopsy to rule out malignant transformation. [source] | ||||||||||