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Rare Tumour (rare + tumour)
Selected AbstractsRegression of post-transplant Kaposi's sarcoma using sirolimusINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 11 2006N. KOLHE Summary Kaposi's sarcoma (KS) is a recognised complication following kidney transplantation, but the incidence varies according to the geographical area. Although it is a rare tumour, its incidence increases dramatically after solid-organ transplantation. The immunosuppressive medications reactivate human herpes virus 8, which has been proposed as the offending agent. The usual treatment of KS is to reduce immunosuppression, chemotherapy and radiotherapy. Nevertheless, the mortality still remains considerably high and has been reported between 8 and 14%. Sirolimus (SRL) has properties which may be useful in the management of some post-transplant tumours such as KS. We report a renal transplant patient with KS, who had multiple relapses after radiotherapy but responded well to the change of immunosuppression from cyclosporine to SRL. [source] Urinary bladder paraganglioma in a case of von Hippel-Lindau diseaseJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 2007RP Athyal SUMMARY Pheochromocytoma is a well-known association in patients with von Hippel-Lindau disease. However, extra-adrenal pheochromocytoma or paraganglioma with this association is rare. We describe a patient with von Hippel-Lindau disease who presented with haematuria and was diagnosed to have a urinary bladder paraganglioma, which is an extremely rare tumour. Herein we report this case which, to the best of our knowledge, represents the first case highlighting the association of urinary bladder paraganglioma with von Hippel-Lindau disease. [source] Solitary extramedullary plasmacytoma of the premaxillaJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 2 2003Vedang Murthy Summary Solitary extramedullary plasmacytoma is a rare tumour with radiotherapy playing an important role in its management. This report describes the case history of a man with a solitary extramedullary plasmacytoma at an extremely rare site, the premaxilla, that posed certain diagnostic and therapeutic dilemmas. The patient underwent surgery followed by postoperative radiotherapy. The use of two modalities of treatment for this localized tumour has been justified by briefly reviewing the literature and defining the various prognostic factors. These prognostic factors in turn should guide the treatment of these uncommon tumours. [source] Prognostic factors of tracheobronchial mucoepidermoid carcinoma,15 years experienceRESPIROLOGY, Issue 2 2008Chien-Hung CHIN Background and objectives: Mucoepidermoid carcinoma of the tracheobronchial tree is a rare tumour which displays a variable degree of clinical aggressiveness and malignancy. The relationship between the patient's prognosis and the tumour's histological features and clinical behaviour is uncertain. The aim of this study was to identify the clinicopathological features and analyse the outcomes of patients with this type of cancer. Methods: A retrospective analysis of the medical records of patients diagnosed with mucoepidermoid carcinoma of the lung between 1991 and 2006 was conducted. Results: The study comprised 15 patients. Higher histological grade tumours had a higher proportion of squamoid cells (P = 0.019); the tumours of patients with lymph node metastases also had a higher proportion of squamoid cells than did the tumours of patients without lymph node metastases (P = 0.015). Patients with early stage tumours (stage IA, IB, IIB) had better outcomes (10-year survival rate = 87.5%), than did patients with late-stage tumours (stage IIIB, IV) (1-year survival rate = 28.6%; 2-year survival rate = 0%, P = 0.001). Patients with lower-grade tumours (grade 1 and grade 2) had better outcomes (1-year survival rate = 80%; 5-year survival rate = 57.1%) than did patients with higher-grade tumours (grade 3) (1-year survival rate = 20%, P = 0.035). Tumour staging was a significant independent predictor of survival on Cox proportional hazards analysis. Conclusions: The proportion of squamoid cells on tumour histology may be an indicator of the level of tumour malignancy. Tumour, node, metastasis staging is a significant determinant of prognosis in patients with tracheobronchial mucoepidermoid carcinoma. [source] Pancreatic solid pseudopapillary tumours , EUS FNA is the ideal tool for diagnosisANZ JOURNAL OF SURGERY, Issue 9 2010Alina Stoita Abstract Background:, Solid pseudopapillary tumour (SPT) is a rare tumour of the pancreas with low malignant potential affecting mainly young women difficult to diagnose preoperatively. The aim of this study is to describe the endoscopic ultrasound (EUS) features and utility of EUS-guided fine needle aspiration (FNA) in diagnosing these tumours. Methods:, A retrospective analysis of SPTs identified in a tertiary institution EUS database between April 2002 and April 2009 was performed. Medical records, imaging, EUS features, cytology and histology specimens were reviewed. Patients were followed up until April 2009. Results:, Seven cases of SPTs were indentified out of 2400 EUS performed. All patients were females with a mean age of 41 years (range 22,69). The tumours were solitary with a mean diameter of 2.9 cm (range 2,4.3 cm). Five tumours were located in the body and tail of the pancreas and two in the neck. All lesions were hypoechoic, heterogenous and well circumscribed, with five having a cystic component and two having a calcified rim. FNA using a 22-gauge needle was performed in six cases with no complications. A preoperative diagnosis of SPT based on cytology was obtained in 5/6 cases (83%). Surgical resection was done in six cases with confirmation of SPT and no metastatic disease. Conclusion:, EUS-guided FNA is a minimally invasive, safe and reliable way of diagnosing SPT by providing characteristic cytological specimens. Definitive preoperative diagnosis leads to targeted and minimally invasive surgical resection. [source] Surgery for gastrointestinal stromal tumour in the post-imatinib eraANZ JOURNAL OF SURGERY, Issue 3 2005Susan J. Neuhaus Gastrointestinal stromal tumour (GIST) is a rare tumour. Historically, surgery has been the only effective treatment. The prognosis of patients with gastrointestinal stromal tumour is poor. Even after apparently ,curative' surgical resection more than 50% of patients relapse. The development of an effective novel targeted therapy against GIST (imatinib mesylate) is a success story of molecular biology that has dramatically altered the management of patients with these tumours. However, as follow up of patients who have initially responded to imatinib has increased, it has become evident that such hopes of cure were premature because responses to imatinib are of limited duration. Unresolved issues include the role of imatinib as an induction (neo-adjuvant) therapy prior to surgery, or as adjuvant treatment after surgery, the role of surgery in patients with a differential or partial response and the role of surgery in patients with isolated metastatic disease. In the present paper the biology and natural history of GIST are reviewed, and the complexities of surgical management that exist in the context of an effective, but not curative, biological therapy, are addressed. [source] Variable expression of tenascin-C, osteopontin and fibronectin in inflammatory myofibroblastic tumour of the lungAPMIS, Issue 2 2010RIITTA KAARTEENAHO Kaarteenaho R, Sormunen R, Pääkkö P. Variable expression of tenascin-C, osteopontin and fibronectin in inflammatory myofibroblastic tumour of the lung. APMIS 2010; 118: 91,100. The aim of this study was to analyse the expression of tenascin-C, osteopontin and fibronectin in inflammatory myofibroblastic tumour of the lung, which is a rare tumour of unknown aetiology. Nine patients with an inflammatory myofibroblastic tumour of lung were studied by immunohistochemistry for the presence of tenascin-C, osteopontin, fibronectin and alpha-smooth muscle actin, which is a common marker for myofibroblasts. The ultrastructure of myofibroblasts was confirmed by transmission electron microscopy. The expression of tenascin-C, osteopontin, fibronectin and alpha-smooth muscle actin was also studied by immunoelectron microscopy. All cases displayed all of the studied extracellular matrix proteins and also alpha-smooth muscle actin-positive spindle-shaped fibroblastic cells that were undoubtedly myofibroblasts. The immunoelectron microscopic studies demonstrated labelling for alpha-smooth muscle actin in intracellular filament bundles within myofibroblasts, for fibronectin in the extracellular filaments of the fibronexus and for tenascin-C extracellularly often adjacent to myofibroblasts. Labels for osteopontin were observed within myofibroblasts and plasma cells. These results demonstrate that tenascin-C, osteopontin and fibronectin were expressed in all three kinds of subtypes of inflammatory myofibroblastic tumours of the lung and further, variable amounts of myofibroblasts could be observed by light and transmission electron microscopy as well as by immunoelectron microscopic techniques. [source] Long-term results of gastrectomy for ,-fetoprotein-producing gastric cancer,BRITISH JOURNAL OF SURGERY (NOW INCLUDES EUROPEAN JOURNAL OF SURGERY), Issue 7 2010M. Inoue Background: ,-Fetoprotein (AFP)-producing gastric cancer is a rare tumour. It is said to have a high incidence of liver metastasis and poor prognosis. This study sought to evaluate long-term outcomes in such patients. Methods: Records of consecutive patients with gastric carcinoma who underwent preoperative measurement of serum AFP levels and gastrectomy were reviewed to identify those who satisfied the following criteria: preoperative AFP level exceeding 40 ng/ml with a decrease after gastrectomy, or raised preoperative AFP level (10,39 ng/ml) and resected tumour showing histologically characteristic features or immunohistochemically positive AFP production. Results: Of 3374 patients with gastric cancer, 53 (1·6 per cent) met the selection criteria. Tumours were characterized by a high incidence of nodal (79 per cent) or liver (53 per cent) metastasis. Preoperative serum AFP levels showed no correlation with tumour size, depth of invasion, disease stage or survival. The 5-year survival rate was 34 per cent. Five patients survived after recurrence following multimodal treatment. A rising AFP level during follow-up always led to tumour recurrence, but the level remained normal in 11 of 31 patients with recurrence. Conclusion: AFP-producing tumours represent a small subgroup of gastric cancer with high metastatic potential. Postoperative serum AFP level can help predict recurrence but a normal level does not mean absence of recurrence. Prognosis is not as poor as previously thought, and multimodal treatment may be worthwhile even in patients with recurrent tumour. Copyright © 2010 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd. [source] Orbital lipomatous haemangiopericytoma: an unusual variantCLINICAL & EXPERIMENTAL OPHTHALMOLOGY, Issue 4 2002Peter EJ Davies MBBS MPH Abstract Haemangiopericytoma (HPC) of the orbit is a rare tumour occurring in all age groups and presenting with slowly progressive proptosis, ocular motility impairment and visual loss. Although most are benign tumours that are resectable at diagnosis, one-third of these tumours demonstrate malignant features and recur locally or metastasize, usually with a fatal outcome. In 1995, a morphologically unique variant, termed lipomatous haemangiopericytoma (L-HPC), was described. Only one case has been reported previously arising in the orbit. In this paper, a case is reported of L-HPC occurring in the orbit and consider the prognostic implications of this HPC variant. [source] An apparently sporadic paraganglioma with an SDHB gene germline mutation presenting at age 68 yearsINTERNAL MEDICINE JOURNAL, Issue 2 2006M. S. Elston Abstract Paragangliomas (PGLs) are rare tumours arising from parasympathetic-associated paraganglia (particularly of the head and neck) or from sympathetic-associated paraganglia such as in the adrenal medulla when they are termed phaeochromocytomas and at extra-adrenal sites in the abdomen and thorax. Recent reports have found frequent germline mutations of VHL, RET, SDHB or SDHD not only in familial cases but also in apparently sporadic cases of phaeochromocytoma. These germline mutations are particularly likely to be found if multifocal disease is present or if the phaeochromocytoma or PGL occurs at a young age. We report a germline splice site mutation in SDHB in a patient presenting with an incidental, apparently sporadic, abdominal sympathetic PGL at 68 years of age. [source] Gastrointestinal stromal tumours: A clinico-radiologic review from a single centre in South IndiaJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 6 2009A Singh Summary Gastrointestinal stromal tumours (GISTs) are rare tumours but are the commonest mesenchymal neoplasms in the gastrointestinal tract. To our knowledge, there is no large case series in Asian countries in which a clinico-radiological descriptive analysis of these tumours has been carried out. In this retrospective study, we analysed our experience of 70 patients with histopathologically proven GISTs, who were presurgically investigated by using CT, and describe the demography, anatomical distribution, imaging features and clinical course of the GIST. We found an unusually large predominance of males in our study, stomach and small bowel appeared to have been involved similarly and small bowel tumours had a higher rate of metastases. We also highlight some unusual CT features of these tumours that we encountered during the study, such as the presence of metastatic lymphadenopathy and satellite nodules, relapse in appendices epiploicae of the bowel, metachronous liposarcoma, adrenal and lung metastases, multiplicity of lesions and aneurysmal dilatation of the bowel. Two of our patients also had multiple neurofibromas, whose association with GIST has been seen in earlier reports. To the best of our knowledge, this article presents one of the largest series of articles on GISTs, to date, in Asian countries. We conclude with a differential diagnosis of GIST, with salient features distinguishing each entity. [source] Pregnancy-related spinal epidural capillary-cavernous haemangioma: Magnetic resonance imaging and differential diagnosisJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 2007T Hakan SUMMARY Epidural haemangiomas are very rare tumours of the spine. Only a few case reports have been published and most of them were cavernous or capillary. To the best of our knowledge, we report the first case of a histologically confirmed epidural capillary-cavernous haemangioma of the thoracic spine presented in the MRI. [source] Angiosarcoma developing in a non-functioning arteriovenous fistula post-renal transplantJOURNAL OF SURGICAL ONCOLOGY, Issue 6 2010Yassar A. Qureshi MBBS Abstract Background Angiosarcomas comprise less than 1% of all sarcomas, arising from endothelial cells of blood or lymph vessels. Chronic immunosuppression increases the risk of many malignancies and an association between the development of angiosarcoma with an immunosuppressed state is established. A few cases have been reported of angiosarcomas arising in the post-renal transplant patient. Specifically, there have been six cases of an angiosarcoma arising in arteriovenous (AV) fistulae in this patient population. We describe a further case and review the relevant literature with specific emphasis on a possible mechanism for the development of angiosarcoma in the post-transplant patient. Case Presentation We report the case of a 48-year-old male who developed an angiosarcoma in a ligated native AV fistula. The lesion arose on the background of immunosuppression following a successful ABO-incompatible renal transplant for chronic renal failure. Conclusion Angiosarcomas are extremely rare tumours but should be considered as a differential diagnosis for an evolving mass near the site of an AV fistula. Diagnosis relies on an index of suspicion and obtaining a definitive histological diagnosis. Both clinicians and patients should be aware that an evolving mass within or around an AV fistula should prompt urgent biopsy. J. Surg. Oncol. 2010; 101:520,523. © 2010 Wiley-Liss, Inc. [source] Surgery for carcinoid tumours of the lower gastrointestinal tractCOLORECTAL DISEASE, Issue 2 2003A. C. Goede Abstract Introduction, Carcinoid tumours are rare neoplasms that originate from neuroendocrine cells of the primitive gastrointestinal tract. Mid- and hind-gut tumours comprise the majority of these rare tumours. With many recent advances in medical treatment the role and importance of surgery and the surgeon needs to be assessed. Method, A Medline, Pubmed and Embase databases search was undertaken. All relevant articles were cross-referenced. Results and conclusions, Incidental findings of carcinoid tumours should be treated at initial surgery whilst elective surgery and further management should be undertaken in specialist centres by a multidisciplinary team. Asymptomatic patients have a better prognosis than those with symptoms. In advanced cases surgery combined with chemotherapy and liver resection is appropriate. The outlook for the majority of cases is good. [source] | ||||||||||||||||||||||