1/17. Mucosal malignant melanomas of the head and neck with special reference to cases having a prolonged clinical course.A histological re-examination and re-classification of primary mucosal tumours of the head and neck region, treated at Radiumhemmet and Karolinska Sjukhuset during the period 1927-1970, revealed that 41 tumours were malignant melanomas. All these 41 tumours were located in the nasal cavity, paranasal sinuses and oral cavity and not a single case of primary mucosal malignant melanoma was found in other locations of the head and neck region. In the present study, the long-term prognosis has been analysed. The follow-up period was at least 5 years and ranged up to 48 years. It was found that mucosal malignant melanomas had a very poor prognosis with a five year survival rate of 17% (7 of the total 41 cases) and a ten year survival rate of 7% (3 of the total of 41 cases). The unpredictability of the clinical behaviour of this tumour type is elucidated by cases with a prolonged clinical course despite a primary relatively limited surgery, repeated local recurrences and regional lymph node metastases in an early stage of the disease. Thus, there is always a never-ceasing risk of death in the tumour disease when once a malignant melanoma has occurred. For this reason a meticulous and lifelong follow-up of tumour patients is stressed, and also the value of repeated surgery of local recurrences and regional lymph node metastases.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/17. Metastatic choroid plexus papilloma: a case report.choroid plexus papillomas (CPPs) are generally regarded as benign tumours, with a favourable long-term prognosis. Complete resection should result in cure. We present a case of diffuse craniospinal seeding from an apparently completely resected fourth ventricular primary tumour. A 51-year-old male is discussed, who presented 5 years following complete resection of a CPP from the fourth ventricle, with a progressive history of left sided tinnitus, hearing loss, impotence and recent low back pain. Imaging demonstrated multiple craniospinal lesions explaining his symptomatology. Differential diagnosis lay between long standing CSF seeding, malignant transformation in the primary tumour, or metastatic spread from an undefined source. He underwent whole body FDG-PET scan which demonstrated a single metabolically active lesion in the sacral canal. A subtotal excision biopsy of this sacral lesion was performed which was indistinguishable histologically from the primary tumour resected from the fourth ventricle. Histological and functional imaging characteristics of the primary tumour have been unhelpful in predicting its subsequent behaviour. The present case illustrates the extremely rare consequences of metastases from this histologically benign tumour and adds to the literature on metastatic craniospinal disease.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/17. Frontal agraphia, (including a case report).Frontal agraphia has always been a subject of interest, although very few documented case reports have been published. A 57 year old male clerk, suffering from adenocarcinoma of the signoid colon, presented with mild headache and difficulty in writing. Minor features of dysphasia could be detected, but there were no alexia, agnosia or apraxia. brain scan and angiography confirmed a metastasis in the posterior part of the left prefrontal region. The patient was able to write single letters, and had no difficulty with automatic writing or copying. The writing of words and of sentences was more affected. After a brief review of the literature and discussion, the authors concluded that frontal agraphia is related to frontal lobe diorders, especially the inability to translate verbal signals into motor behaviour (i.e. writing).- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/17. Aggressive angiomyxoma: a clinicopathological and immunohistochemical study of 11 cases with long-term follow-up.AIMS: To report the clinicopathological and immunohistochemical features and longer term biological behaviour of aggressive angiomyxoma, an uncommon mesenchymal neoplasm occurring predominantly in the pelvi-perineal region of adults. Using immunohistochemistry, possible overexpression of CDK4 and MDM2 was analysed, which might point to (cyto)genetic alteration(s) in chromosome region 12q13-15, an area reported to be altered in this tumour entity. methods AND RESULTS: Cases (n=11) of aggressive angiomyxoma were retrieved from the consultation files of the Comprehensive Cancer Centre of the Middle netherlands (IKMN) panel for soft tissue tumours. Clinical and follow-up information were obtained, and immunohistochemical analysis was performed using antibodies directed against vimentin, cytokeratin AE1/AE3, desmin, alpha-smooth-muscle actin, CD34, S-100 protein, oestrogen receptors, CDK4 and MDM2. Five patients were female (age range 24-47 years; median 39 years), and six patients were male (age range 36-69 years; median 44.5 years). Of 11 cases, 10 arose in the pelvi-perineal area and 1 arose in the abdominal cavity in close relation to the bladder. Morphology was consistent with previous reports of this entity. Immunohistochemically, 8 of 11 cases were desmin positive (5 of 5 positive in females; 3 of 6 positive in males), 6 of 11 cases were positive for alpha-smooth-muscle actin, 5 of 11 cases were CD34 positive, 11 of 11 cases, irrespective of gender, were positive for oestrogen receptors and 3 of 11 cases were positive for cytokeratin AE1/AE3. Strong, diffuse nuclear positivity for CDK4 expression was present in all 6 cases tested, while only 1 of 11 cases tested for MDM2 showed weak focal positivity. Clinical follow-up in all cases (range 1-216 months; median 72 months) showed one local recurrence (9%) after 36 months. No metastases or tumour-related deaths were noted. CONCLUSIONS: The sex distribution of cases reported in this study was roughly equal, in contrast to previous reports emphasising the predominance of this tumour in females. Our study confirms the local aggressive nature of aggressive angiomyxoma, although our local recurrence rate is lower than previous reports (9% versus 36-72%); no metastases and/or disease-related patient deaths were documented. All cases arising in females were positive for desmin, while three of the six cases arising in males were negative for desmin, supporting previous findings and indicating that the lesion may be somewhat different in males. The strong diffuse positivity for CDK4 in all six cases tested goes some way in implicating CDK4, either directly or indirectly, in tumourigenesis. The negative immunostaining for MDM2 would argue against functional amplification of this gene.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/17. Metastatic solid-pseudopapillary tumour of the pancreas: clinico-biological correlates and management.Solid-pseudopapillary tumour of the pancreas is a rare neoplasm of young women, currently categorised in the world health organization classification under exocrine pancreatic tumours. Increased awareness of this condition correlated recently with an apparent rise in incidence as well as recognition of more aggressive clinical courses. We describe two patients with solid-pseudopapillary tumour of the pancreas. A smaller, localised tumour in an unusually young white man was surgically excised with no evidence of recurrence after 2 years. The other case also had an uncommon presentation, with an aggressive course resulting in vascular encasement of the superior mesenteric bundle and aorta, and local involvement of the mesenteric lymph nodes. A literature review was carried out, and the main clinico-pathological features and strategies of treatment of solid-pseudopapillary tumour of the pancreas are presented. Pathological, genetic and molecular features distinguish solid-pseudopapillary tumours from pancreatic ductal adenocarcinoma. Furthermore, neuroendocrine differentiation can be found focally in occasional cases of solid-pseudopapillary tumour. patients with localised disease are usually cured by surgery. Prolonged survival can be seen in the presence of distant metastasis, if such lesions are resected surgically. Chemotherapy and radiation therapy are used in rare cases when resection is not possible. No current chemotherapy regimens are considered standard in the treatment of this tumour. A rational chemotherapy protocol for such a rare tumour needs to consider its origin and clinical behaviour. However, the indolent clinical progression of solid-pseudopapillary tumours is similar to that of pancreatic neuroendocrine tumour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/17. Clear cell neoplasm.We are reporting four cases of clear cell neoplasm. Local infiltration and destruction was observed in one case while in a second case, originating in the sublingual gland, metastasis to the lymph nodes occurred. The behaviour of these neoplasms has prompted the suggestion that these tumours be designated carcinomas rather that noncommittally tumours or neoplasms (Batsakis and Regezzi, 1977). The histopathological characteristics of our four cases conform to those that have been articulated and believed to be the distinctive features of these tumours (Batsakis and Regezzi, 1977). It is hoped that ours and similar reports will be helpful towards clearing the diagnostic and taxonomic confusion regarding these tumours.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/17. Acute retrograde amnesia as first presentation in terminal metastatic cancer.A case is presented of a 2-week onset of acute retrograde amnesia as initial presentation, caused by presumed cancer. While acute retrograde amnesia has been reported in the literature, a report linked to cancer has not previously been published. An 82-year-old Caucasian woman presented to the emergency department, with 3-day history of increasing confusion and mild frontal headaches. Until 2 weeks previously she had been living on her own and coping with her activities of daily life. She believed very firmly that she was living with her husband in the house in which they lived in over 30 years ago. A magnetic resonance imaging scan demonstrated a lesion extending from cortex anteriorly to the right basal ganglia posteriorly. Postgadolinium enhancement was consistent with a diagnosis of a primary or secondary neoplasm. In some patients, cognitive behaviour changes or amnesia is the sole presenting feature of a serious underlying pathology. A lesion in either the temporal or frontal lobe can lead to this presentation. A family conference was convened and there was decision to take a conservative approach and not to investigate further. She was discharged to the care of her daughter and died 3 months later without return of her memory.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/17. Haemangiosarcoma following irradiation of a haemangioma of the face. (Case report).The malignant complications of irradiating benign conditions are well known and fortunately less frequent than in a previous era of widespread use of superficial roentgen therapy. However, because of the long latent period from exposure to irradiation until the development of the tumour, the clinician must continue to be alert. The behaviour and treatment of a haemangiosarcoma occurring 18 years after irradiation of a haemangioma is described.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/17. Salivary duct carcinoma in major and minor salivary glands. A clinicopathological analysis of four cases.Salivary duct carcinomas are an uncommon but distinct group of highly malignant salivary gland tumours. We report the clinical course, pathological findings and surgical treatment of this tumour in 4 patients. In one patient an intraductal tumour developed in a minor salivary gland, while in the other three patients, a major salivary gland was involved by an infiltrating salivary duct carcinoma. We point out the highly aggressive biological behaviour of the tumour when occurring in the major salivary glands, in contrast to the benign course of the intraductal carcinoma in the minor salivary gland.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/17. Secretory carcinoma of the breast in adults: emphasis on late recurrence and metastasis.Secretory (so-called juvenile) carcinoma of the breast, first described in children, occurs also in adult women, predominantly in the third decade. Less commonly it is seen in older age groups, up to the eighth decade. We report five patients with this tumour; one, a female aged 73, is the oldest age at which the tumour has been recorded, and one is the first report in an adult male in whom the disease recurred after 20 years, only the second recorded death attributable to this tumour type. Secretory carcinoma in adults is potentially more aggressive than in childhood. Nodal metastases are more frequent and sometimes more extensive. recurrence of tumour after surgery developed in four of our five patients at 3, 8, 15 and 20 years. Slow growth and delayed recurrence are characteristic of many of these tumours. death from systemic metastases is rare, but may ensue either rapidly or following a long latent period after treatment. Prolonged follow-up is needed to assess accurately the biological behaviour of this tumour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
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