1/29. Rhabdoid tumour of the lung is a dedifferentiated phenotype of pulmonary adenocarcinoma.AIMS: Primary rhabdoid tumour of the lung is rare, and histological and biological characteristics have not been fully documented. We describe three cases of primary lung rhabdoid tumour, all associated with adenocarcinoma, and investigate the histological features and biological characteristics. methods AND RESULTS: Three cases were obtained from a total 902 cases of surgically removed primary lung tumours between 1986 and 1998. The rhabdoid cells were found to occupy about 50-90% of each tumour. All of the tumours had nonrhabdoid adenocarcinoma foci in the centre of the tumours. Transition between the adenocarcinomatous and rhabdoid components was demonstrated. Detailed immunohistochemical studies were carried out. The epithelial markers, cytokeratins and epithelial membrane antigen (EMA), were strongly expressed in rhabdoid and adenocarcinomatous components. Furthermore, surfactant apoprotein A was positive in both components in one case, but myoglobin, MyoD and HHF35 were not expressed. vimentin was strongly and diffusely stained in all cases. The neuroendocrine markers, chromogranin a (all cases), neuron-specific antigen (NSE) (two cases) and CD56 (one case) were occasionally positive in only a small number of the rhabdoid tumour cells. GM-CSF was positively stained in one case, and the dedifferentiated characteristics of the rhabdoid cells was suggested. Proliferative cell nuclear antigen (PCNA) was strongly demonstrated in the rhabdoid tumour cells (all cases). To gain better understanding the highly proliferative characteristics of the tumours, p53 gene (exons 5-8) mutation was examined by dna sequencing analysis; mutation of the p53 dna was not detected. Overexpression of p53 protein was also not demonstrated in all cases. HPV6 was demonstrated in one case by PCR method and also non-isotopic in-situ hybridization (NISH). Two cases died in a short period of time (3 years and 4 months, respectively). CONCLUSION: The rhabdoid cells in these three cases were considered to represent the dedifferentiated components of the accompanying adenocarcinoma. Dedifferentiated characteristics (neuroendocrine markers, GM-CSF, vimentin, and the aggressive behaviour) were evident.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/29. Metastatic placental site trophoblastic tumor: a case report.Placental site trophoblastic tumor (PSTT) is very rare. It is an unusual variant of gestational trophoblastic neoplasia usually confined to the uterus, although 10% of patients have metastases. The clinical behaviour of PSTT varies and despite knowledge of its histology, diagnosis of this rare form of trophoblastic disease and prediction of its biological behaviour remains difficult due to only a few cases reported in literature.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
3/29. Cutaneous metastases from Ewing's sarcoma: report of two cases.Ewing's sarcoma is a malignant osseous neoplasm that affects mostly children and young adult males. Clinically, the neoplasm presents with oedema, swelling, and pain of the involved area. Histopathologically, Ewing's sarcoma consists of solid sheets of small round cells, with vesicular nuclei and scant cytoplasm, arranged in irregular masses separated by strands of fibrous tissue, with areas of necrosis en masse intermingled with intratumoural haemorrhage. Ewing's sarcoma is an extremely aggressive neoplasm and metastases to sites such as lung, pleura, other bones, central nervous system, liver, and regional lymph nodes frequently develop in early stages of the disease. Surprisingly, despite the highly aggressive biological behaviour of this neoplasm, cutaneous metastases from Ewing's sarcoma are very uncommon. We report two patients with Ewing's sarcoma of the bone who developed cutaneous metastases. As in other internal malignancies, the onset of cutaneous metastases in patients with Ewing's sarcoma indicates a poor prognosis.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/29. Malignant by name or malignant by nature: a case of Wegener's granulomatosis diagnosed as adenocarcinoma of unknown primary.Most malignancies are characterised by malignant behaviour at some point in the disease trajectory. However, non-malignant diseases may exhibit malignant behaviour. A diagnosis of malignancy based on fine needle aspiration cytology (FNAC) can be misleading. We report a case of Wegener's granulomatosis initially diagnosed by FNAC as adenocarcinoma of unknown primary. The limitations of FNAC, the disparate prognoses of the two diagnoses despite a compatible clinical picture and the need for clinical vigilance in monitoring a disease course are highlighted.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
5/29. Late relapse of adrenocortical carcinoma in beckwith-wiedemann syndrome. Clinical, endocrinological and genetic aspects.We report on a girl with an unusual beckwith-wiedemann syndrome (BWS) and hemihypertrophy, who developed an adrenocortical carcinoma with atypical clinical behaviour. At 4 y of age the girls was admitted to hospital with cushingoid features, virilization, increased excretion of steroids and low serum ACTH. A right-sided adrenocortical carcinoma was removed. At age 12.5 y the cushingoid features reappeared together with a tumour in the left thigh. A CT scan of the thorax and abdomen revealed pulmonary metastasis only. Corticosteroid excretion was increased and serum ACTH level suppressed. The femoral and the pulmonary metastases were removed and histology showed adrenocortical carcinoma. Excretion of corticosteroids subsequently normalized. Meningeal and pulmonary metastases with similar histologies appeared one year later with normal hormone values. Twenty-two months after the recurrence the girl died of an intracranial metastasis. Southern blot analysis of the LITI transcript in the KvLQT1 gene in the BWS region on chromosome 11p15 revealed hypomethylation of the maternal allele. CONCLUSION: adrenocortical carcinoma in childhood may recur years after onset and at rare sites and hormonal levels may be an insufficient indicator of small metastases.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/29. Late pulmonary metastasis in uterine PEComa.Perivascular epithelioid cell tumours (PEComas) other than angiomyolipoma, clear cell "sugar" tumour of the lung, and lymphangioleiomyomatosis are very rare mesenchymal tumours. The uterus seems to be the most prevalent site of involvement, but only 13 cases of uterine PEComa have been described. Three of these cases exhibited local aggressive behaviour and only one showed metastasis. Because of the extremely small number of cases, PEComas are considered tumours of uncertain malignant potential. This report describes a 68 year old woman, who presented with multiple pulmonary lesions seven years after the initial diagnosis of well differentiated endometrial stromal sarcoma. Histological and immunohistochemical analysis of the pulmonary lesions, in addition to re-evaluation of the primary uterine tumour, led to the final diagnosis of metastatic uterine PEComa. The findings indicate that any PEComa might have malignant potential. Spreading of this tumour to other organs might become evident even several years after primary manifestation.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/29. Primary peripheral adenoid cystic carcinoma of the lung. A case report.We report a rare case of peripheral adenoid cystic carcinoma of the lung, showing unusual pathological and clinical features, namely rapid growth, local aggressive behaviour, huge tumour size, no endobronchial component or submucosal infiltration, and a rapidly progressive clinical course. Extensive surgery resulted in considerable palliation of symptoms, but not in prolonged survival. The reported case emphasizes the malignant potential of peripheral adenoid cystic carcinoma of the lung.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/29. Hypergraphia associated with a brain tumour of the right cerebral hemisphere.Two different neurobehavioural abnormalities have been reported under the term hypergraphia. One has been described in temporal lobe epilepsies and the other in the acute stage of strokes of the right cerebral hemisphere. The latter type of hypergraphia in a patient with a metastatic brain tumour confined to the right hemisphere is reported. Such hypergraphia is a general right hemisphere sign that is not peculiar to strokes in the acute stage and the writing behaviour is inattentive.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
9/29. Paraneoplastic limbic encephalitis--case report and review of literature.We report a 77-year old man who presented with a sub-acute dementia associated with aggressive behaviour, ataxia, rapid progression and death. No cause for his illness could be detected in his lifetime, but at post mortem he was found to have paraneoplastic limbic encephalitis and a bronchogenic tumour. A diagnosis of paraneoplastic limbic encephalitis should be considered in the differential diagnosis of unexplained dementias and appropriate investigations performed to diagnose the condition.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/29. 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) |
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