1/11. Desmoplastic small round cell tumour: a description of two cases and review of the literature.BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a recently described neoplasm, typically occurring in adolescent and young males. It usually shows an aggressive behaviour, presents in the abdomen, often with diffuse peritoneal implants. It has been demonstrated to be a chemosensitive tumour, generally with short-lasting response and poor survival gain from systemic chemotherapy. The authors report two additional cases of DSRCT and review the available medical literature. patients AND methods: Two young males with intra-abdominal DSRCT were treated with a first-line chemotherapy including carboplatin, doxorubicin and etoposide. Results: Both of the patients obtained a partial response after first-line chemotherapy. The first patient started, subsequently, CD34 stem cell mobilisation with high-dose cyclophosphamide (7 g/m(2)) in order to perform high-dose chemotherapy, but CD34 cell count was insufficient to practice leukapheresis; he died 34 months after the diagnosis because of progression of the disease. The second patient underwent cytoreductive surgery, but progressed 2 months later despite second-line treatment; he died 16 months after the diagnosis. CONCLUSION: This experience confirms that DSRCT may be considered a chemosensitive tumour, highly aggressive, with short-lasting response to chemotherapy. Anyway, the recent literature suggests that multidisciplinary treatment including chemotherapy, surgery and radiation might be the proper approach to this rare malignancy.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/11. Case report: adenosquamous carcinoma of the liver successfully treated with repeated transcatheter arterial infusion chemotherapy (TACE) with degradable starch microspheres.This report describes a 58-year-old female with adenosquamous carcinoma (ASC) of the liver presenting with right lower abdominal pain. In most ASC of the liver, surgery is the first choice of treatment. However, surgery often seems to be ineffective because of the aggressive behaviour of this disease. At surgical laparotomy in the present case, there was a large tumour occupying the entire right lobe of the liver and invading the diaphragm. Thus, partial hepatectomy was performed. For the residual tumour, we performed repeated transcatheter arterial infusion chemotherapy with degradable starch microspheres (DSM). After four sessions of transcatheter arterial infusion chemotherapy, tumour size decreased remarkably and tumour markers had also decreased. Despite the poor prognosis, the patient remains alive and well 12 months after laparotomy. It is suggested that minimally invasive transcatheter infusion chemotherapy with DSM can be an effective treatment preserving a high quality of life.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/11. Presacral carcinoid tumour. review of the literature and report of a clinically malignant case.Carcinoid tumours arising in the presacral region are extremely rare and they are usually benign. We report the case of a 37-year-old black man with a clinically malignant carcinoid tumour (well differentiated endocrine carcinoma) occurring in a sacrococcygeal teratoma and already metastasised to pelvic nodes, liver and bone at the time of the initial diagnosis. Such an aggressive behaviour of the presacral carcinoid tumours has never been described. The derivation of these tumours from hindgut rests with reference to embryological development of the tailgut cysts is discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/11. Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease.Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder, with variable clinical manifestations and unpredictable course, associated with an increased incidence of various tumours. Plexiform neurofibromas are hallmark lesions of NF1; they are slow-growing tumours, which account for substantial morbidity, including disfigurement and functional impairment, and may even be life-threatening. Neuroendocrine tumours (NETs), a rare diverse group of neoplasms, are occasionally associated with neurofibromatosis. Pancreatic NETs are tumours with an incidence of less than 1/100 000 population/year and complex patterns of behaviour, which often need complicated strategies for optimal management. We present the case of a young adult with NF1, having a unique concurrence of plexiform neurofibroma involving the liver with an ampullary NET, and we discuss step by step the management in a specialist centre.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/11. Sequential recurrences of ovarian granulosa cell tumour 10 and 11 years after initial diagnosis as haemoperitoneum and subhepatic mass: a case report and review of the literature.Adult granulosa cell tumours (GCTs) are rare ovarian neoplasms characterised by an indolent course and a propensity for late recurrence. Due to frequent endocrine manifestations most GCTs are diagnosed at an early stage. However, clinical behaviour can not be safely predicted on the basis of conventional clinicopathologic parameters. Surgery remains the cornerstone of therapeutic management. We report on a rare case of a Stage IA GCT twice recurring ten and 11 years after initial surgical treatment. The first recurrence presented as an acute abdomen due to haemoperitoneum after tumour rupture. The second recurrence presented as a subhepatic mass. This case emphasises the need for extended, lifelong follow-up even for patients with early stage, apparently completely removed GCTs. Prognostic parameters and therapeutic options especially for patients with recurrent disease are discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/11. Primary carcinoid tumour of the pancreas.Pancreatic carcinoid tumours are extremely infrequent. Usually, the biological behaviour is indolent and diagnosis is late and often casual. We present the case of a patient initially diagnosed as having liver metastasis of unknown origin. PET identified a primary pancreatic site and the initial histologic diagnosis was adenocarcinoma. Following an uncertain response to chemo- and radio-therapy the repeat histologic assessment indicated a carcinoid tumour of the pancreas. After complete surgical resection and liver transplantation, patient remains free of disease. CONCLUSIONS: The co-existence of several diseases with similar morpho-structural features makes diagnosis complicated. PET is of uncertain use in the evaluation of carcinoid tumours, and is considered inferior to 111Indium-octreotide scan. The only curative treatment is surgical resection, with liver transplantation as a valid option in the treatment of these tumours.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/11. Malignant insulinoma presenting with recurrent episodic loss of consciousness.A 57 year old male presented with episodic behavioural abnormalities and loss of consciousness for 2 years. His fasting blood glucose was 20 mg/dl and corresponding insulin level 119 uU/ml. His EEG showed intermittent rhythmic delta activity. Abdominal CT scan revealed an enhancing mass in the tail of the pancreas and secondaries in the liver. After distal pancreatectomy, resection of the left lobe of the liver and chemotherapy, the hypoglycaemic spells subsided. Histopathology revealed an islet cell tumour with metastases in the liver. Episodic neurobehavioural dysfunction should alert towards the possibility of hypoglycaemia.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
8/11. angiomyolipoma of the liver.Hepatic angiomyolipoma is a rare tumour of the liver. Its behaviour is benign and this paper reports the first case described in australia. A review of the literature suggests that the use of ultrasonography, computerized tomography and angiography should enable pre-operative diagnosis to be made with relative certainty, yet the difficulties with histological diagnosis, particularly on needle biopsy, may necessitate resection.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/11. Imaging of small cell carcinoma of the male urogenital tract.Five cases of small cell carcinoma (three of the prostate and two of the bladder) are described to illustrate the unusual behaviour of these tumours. Cerebral, hepatic and bulky lymph node spread with large volume local disease occurred. The role the radiologist may play in these diseases is discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/11. Metastasis in a benign duodenal stromal tumour.Gastro-intestinal stromal tumour (GIST) is increasingly recognized as a distinct entity within the group of soft tissue tumours. Mostly, GIST arises from the muscular components of the stromal layer, but the tumour may also originate from the autonomic nerve system, recently designated as gastro-intestinal autonomic nerve tumour (GANT). The majority of GIST is located in the stomach and small intestine; only 4% of GIST is found in the duodenum. Clinical and pathological criteria to differentiate benign from malignant GIST are not well established. Tumour size and mitotic activity are commonly considered as important features, predicting biological behaviour and outcome. It has been suggested that the clinical course of the GANT-type tumours may be more aggressive. We present a case of a radically resected duodenal stromal tumour with benign features, in a young woman, with metastases to the liver and peritoneum occurring 8 years after the initial diagnosis.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
| | Next -> |